Neurology Flashcards

1
Q

Anton syndrome

A

Anton syndrome is due to bilateral parieto occipital infarcts causing cortical blindness with accompanying denial and confabulation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Leber’s hereditary optic atrophy

A

Leber’s optic atrophy is a maternally transmitted mitochondrial disorder with identified mutations at positions 11778, 3460, 15257, 14484. Presentation is in young men with rapid permanent central vision loss.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Uhthoff’s phenomenon

A

It is associated with demyelination and optic neuritis. Temporary decrease in visual acuity with increased body temperature after a hot shower bath or exercise. Symptoms increase with rise in body temperature.
Patient can get into a hot bath but cannot get out.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pulfrich phenomenon

A

It is seen in demyelination and optic neuritis. There is misperception of the trajectory of moving objects. Fading of vision might be there.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Cauda Equina Syndrome

A

Cauda Equina Syndrome - LMN
caused by the compression or injury to the nerve roots in the thecal sac of the lumbar spine and perineum (saddle anesthesia).

This syndrome presents as:
- low back pain
- motor and sensory deficits in the lower extremities (Flaccid)
- urinary retention
- fecal incontinence

Cauda equina syndrome can also present due to an intradural extramedullary abscess. If a spinal abscess with epidural collection is suspected, then a lumbar puncture is contraindicated.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Tabes Dorsalis
tabes means wasting away

A

Tabes Dorsalis - occurs 10-35 yrs after primary syphillis
- dorsal sensory columns degenerates
- Rhomberg becomes positive
- Argyl Robertson pupil present
- Bilateral forehead wrinkling + ptosis
- General hypotonia, Absent reflexes and absent or extensor plantars
- Deep achilles tendon pain absent
- Charcot joints

Other Features rare now:
- Wide based high stepping gait of sensory ataxia
- Zones of cutaneous analgesia
- Ligament laxity allowing extremes of leg movements
- Foot ulcers
- Lightening pains along limbs
- Pel’s crisis: Ocular crises in tabes dorsalis: paroxysmal sudden pains affecting the eyes + tearing + photophobia
————————————-
D/d - Freidrich ataxia, MS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

General paralysis of insane

A

General paralysis of insane
- feature of neurosyphillis
- Early dementia progresses to euphoria
- delusions of grandeur
- Epileptic fits
- Hand, Lip, Tongue tremors (trombone tremor)
- tongue darts in and out involuntarily
- Spastic paraparesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Meningo-vascular syphillis

A

Meningo-vascular Syphillis
- isolated cranial nerve palsies (3, 6 common)
- Cerebral or spinal stroke
- Meningism
- Epilepsy
Other Rare features:
- syphillitic amyotrophy - resembles MND
- Meningomyelitis
- Pachymeningitis
- Acute transverse myelitis
- Erb’s spastic paraplegia
- Endarteritis with aortitis, aneurysm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Circle of willis

A

Circle of willis
MCA splits into 3 - ACA, PComA, Main MCA
Branches- Ophthalmic + ant choroidal artery
Anterior Cerebral artery joins by Ant communicating Art

Posterior communicating artery joints PCA
Posterior Cerebral artery
2 pca joints to form basilar
basilar artery with pontine perforating branches
branch - AICA, PICA

2 vertebral arteries joins other end into basilar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Subclavian Steal syndrome

A

Subclavian steal syndrome
- stenosis at origin of subclavian artery on R/L side
- Blood flow diverts to vertebral artery, specially during exercise due to this stenosis and BP will be low on affected side and features of vertebro-basilar insufficiency will be present as:
- Vertigo, Syncope,
- Auditory hallucinations
- Diplopia
- Bilateral blurring of vision or transient bilateral blindness
- Affected arm easily tires during exercise
- fainting attacks while drying out laundry or taking out stuff from top shelf
- Bruit over subclavian artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Parasympathetic Ganglions of Head - SPOC - 7793

A

SPOC-7793: Parasympathetic ganglions of head, numbers are for cranial nerve involved.
1. Submandibular Ganglion:7
Preganglionic - 7th facial nerve (chordaTympani branch)
PostGanglionic - directly to sublingual and submandibular glands
————————
2. Pterygopalatine Ganglion: 7
Preganglionic - 7th nerve, Greater petrosal branch
Postganglionic - V2 branch of Trigeminal,
To - Nasopharynx, Lacrimal, Palate
—————————
3. Otic Ganglion: 9
Preganglionic: 9th, Lesser petrosal N.
Postganglionic: Auriculotemporal N. to parotid gland
————————–
4. Ciliary Ganglion: 3
Preganglionic: 3rd, Oculomotor nerve
Postganglionic: Short ciliary nerves to sphincter pupillae and ciliary muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Superior Orbital fissure contents

A

contents of Superior Orbital Fissure:
it is in sphenoid bone at upper orbit
- Superior Ophthalmic vein
- Inferior Ophthalmic vein
- Oculomotor nerve, 3
- Trochlear nerve, 4
- Abducens nerve, 6
- Branches of Opthalmic division V1:
1.Lacrimal nerve
2. Frontal nerve
3. Nasociliary nerve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Inferior Orbital Fissure contents

A

Inferior Orbital fissure is in sphenoid and maxilla,
what passes through it:
- Inferior ophthalmic Vein
- Infra Orbital artery
- Infra Orbital vein
- Zygomatic branch of V2
- Infra orbital branch of V2
- Orbital branches of Pterygopalatine ganglion (remember SPOC 7793)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Jugular foramen contents

A

Jugular foramen is in occipital & temporal bones & contents are:
- 9, 10, 11th cranial nerves
- Posterior meningeal artery
- Ascending pharyngeal artery
- Inferior petrosal sinus
- Sigmoid sinus
- Internal jugular vein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Branches of External Carotid Artery -
Mnemonic:
Some Anatomists Like Freaking Out Poor Medical Students

A

Branches of External Carotid Artery - Mnemonic: Some Anatomists Like Freaking Out Poor Medical Students
1. Superior Thyroid Artery
2. Ascending pharyngeal artery
3. Lingual artery
4. Facial artery
5. Occipital artery
6. Posterior auricular artery
7. Maxillary artery - to ear, nose, teeth, TM, Palate - middle meningeal artery
8. Superficial temporal artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pterygoid venous plexus communications

A

Important communications of pterygoid venous plexus are:
- Anterior Facial vein
- Inferior ophthalmic vein
- Maxillary vein to Retromandibular vein to external subclavian vein to Jugular vein
- cavernous sinus through emissary veins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

branches of ICA - Internal carotid artery
A VIPS COMMA

A

A VIPS COMMA - branches of ICA
A- Anterior choroidal artery
V- Vidian artery
I - Inferolateral trunk
P- Posterior communicating artery
S- Superior Hypophyseal artery
C- Carotid tympanic artery
O- Ophthalmic artery
M- Meningo-Hypophyseal trunk
M- MCA
A- Anterior Cerebral artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Dystrophia myotonica - clinical features - ABCDEFGH
1 form of muscular dystrophy

A

Mnemonics as ABCDEFGHI

A - Auto Dominant, by 20-30 yrs
B - Balding (frontal)
C - Cardiomyopathy,
C - Cataracts,
C - CTG repeats upto 1000 in DNA
D - Diabetes, Distal myopathy
E - Expressionless face (myotonic)
F - Facial muscle dystrophy
G - Gonadal (Testicular) atrophy
H - Heart block,
H - Hypo-Gamma-globulinaemia
I - Infections, recurrent

sagging sad face due to poor muscle tone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Trigeminal nerve branches

A

Trigeminal nerve, 5th cranial nerve, pontine origin, Sensory, Has 3 main divisions:
V1, Opthalmic Division: branches into
- SupraOrbital nerve
- SupraTrochlear nerve
- Infratrochlear nerve
- External nasal nerve
- Lacrimal nerve
V2, Maxillary Division, branches into
- Infraorbital nerve
- Zygomaticofacial nerve
- Zygomatico-temporal nerve
V3, Mandibular Division, branches into
- Mental nerve
- buccal nerve
- Auriculo-temporal nerve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Abducens nerve

A

Abducens, 6th cranial, pontine origin, longest course, supplies Lateral Rectus
Palsy causes weakness of lateral eye movements - with diplopia on looking laterally (side of palsy)- outer image disappears on covering weak eye
Causes of palsy:
- Stroke, Trauma, Raised ICT, DM, MS
- Lyme disease, Syphillis, TB, Meningitis
- Vasculitis, Sarcoidosis, SLE
- Orbital tumor, cellulitis,
If abducens palsy is noted - check Limb power and Deep tendon reflexes to rule out cortico-spinal involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Cervical Spondylitis

A

Cervical spondylitis:
- Cord myelopathy due to disc herniation, osteophytes, bony spurs
- Starts as neck, hand pain, paraesthesia referred to scapula & progresses to:
- Gait problems
- Urinary issues - retention/incontinence
- gradual spastic paraparesis,
- Cough, straining causes sudden leg weakness
- Loss of Bicep DTR’s
- Wasting of Hand muscles, C5C6 common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Leg Deep Tendon Reflexes and
Babinski/Chaddock/Oppenheim/Gordon signs

A

DTR’s in Leg
Knee DTR - L3L4 - tap quadriceps
Ankle DTR - L5S1 - tap achilles
Plantar reflex - scratch along lateral sole to(down to up), also called babinski reflex - upgoing is UMN sign, absent in tabes dorsalis

Chaddock sign - stroke foot laterally below malleolar skin back to front and look for babinski response - if up going - UMN lesion suspected

Oppenheim Sign - stroke with fingers along anterior tibial shin downwards, babinski like upgoing response indicates UMN lesion

Gordon Sign: - firm squeezing of calf muscle firmly shows babinski like upgoing response- suspect UMN lesion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Foot movements-Muscles-Nerves

A

Foot movements-Muscles-Nerves
Plantar flexion - by Gastrocnemius, soleus - S1 Sciatic

Foot Dorsi flexion - Tibialis anterior, Long foot extensors, L4L5 (common peroneal-sciatic)

Foot Inversion - Tibilais anterior, Tibialis posterior - L4, Tibial nerve

Foot Eversion - Peronei muscles, S1, common peroneal-sciatic

Great Toe Extension = Extensor hallucis longus - L5 root

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Lumbo-Sacral PLexus

A

Lumbo-Sacral Plexus of nerves
Major nerves - FOSIPS (6)
- Femoral nerve L2L3L4
- Obturator nerve L2L3L4
- Superior Gluteal Nerve - L4L5S1
- Inferior Gluteal nerve L5S1S2
- Pudendal nerve S2S3S4
- Sciatic Nerve L4L5S1S2
————————————–
Minor branches of this plexus 11
- Iliohypogastric nerve
- Ilioinguinal nerve
- Genitofemoral nerve
- Lateral cutaneous nerve of thigh
- Nerve to quadratus femoris
- Nerve to inferior gemellus
- Nerve to obturator internus
- Nerve to superior gemellus
- Nerve to piriformis
- Nerve to Levator ani
- Nerve to external anal sphincter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Histamine Response Test for nerve lesion
Histamine Response Test is for determination whether a nerve lesion is pre ganglionic or post ganglionic- Procedure - a drop of 1% Histamine is applied to affected dermatome and skin is pricked through it. Normal Response = Triple flare within 10 min Post Ganglionic Lesion = no response Pre Ganglionic Lesion = persistent flare response even at 3 weeks
26
Tonic Clonic meaning
Tonic Clonic Seizures A tonic seizure causes a sudden stiffness or tension in the muscles of the arms, legs or trunk. The stiffness lasts about 20 seconds and is most likely to happen during sleep. Tonic seizures that occur while the person is standing may cause them to fall. Clonus" (KLOH-nus) means fast stiffening and relaxing of a muscle that happens repeatedly. In other words, it is repeated jerking. The movements cannot be stopped by restraining or repositioning the arms or legs. Clonic (KLON-ik) seizures are rare and most commonly occur in babies. Tonic-clonic seizures involve both tonic (stiffening) and clonic (twitching or jerking) phases of muscle activity. Tonic-clonic seizures may start with a simple partial seizure or aura. The person may experience changes in sensation, mood or emotion leading up to the tonic-clonic seizure.
27
Rule of 2 for Lumbar Plexus
Rule of 2 of Lumbar plexus 2 roots supplies 1 action and next 2 other opposite action. At Hip Joint: Flexion by L2L3 - Iliopsoas muscle Extension by L4L5 - Glutes & Hamstrings ---------------------------------- At Knee Joint: Extension: by L3L4 - Quadriceps Flexion: by L5S1 - Hamstrings --------------------------------- At Ankle Joint: Flexion by L4L5 - Dorsiflexion, Posteriaris tibialis extensor Extension: S1S2 - Plantar flexion, Calf muscles
28
Ulnar Nerve
Ulnar Nerve, Medial cord, C8T1, Sensory to 2 and motor to 6 Muscles supplied: 1. FCU - flex wrist & extend against resistance to feel FCU tendon 2. FDP - ULNAR 1/2, flex distal phalanx of little finger against resistance 3. Hypothenars-abduct little finger against resistance 4. Interossei - Hold paper between little and ring finger tightly, dont let pull 5. Medial 2 lumbricals 6. Adductor pollicis - grasp paper between index & thumb, weak AP makes thumb to flex to hold paper-known as Froment or Journal Sign Wardenberg sign - Involuntary abduction of little finger due to weakness or paralysis of the adducting palmar interosseous muscle and unopposed action of the radial innervated extensor muscles (digiti minimi, digitorum communis ). Treatment of isolated Wartenberg's sign consists of re-routing the extensor digiti minimi. Among the other techniques, Belmahi's "tie lasso" is preferred when flexible claw hand is associated with Wartenberg's sign
29
Wardenberg Sign
Wardenberg sign - Involuntary abduction of little finger due to weakness or paralysis of the adducting palmar interosseous muscle and unopposed action of the radial innervated extensor muscles (digiti minimi, digitorum communis ). Lesion is proximal to wrist. Treatment of isolated Wartenberg's sign consists of re-routing the extensor digiti minimi. Among the other techniques, Belmahi's "tie lasso" is preferred when flexible claw hand is associated with Wartenberg's sign
30
High Ulnar Lesion
Ulnar paradox Less obvious claw hand as FDP paralysed and doesnot flex DIP so now claw obvious. Lesion is at elbow or at cubital tunnel or ulnar groove
31
Ulnar claw Hand (Low Lesion)
Distal digits of index, middle fingers flexes but of ring + little fingers do not (ulnar side) -when lesion is beyond wrist. Muscles innervated by the ulnar nerve: - Abductor digiti minimi. - Flexor digitorum profundus. - Flexor digiti minimi. - Opponens digiti minimi. - Ring finger lumbricals. - Small finger lumbricals. - Dorsal & palmar interosseous muscles. - Adductor pollicis. Primary Site of lesions: 1. Ulnar tunnel between pisiform & hook of hamate Or 2. at Guyon’s canal at wrist 3. Medial epicondyle 4. Brachial plexus injury
32
Median Nerve Supplies LOAF muscles in Hand = Lumbricals, 1 & 2 = Opponens pollicis = Abductor pollicis brevis = Flexor pollicis brevis
Median Nerve, Lateral +Median cord C5C6C7C8T1 roots, Feel between tendon of FCR & palmaris longus@wrist Sensory to Radial 3&1/2 fingers Motor to 9 muscles 1. Pronator teres - palm up-to palm down against resist 2. FPL to thumb-distal phalanx flexes 3. FDP - distal phalanx flexes of 2+3no 4. APB - thumb abduction against resist 5. Palmaris longus 6. Flexor digitorum superficialis and 7. Flexor carpi radialis Clinical relations: Carpal tunnel syndrome, hand of Benediction, simian/ape hand deformity, pronator syndrome
33
Carpal Tunnel Median Neuropathy
Tingling numbness beyond wrist or base of thumb Thenar wasting Numbness on lateral 3 & 1/2 fingers on all palm side and only distally on dorsum of fingers. Tinel’s Sign: Tap on wrist for symptoms Phalen’s Sign: wrist flexion induces symptoms Hyperalgesia in median nerve territory Katz Hand Diagram: pt draws area of numbness Weakness of abductor pollicis brevis (thumb) Lesion above Elbow: Hand of benediction, (thumb + index extended) + lateral forearm wasting Anterior Interosseus Branch lesion: isolated weak pincer grip Rx: Splinting at wrist, specially at night Steroid injections into wrist Yoga techniques NCV study & decompression (open/ Endoscopic)
34
Radial Nerve Testing
Radial Nerve - extensor nerve Sensory to small patch skin on dorsum between thumb & index & post arm Motor to all extensor muslces of ARM+Forearm+Hand - Triceps testing @ elbow extension C7C8 - Wrist extension against resistance - Finger extensions - Elbow flexion with midpronation-Brachioradialis - Supination against resistance Radial palsy will cause wrist drop Common sites of injury: = at elbow or below elbow: #'s, dislocation, ganglions = Shaft of humerus fracture - in groove = Axilla: crutches, Saturday Night palsy due to overnight stupor sleep over arm of chair pressing radial nerve Low Lesion: Tricep Jerk preserved High Lesion: Tricep jerk Lost C7 root lesion: Tricep DTR lost + wrist drop + weak shoulder adduction + weak elbow extension
35
Pre-trunk branches of Brachial plexus
Pre-trunk branches of brachial plexus 1. Dorsal scapular nerve - to rhomboids (C5) - test: ask to put both hands on hips - push elbow forward against resistance - feel rhomboid on back 2. Long thoracic nerve - to Serratus anterior muscle - ask to put both hands on a wall and push it away - look for winging of scapula in weak muscle 3. Phrenic nerve - C5C6C7, innervates diaphragm - do sniff test
36
Sniff test for Phrenic nerve function
The fluoroscopic sniff test, also known as diaphragm fluoroscopy, is a quick and easy real time fluoroscopic assessment of diaphragmatic motor function (excursion). It is used most often to confirm absence of muscular contraction of the diaphragm during inspiration in patients with phrenic nerve palsy or breathing difficulties following stroke. Chest radiograph demonstrating a newly elevated hemidiaphragm often precedes a sniff test. Technique = ask the patient to practice sniffing before the study with the patient either standing (preferred) or supine, perform frontal fluoroscopy of the diaphragm at rest, breathing quietly through an open mouth ask the patient to take a few quick short breaths in with a closed mouth ('sniffs') causing rapid inspiration occasionally, repeating (3) in the lateral projection is required to evaluate the posterior hemidiaphragms Findings = In normal diaphragmatic motion: the diaphragm contracts during inspiration: moves downwards the diaphragm relaxes during expiration: moves upwards both hemidiaphragms move together in healthy patients 1-2.5 cm of excursion is normal in quiet breathing = 3.6-9.2 cm of excursion is normal in deep breathing 2 up to 9 cm can be seen in young or athletic individuals in deep inspiration 2 excursion in women is slightly less than men 2 In abnormal diaphragmatic motion: the affected hemidiaphragm does not move downwards during inspiration paradoxical motion can occur Interpretation = Absence of diaphragmatic movement confirms phrenic nerve palsy in the appropriate clinical setting. A mass anywhere along the course of the phrenic nerve requires further workup, usually with neck and chest CT. A hilar mass due to lung cancer is the most common finding on CT and a classic exam case. Normal diaphragmatic excursion can also be impaired in patients with: previous diaphragmatic trauma or surgery neuromuscular disorders previous stroke
37
Trunk Level Branches of brachial plexus
Trunk Level branches of brachial plexus 1. Supra scapular nerve to supra-spinatus, infra spinatus - test by shoulder abduction and rotation (rotator cuff function) 2. Nerve to subclavius - C5C6 - The subclavius muscle is a short, triangular muscle of the thoracic wall that lies underneath the clavicle. It originates from the first rib and courses laterally to insert on the undersurface of the middle third of the clavicle. The main function of the subclavius is to stabilize the clavicle during movements of the shoulder girdle. In addition, it is significant in preventing injuries to the adjacent neurovascular structures due to fractures of the clavicle.
38
Costo-clavicular syndrome
Costo-clavicular syndrome The vessels and nerves running behind the subclavius muscle can sometimes become entrapped between the clavicle and the first rib, inside the costoclavicular space. This is referred to as costoclavicular syndrome and marks one of the three types of thoracic outlet syndromes (TOS). This syndrome can be caused by fractures and thoracic deformities, amongst others. Typical symptoms include irritations of the brachial plexus and circulatory disorders of the arm
39
Brachial Plexus Brachial means pertaining to upper limb
Brachial plexus - 3-6-3=3-5-5 Trunks 3, Divisions 2x3=6, Cord 3 Trunks: - Upper, C5C6 - Middle, C7 - Lower, C8T1 -------------------- Divisions - Anterior and Posterior, 2x3=6 ---------------- Cords: - 3 Lateral - joining of Anterior division of Upper trunk & Anterior division of lower trunk - Has 3 branches Medial Cord - posterior division of Lower trunk - Has 5 branches Posterior Cord - all posterior division of all trunks input to this one - Has 5 branches
40
Radial Nerve Palsy: Wrist Drop
Radial Nerve Palsy - Posterior Cord, Extensor to whole arm - 2 sites of injury 1. Axillary Radial injury - - Triceps extension & Reflex both lost - Brachioradialis extension lost - Supination lost - Wrist Extension + MCP extension lost 2. Mid arm injury against Humerus - Saturday Night Palsy-recovers in weeks. Brachioradialis, Supinator, Wrist and MCP extension lost but Tricep preserved. # First dorsal interossei skin numbness ----------------------------- # If wrist is passively extended, Power grip improves and person can extend IP joints but cannot extend (still) MCP joints. # Finger adduction & abduction normal if hand placed flat on a table with radial action eliminated (as lumbricals & Interossei normal)
41
Pseudobulbar Palsy: Features
Pseudobulbar UMN Features in brainstem distribution - salivating at angle of mouth - Unable to protrude tongue, lies in mouth, small and spastic - Donald duck speech, High pitch, slurred, monotonous - Dysphagia with nasal regurgitation @swallowing attempts - Palatal Movements absent - Exaggerated Jaw jerk reflex - Bilaterally Generalised spasticity, - Extensor Plantars - Emotionally labile - Bilateral internal capsule stroke is the most common cause
42
Pseudo-Bulbar Palsy: Causes
Pseudo-Bulbar Palsy - resembles a bulbar palsy but due to UMN cause - Bilateral IC strokes is most common - Multiple sclerosis - Motor neuron disease - High Brainstem tumors - Head injury
43
Lateral Medullary Syndrome(Wallenberg, PICA) Posterior Inferior Cerebellar artery occlusions - Posterior circulation Stroke
Lateral Medullary-PICA syndrome Face numb + oppositie body numb Structures affected: ipsilateral = Tractus solitarius - loss taste = Sympathetic pathways - Horner's = Cerebellar connections - cerebellar signs - DANISH = 5th nerve nucleus-same side face numb = 9, 10th nerve - palatal paralysis, no gag reflex, no palatal movements and: = Spino-thalamic tract: contralateral loss of pain & temperature sensation Hiccups Vertigo, nystagmus, Nausea, Vomiting (vestibular nuclei affected)
44
Medial Medullary Syndrome (Dejerine)
Medial Medullary Syndrome: - The medial medullary syndrome is a result of blockage of the **anterior spinal artery** or medial medullary **branches of the vertebral** artery. It affects the pyramid, medial leminiscus and hypoglossal nerve resulting in: - contralateral hemiparesis of the limbs, - ipsilateral lingual paralysis, and - contralateral impaired perception of joint position, vibration and discriminative touch.
45
Basal Pontine Infarction (Millard-Gubler)
Basal infarction of the pons = follows occlusion of pontine branches of the basilar artery. Corticospinal fibres are dispersed at this level and partial contralateral hemiplegia results. Abducent nerve damage paralyses the lateral rectus muscle. If the lesion extends dorsally the facial nerve may be affected.
46
Internuclear Ophthalmoplegia (INO)
Internuclear ophthalmoplegia arises when the medial longitudinal fasciculus is involved either in a vascular lesion or in a demyelinating disease such as multiple sclerosis. Ipsilateral 3rd nerve connection to contralateral 6th nerve nucleus is lost affecting conjugate horizontal eye movements.
47
Weber Syndrome - Basal Midbrain Lesion, POCS, PCA occlusion Superior alternate Hemiplegia
thrombosis of central branches of the posterior cerebral artery (PCA), affecting = corticospinal and corticobulbar fibres in the basis pedunculi, with contralateral hemiplegia. Destruction of the oculomotor nerve causes severe ipsilat ophthalmoplegia, only the lateral rectus and superior oblique muscles escaping. = Ipsilateral 3rd nerve: Down, Out eye with dilated fixed pupil = Contralateral Hemiplegia
48
Nothnagel's syndrome, (Midbrain Lesion)
Midbrain lesion Ipsilateral 3rd nerve palsy Cerebellar ataxia
49
Foville's syndrome, Pons
Foville's syndrome Pontine Lesion = inferior medial pontine stroke syndrome that most commonly presents with contralateral hemiparesis and ipsilateral abducens and facial palsies. Same as Millard-Gubler but with lateral conjugate gaze palsy
50
Jugular foramen syndrome, 9,10,11
Jugular foramen syndrome: - Palsies of 9, 10, 11 cranial nerves - Loss of taste along posterior 1/3rd of tongue - Reduced Gag & palatal movements - Uvula deviated to other normal side - Weak wasted sternocleidomastoid - Weak shoulder shrug & looks flat Look for brainstem signs as well such as: - wasted fasciculated deviated tongue - Ipsilateral horners syndrome -Spastic limb paraparesis -------------------------------- Causes: = Neurofibroma of 9,10,12 nerves = Meningioma = Epidermoid tumors = Glomus or carotid body tumors = Metastasis in the area = CP angle lesions with 8th nerve + = Middle ear infection to post fossa = Cholesteatoma = Granulomatous meningitis
51
Syndrome of Vernett
Vernet syndrome refers to paralysis of the IX, X, and XI cranial nerves traversing the jugular foramen. A variety of lesions can involve the jugular foramen, such as tumors, vascular lesions, infections, and trauma
52
Syndrome of Collet-Sicard
Collet-Sicard syndrome (CSS) is a condition showing unilateral paralysis of lower cranial nerves (CN) IX, X, XI, XII. It was first described by Collet, in 1915, in a World War I soldier with a bullet injury in the mastoid region. Hypoglossal foramen is near external opening of jugular foramen and external lesions can involved 9+10+11+12 cranials.
53
Syndrome of Villaret
Unilateral lesion affecting nIX to nXII, Collet-Sicard syndrome, in combination with ipsilateral Horner syndrome is called Villaret syndrome. Lesions affects 9,10,11,12 and cervical sympathetic chain as well. # 10+11 nerves only affected - Syndrome of Schmidt # 10+11+12 nerves affected - Syndrome of Hughlings Jackson
54
Infantile Hemiplegia
Infantile Hemiplegia - some CNS insults in first yr of life/ birth/ Fetus leads to unilateral hemiplegia with some times Mental retardation. - Encephalitis - Toxic Encephalopathy - Infections as Measles, Pertusis, Scarlet Fever Features: - Unilateral small limbs with weakness - Epilepsy - Chorioid or athetoid limb movements - Ankle clonus, Jaw Clonus - Limb spasticity - Brisk reflexes on weak side as UMN - Spastic Dysarthria
55
Horner's Syndrome - Sympathetic chain
Horner's Syndrome features: = Ipsilateral meiosis - due to excess parasympathetic activity = Enophthalmous (smallish eye) = Anhidrosis (no forehead sweating) = Partial Ptosis same side -------------------------------- causes are as: Carotid Dissection Vertebral Dissection Brainstem strokes - wallenberg syn. Multiple sclerosis Syringomyelia Neck surgery or trauma Neck invasive pancoast tumor Neck Lympnodes affecting sympathetic Carotid artery aneurysm
56
Clinical triad of Carotid Dissection
Clinical Triad of Carotid Dissection 1. Partial Horner's syndrome + 2. Pain on same side of face, neck, head 3. Later Cerebral or retinal ischemic signs typical history of - hyperextension or rotation of neck as - painting the ceiling, coughing, vomiting, Sneezing, post anaesthesia administration. Rx by anticoagulation if no intra cranial extension.
57
SACD - Subacute Combined Degeneration of the Cord
SACD features: = Peripheral paraesthesias (burning soles). unsteady gait = Absent ankle DTR + Extensor plantars = Stocking & Glove sensory loss = Rhomberg sign positive = Dorsal column degenerates, vibration, Position, Touch lost = Lhermitte sign = Lemon yellow skin pallor-B12 deficient anemia = Glossitis, Spleenomegaly, Ca stomach, --------------------------------------- SACD causes: = Addisonian pernicious anemia, Autoimmune associations = Total/Partial gastrectomy = Stagnant loop syndrome = Ileal resection or Crohn's disease = Strict vegan diet (Lacks B12) = Fish Tapeworm = Chronic Tropical sprue = Congenital Intrinsic factor deficiency
58
Lhermitte phenomenon
Lhermitte phenomenon: electrical sensation down the spine into the legs on flexing the neck seen with = Multiple sclerosis = SACD = Cervical Spondylosis = Cervical cord tumors Reverse Lhermitte sign in neck extension is strongly seen in Cervical spondylosis
59
Dysarthria - speech issue, broken
Dysarthria varieties: 1. **Cerebellar dysarthria** - slow staccato scanning speech - reading each syllable slowly, look for other cerebellar signs-DANISH 2. **Spastic Dysarthria** - tongue stuck in mouth - hot potato or Donald duck speech, High pitched, rigid parts = Pseudobulbar palsy = Parkinsons disease = Myotonic dystrophy = Huntington's chorea = General paralysis of insane 3. **Flaccid dysarthria**: = Bulbar palsy = Stroke involving 7,9,10, 12 nerves 4. **Myopathic dysarthria**: weak muscles = Myasthenia gravis - worse with repeat 5. **Structural dysarthria**: = Hypothyroidism, low pitch, guttaral, = Amyloidosis, large tongue = Multiple oral ulcers or painful thrush = Parotitis or TM joint arthritis with pain
60
Facial nerve Palsy - clinical
Clinical features of Facial Palsy - frontalis forehead wrinkling lost LMN - Bell's Phenomenon - protective as corneal exposure minimized - eye turns upward when closed - Nasolabial angle flattening - Unable to close eye completely - angle of mouth flattened - Anterior 2/3rd of tongue lost taste - Hyperacusis due to stapedius palsy
61
Facial Nerve Palsy - Causes
Unilateral Facial palsy = Bell's palsy (idiopathic) = CP angle tumors (5,6,7,8 all affected) Acoustic neuroma, meningioma = Parotid Tumors = Herpes zoster also affects 5, 8 nerves = Pontine lesion - MS, stroke etc = Middle ear- otitis, cholesteatoma = Mononeuritis multiplex: due to DM, PAN, Churg-strauss, Wegner's, Sarcoid, Carcinoma, SLE, RA, Amyloidosis, Leprosy ----------------------------------------- Bilateral Facial Palsy: = Lyme disease = Bilateral bell's palsy = Guillain Barre syndrome = Myasthenia gravis = Congenital facial diplegia = Motor neuron disease, rare = Sarcoidosis
62
Tardive dyskinesia Means late happening abnormal movement
Tardive dyskinesia - means late happening abnormal movements like lip smacks, chewing, pouting, grimacing (orofacial-dyskinesia) - there might be choreoathetosis of the limbs and trunk. It started later upto an year after starting or stopping medications as Phenothiazines. Neuroleptics causing dyskinesia: = Phenothiazines (chlorpromazine) = Butyrophenones (Haloperidol) = Substituted Benzamide - metoclopramide = Reserpine = Tetrabenazine Other abnormal movements due to Neuroleptics: = Acute dyskinesias-soon after drug = Akathisia - uncontrollable restlessness with inner feeling of unease = Parkinsons syndrome - responds to anticholinergics rather than L-DOPA
63
Carpal Tunnel Syndrome - Causes
CTS - Causes of Median Neuropathy - Amyloidosis of chronic diseases as - Hypothyroidism, Myxoedema - Rheumatoid arthritis - Pregnancy, OC pills - Acromegaly - Osteoarthritis of the carpus - Primary Amyloidosis, thick nerves - Trophaceous Gout - Tuberculous tenosynovitis
64
Internuclear Ophthalmoplegia INO
It is due to Lesion of medial longitudinal fasciculus which connects 3rd nerve nucleus to opposite 6th nerve nucleus, usually due to demyelination of MS. Clinical - adduction failure on affected side (RE adduction failure with Right MLF lesion) - and nystagmus and diplopia. When other eye is covered - adduction is normal. Causes: - MS - Diabetic vascular lesions - Werrnicke's encephalopathy - SOL
65
Wernicke's encephalopathy
Acute Thiamine (B1) deficiency causes it, usually in alcoholic but also possible in Hyperemesis gravidarum, Malnutritions - triad of ocular dysfunction, ataxia, and altered mental status. - Ocular palsies ie ophthalmoplegia - Nystagmus - Loss of pupillary reflexes - Ataxia - peripheral neuropathy - Korsakoff psychosis, irreversible Diagnostic Triad of Wernicke Encephalopathy Encephalopathy = Disorientation = Inattentiveness = Apathy = Decreased level of consciousness = Memory Impairment Ataxia = Wide-based gait = Inability to stand Ophthalmic abnormalities = Gaze-evoked nystagmus = Impaired abduction = Conjugate gaze palsy = Internuclear ophthalmoplegia = Impaired horizontal vestibulo-ocular reflex = Optic disc edema = Retinal hemorrhage
66
Nystagmus Classification
Nystagmus Types: - Physiological nystagmus - Ocular nystagmus - Vestibular nystagmus - Cerebellar nystagmus - Ataxic nystagmus
67
Vestibular Nystagmus
Vestibular Nystagmus - can be 1. Peripheral: Lesion in labyrinth or vestibular nerve = = Fast component to opposite of lesion = Fatiguable = Unsteadyness on same side = Open eye unsteadyness = Vertigo, Deafness 2. Central - Lesion in Vestibular nucleus and its connections (Medullary) = Spontaneous but can with head spin = not fatiguable = Has vertical component usualy = Downbeat can be
68
Peripheral Vestibular Nystagmus:Cause
Causes of Peripheral vestibular nystagmus are as: = Labyrinthitis = Menier's syndrome = Acoustic neuroma = Vestibular neuronitis with or without deafness = Middle ear degenerative disease = Hypertension = Head injury
69
Central Vestibular Nystagmus-Causes
Causes of central Vestibular nystagmus are as : = MS, CVA = Encephalitis = Tumors = Syringobulbia = Alcoholism = Anticonvulsants --------------------------------------- Arnold-Chairy malformations - has downbeat nystagmus while looking straight ahead ----------------------------- Formaen Magnum lesions as tumors, syringomyelia, cerebellar degeneration - downbeat nystagmus on lateral gaze
70
Holmes-Adie-Moor Syndrome
Holmes-Adie-Moore Syndrome: = Very Slowly reactive (tonic)pupil to Light - remains dilated in light - Doesnt constrict fast - in dark also do not dilate fast = constrict slowly to accomodation/ convergence and might become smaller than other normal one = Absent DTR's acute development might cause - blurring and photophobia = no eye palsy, no ptosis, no diplopia (no features of 3rd nerve palsy) = Hyperreactive to Pilocarpine = Normal reaction to mydriatics
71
Syringomyelia - upper cord
Syringomyelia - upper cord syrinx - Bilateral loss to Pain and heat - Intact to cold but not warm(early) - UL: absent DTR's, wasted ulnar forearm, painless burns & cuts, weak and wasted small Hand muscles - LL: exaggerated DTRS, extensor plantars (Spastic paraparesis) - Kypho-scoliosis - Horner's syndrome may be present expanding syrinx destroys spino-thalamic tract neurons first as they are more medial than other hence pain and temp lost (warm lost earlier than cold)
72
Syringobulbia - brainstem
Syringobulbia - when syrinx extends up into brainstem or starts there = Nystagmus = Ataxia = Lower Cranial nerve palsies = Onion skin facial sensory loss with peripheral lost first = Bulbar Palsy: Wasted tongue with fasciculations, weak SCM+Trapezius, numb face, dysphasia, dysarthria, Palatal palsy, gag weak, aspiration risk = La main succulente: cyanosed puffy cold hands with stumpy fingers = Lost or excessive sweating areas = Charcot Jts: Elbow, Shoulder
73
Syringomyelia Associations
Associations in Syringomyelia, may be = Fused cervical vertebrae (Klippel-Feil syndrome) with short neck = Kypho-scoliosis = Asymmetrical thorax = Sternal depression or Prominence = Cervical ribs
74
Myasthenia gravis - Clinical
MG - clinical features - snarling weak face - ptosis, B/l or unilateral - Diplopia with squint - Fatiguable limb weakness - SOB dangerous, monitor FVC - Proximal myopathy features - Jaw supporting sign - Anti AcH reception antibodies 90% - Do Tensilon test (Edrophonium), muscle improves after it
75
MG associations
Associated conditions with Myasthenia - Thyrotoxicosis, 5% - Hypothyroidism - Rheumatoid Arthritis - Sarcoidosis - Diabete mellitus - Polymyositis, dermatomyositis - SLE - Pernicious anemia - Sjogren's syndrome - Pemphigus
76
Myasthenic Crisis
Myesthenic crisis, Cholinergic crisis - Nausea, vomiting - Salivation, sweating - Lacrimation - Miosis - Pallor - Breathlessness due to weakness (FVC) - Collapses, confusion Can be triggers by - emotional stress, infections, stress, drugs as Aminoglycosides, Colistin, Polymyxin, Curare, Quinine, Quinidine etc
77
Lambert Eaton Myasthenic syndrome
Eaton Lambert: - Malignancy associated MG like (oat cell Ca of bronchus) - Fatiguability improves with exercise - Absent Tricep reflex improves with exercise of the limb - Proximal muscle wasting - Ulnar nerve EMG shows increasing amplitude response which decreases in MG
78
Cervical Myelopathy
# Most common cause if Cx spondylosis Cervical Myelopathy, UMN = Brisk DTR's + Spastic weakness Legs = extensor plantars + ankle clonus = Lost Vibration and position senses (Rhomberg becomes +) = Writhing, pseudoathetosis of Hands (with eye closed) = Rarely spinothalamic sensations lost = UL biceps, Supinator DTR's inversion means they are absent but distal finger or Triceps reflex is brisk (increased) = Myelopathy Hand sign, abducted 5th finger, Rare sphincter issues = Hand wasting uncommon as supplied by C8T1) by Cx spondylosis affects up = +_ Lhermitte phenomenon
79
Proximal Myopathy
Proximal Myopathy causes: - Polymyalgia rheumatica - Diabetic amyotrophy - Cushing syndrome, moon face, acne - Hypothyroidism, Hyperthyroidism - Carcinimatous myopathy, small cell lung cancer mostly - Polymyositis-Dermatomyositi - McArdle disease - myophosphorylase deficiency - MERRF syndrome - Drugs: Alcohol, Steroids, Beta blockers, Amiodarone, INH, Chloroquin, Lithium, Labetolol, Methadone etc
80
Lateral Popliteal Palsy
Lateral Popliteal Palsy (aka Common Peroneal nerve palsy): = Foot drop, cannot dorsiflex = Numbness on lateral side of foot = Numb patch between 1+2 Toe = cannot evert foot --------------------- Common peroneal nerve has 2 branches - superficial and deep. Superficial peroneal supplies skin on lateral side of foot and peroneus longus & brevis muscles. Deep branch supplies skin between 1 & 2 toe and anterior tibial muscles ------------------------- Preservation of inversion of ankle by tibialis posterior distiguishes common peroneal palsy from L4L5 root lesion.
81
Bulbar Palsy Clinical+Causes Dysarthria, Dysphagia, S0B Lesions of 10, 12 cranials
Bulbar Palsy clinical features: = Absent palatal movements = Pooling of oral secretions(cant spit out toothpaste) = Dysarthric indistinct speech = Wasted atrophic tongue with fasciculations, Nasal twang and aspiration, hangs out = Nasal regurgitation of food/fluids -------------------------------- Causes of Bulbar Palsy: = Motor neuron disease = Syringobulbia = Gullain barre syndrome = Poliomyelitis = Neurosyphylis = Base of skull or retropharyngeal pathology
82
Choreoathetosis
Choreo-athetosis - brief, jerky abrupt quasi-purposeful involuntary mainly facial + UL movements - present at rest & accentuated by activity - gives general air of restlessness. - Cannot keep tongue protruded, moves in & out. - Abnormal posturing of hands with wrists flexed, MCP hyperextended and prone when raised ----------------------------- Causes of chorea: = Sydenham's chorea of Rheumatic fev. = Huntington's chorea, LL>UL, age >35 = Drugs: L dopa, Neuroleptics = Senile chorea without dementia = Rare: Epidemic encephalitis, Wilson's disease, Hypocalcemia, Thyrotoxicosis, SLE, CO Poisoning,
83
Hemiballismus
Hemiballismus - flinging wild movements of half side of body, mostly UL - due to stroke or some lesion of sub-thalamic nucleus - disappears during sleep - might wear out in 6-8 weeks Dystonia: sustained contraction of some body part - usually neck as in Torticollis, Lordosis, Scoliosis Myoclonus: rapid shock like muscular jerks, often repetitive and may be rhythmic - commonly due to - epilepsy - Essential familial myoclonus - Physiological: stress, exercise, anxiety - Metabolic(renal, respiratory, Liver) - Encephalitis
84
Guillain-Barre syndrome
Guillaine-Barre Syndrome - Acute inflammatory demyelinating polyneuropathy: = usually preceded by URTI or GE due to Campylobacter jejuni in 60% cases = ascending symmetrical paralysis, foot upwards (LMN) (some has only foot drop but might suddenly worsen) = absent DTR's as nerves demyelination = Bilateral facial weakness, Bulbar weak = Autonomic: Labile HTN, Tachycardia, Orthostatic Hypotension = Respiratory paralysis - intubate early, monitor FVC daily Rx - 1. Plasmapheresis initial 2 weeks or IVIG 2. Monitor Vital capacity, Blood gases, ability to cough and swallow daily 3. Supportive
85
Miller-Fischer Syndrome
Miller Fischer Syndrome: = a variation of GBS, = Anti GQ1b ganglioside antibodies ++, = External Ophthalmoplegia + = Ataxia + (as opposed to total paralysis in GBS) = Areflexia, = some motor weakness = High CSF Proteins = also a AIDP
86
Ptosis evaluation
Ptosis with 1. Unilateral small pupils - horners 2. Unilateral Dilated pupils - 3rd nerve palsy 3. B/L Normal pupils fatiguability- MG 4. B/ L Normal Pupils with myotonia - MD Pseudoptosis - thinning of eyelids due to repeated angioneurotic edema Voluntary Ptosis - to suppress diplopia Apraxia of the Lids - patient may have to pull down lower eye lids and tilt the head back to open the eyes.
87
Unilateral Ptosis
Unilateral ptosis causes: = Horner's syndrome with meiosis = 3rd nerve palsy with mydriasis = Myasthenia gravis, early with 1 eye = Congenital/Idiopathic - Tensilon -ve = Myotonic dystrophy: usually B/L
88
Bilateral Ptosis
Bilateral Ptosis causes = Myasthenia gravis = Myotonic dystrophy with other signs = MERRF or CPEO (chronic progressive external ophthalmoplegia) = OculoPharyngeal dystrophy (MERRF) # Kearns Sayre syndrome, (mitochondrial cytopathy with MERRF) = Progressive ophthalmoplegia = Retinopathy = Cardiomyopathy = Ataxia
89
Spinal Cord-Root compression
Spinal Cord-Root compression causes 1. Vertebrae and disc lesions 2. Tumors 3. Vasular causes 4. Inflammatory disorders
90
Spinal Cord-Root Compression-1
1. Vertebrae or disc lesions causing it = Fracture and/or dislocation = Prolapsed intervertebral disc = Spondylosis = Osteoporotic Vertebral collapse = Paget's disease = Atlanto-axial dislocation = Ankylosing spondylosis = Sickle cell disease with lysis
91
Spinal Cord-Root Compression-2
2. Tumors causing compression = Extra-dural: Mets, Lymphoma, Primary Bone tumors = Intra-dural but extra-medullary: Neurofibroma, Meningioma, Sarcoma = Intra-medullary: Ependymoma, Astrocytoma, Secondaries
92
Spinal Cord-Root Compression-3
3. Vascular causes of cord compression = Trauma, haemorrhage = spinal vascular malformations = Intra-dural spinal neoplasms = Coarctation of aorta = Ruptured spinal artery aneurysm = Blood dyscrasias = Anticoagulant causing bleeding
93
Spinal Cord-Root Compression-4
4. inflammatory causes = Transverse myelitis = Spinal Osteomyelitis = TB, Pott's spine = Spinal arachnoiditis = Acute disseminated encephalitis = Neuromyelitis optica (Devic's) -autoimmune, affects optic + cord = Multiple sclerosis = Progressive necrotising myelopathy
94
Spinal Cord-Root Compression-clinical
Spinal Cord-Root Compression features - UMN features- Legs, spastic weakness - Extensor plantars - Exaggerated DTR's or absent if root - Sensory Levels - Spinal back pain - weak plantar flexion in S1 root compr.
95
Abducence Palsy
Abducence palsy causes = Mononeuritis multiplex = Multiple sclerosis = Any raised ICT cause = Neoplasm = Myasthenia gravis = aneurysmic compression = Vascular lesions = Subacute meningitis clinically- cannot abduct eye, convergent squint, diplopia
96
Oculomotor nerve Palsy
# prominent ophthalmoplegia - intrinsic cause as vascular Oculomotor Palsy: eye down & out with ptosis + dilated pupil, Unilateral and angulated diplopia = PCA or IC aneurysm, painful = Mononeuritis multiplex causes (DM, Vasculitis etc) = Brainstem stroke = Multiple sclerosis = Myasthenia gravis = Migraine with ophthalmoplegia = Subacute meningitis = 3rd N. palsy + contralateral hemiplegia is weber's syndrome of brainstem stroke. # Ophthalmoplegia minimal but other features prominent- external compression
97
Cavernous/ Superior orbital fissure syndrome
Cavernous/Superficial Orbital fissure syndrome - - total or near total ophthalmoplegia as 3,4, 6 all nerves affected at both locations - Pain or paraesthesia along V1 division - absent corneal reflex as V1 involved causes: Tumor, thrombosis, Carotid aneurysm
98
Visual Field Defects
1. Homonymous hemianopia - vision loss on same side in both eye- say right side - lesion behind optic chiasma 2. Bitemporal Hemianopia - vision loss on lateral field of both eyes - lesion at chiasma - pituitary tumor, craniopharyngioma, suprasellar meningioma, aneurysm 3. Tunnel vision - central only: RP, Extensive chorioRetinitis, Glaucoma, Hysteria 4. Central scotoma - = with pale optic disc(atrophy), = swollen pink (papillitis) or = normal (Retro-bulbar neuritis)
99
Foster Kennedy Syndrome
Foster Kennedy Syndrome: = visual loss due to compressive optic atrophy in one eye and papilledema in the contralateral eye brought on by increased intracranial pressure. = The classical causes of a true Foster-Kennedy Syndrome are frontal lobe, olfactory groove, or sphenoid wing meningiomas. = The Foster Kennedy syndrome was originally described as the triad of optic atrophy, contralateral papilledema, and anosmia, caused by an olfactory groove meningioma or large frontal lobe tumors. With modern neuroimaging, the diagnosis is often made earlier and anosmia may not be present.
100
Friedreich Ataxia, GAA repeats, inherited, Chr-9 AutoRecessive Early onset, chair bound in teenage
Friedreich Ataxia: degenerative, hereditary, affects dorsal columns, spinocerebellar, optic, CST and cerebellum itself. - Pes cavus as long standing neuropathy - Absent Ankle+knee DTR's+Extensor Plantars - Ataxia + intention tremor, head shake - Coarse nystagmus - Dysarthria - slow slurred or scanning - Impaired Heel-Shin, Finger-Nose, Rapid alternate hand movements(cerebellar) - scoliosis D/d = 1. Tabes Dorsalis (ARP, no deep pain, rhomberg sign+) 2. Multiple sclerosis (brisk DTR, ext Plantars, Optic neuritis history) 3. DM with Cervical spondylosis -absent ankle DTR + ext Plantars 4. Bassen-Kornzweig syndrome 5. Refsum disease 6. Roussy-Levy syndrome
101
Bassen-Kornzweig Syndrome (Abetalipoproteinemia)
- AutoRecessive - no LDL, VLDL, Chylomicrons in serum - very low levels of TG in serum - Steatorrhoea with malabsorption - Acanthocytosis - pigmentary retinal degeneration - spino-cerebellar degeneration resembling clinically Friedreich's ataxia
102
Refsum disease - defective Lipid oxidase
Refsum disease - Phytanic acid accumulation in body due to defective alfa lipid oxidase, AutoRecessive = Abnormal pupils = Optic atrophy, Deafness = Pigmentary retinal degeneration = Friedreich like ataxia = Cardiomyopathy = Icthyosis
103
Roussy-Levy Syndrome, Variant of HSMN (CMT)
Features of **Roussy-Levy syndrome** = demyelinating HSMN, AutoRecessive = Ataxia like Friedreich = Areflexia = Pes cavus = Upper Limb tremors = Kyphoscoliosis = distal limb weakness and atrophy = clumsiness, Impaired Pain, vibration
104
Motor Neuron Disease (MND), Types
MND - Types affects Anterior horn cell i.e. motor spinal nuclei or cranial nerves motor nuclei and has 3 types 1. ALS - Amyotrophic Lateral sclerosis - mixed UMN+LMN involvement with mixed signs, most common, Bulbar weakness can be pre dominant (9, 10) 2. PLS - Primary Lateral Sclerosis - only UMN involvement 3. PMA, Progressive muscular Atropy - only LMN involvement Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to scarring or hardening ("sclerosis") in the region.
105
Motor Neuron disease, Clinical
MND, Clinical features: = adult onset focal muscle weakness and wasting, = Muscular fasciculations = Absent Ankle jerks + extensor plantar = Bulbar weakness with saliva pooling, aspiration risk, nasal speech = a weak grip, you might drop things, or find it hard to open jars or do up buttons = weight loss – your arms or leg muscles may have become thinner over time = difficulty stopping yourself from crying or laughing in inappropriate situations ie labile emotions ----------------------------------- always exclude cervical cord compression
106
MND, Differentials
D/d for MND: = Cervical cord compression = Syphilitic amyotrophy = CNS Lymphoma = SMA of Juvenile type 3
107
Multiple sclerosis, - Relapsing remitting - Primary Progressive - Secondary Progressive
Demyelination is believed to be the result of a chronic T cell-mediated, autoimmune inflammatory response against myelin proteins. Thus, MS plaques often are distinguished by gliosis, inflammatory cell infiltration, and localized myelin/oligodendrocyte destruction. = Fatigue = Blurred vision, Optic neuritis, pale disc = Spastic paraplegia (UMN signs) = Cerebellar signs = 6th nerve Palsy = Trigeminal neuralgia = Pure sensory symptoms = Uhthoff phenomenon = Pulfrich phenomenon = CSF: Proteins rise, Oligoclonal bands = Visual evoked response delayed = MRI demyelination plaque + gadolinium enhancement if active inflammation going on
108
Types of Muscular dystrophy, Genetic, AD, AR or X linked, Do Muscle Biopsy, CK levels & Genetics
Duchenne MD – one of the most common and severe forms, it usually affects boys in early childhood; people with the condition will usually only live into their 20s or 30s ----------------------------------- Myotonic dystrophy – a type of MD that can develop at any age; life expectancy isn't always affected, but people with a severe form of myotonic dystrophy may have shortened lives ---------------------------------------- Facioscapulohumeral MD – a type of MD that can develop in childhood or adulthood; it progresses slowly and isn't usually life-threatening -------------------------------------- Becker MD – closely related to Duchenne MD, but it develops later in childhood and is less severe; life expectancy isn't usually affected as much ----------------------------------------- Limb-girdle MD – a group of conditions that usually develop in late childhood or early adulthood; some variants can progress quickly and be life-threatening, whereas others develop slowly --------------------------------------- Oculopharyngeal MD – a type of MD that doesn't usually develop until a person is between 50 and 60 years old, and doesn't tend to affect life expectancy --------------------------------------- Emery-Dreifuss MD – a type of MD that develops in childhood or early adulthood; most people with this condition will live until at least middle age
109
Myotonic dystrophy
Myotonic dystrophy Features - AutoDom, chromosome 19, both sex - CTG expansion repeat > 50 - 1000s - Classic Type 1, or type 2 proximal - Gradual muscle weakness, foot drop - Hypogonadism, Percussion myotonia - Myotonic facies, grip tonic, - Cataracts, Hypotonia - Cardiomyopathy - Myotonic dystrophy type 1(DM1), also called Steinert disease, has a severe congenital form and a milder childhood-onset form. --------------------------------------- In myotonia congenita, unlike myotonic dystrophy, there is no element of progressive muscular weakness and wasting, cardiac disease, eye abnormalities, endocrine disturbances, or dementia. The life expectancy is the same in patients with myotonia congenita, both dominant and recessive, as in normal individuals.
110
Localization in Hemiplegia
Hemiplegia + Facial Weakness + Dysphasia (speech): = Dominant Cortex lesion - precentral (Motor) or Post central gyrus (Sensory) +++ = Brainstem Signs (nystagmus, ocular palsies, dysarthria, cerebellar signs) - Brainstem lesion or Vertebrobasillar arterial lesion.
111
Parietal Lobe Signs
Sensory cortex involved so Wernicke's Dysphasia + Hemiplegia or Hypotonia (rather than spastic) - + Apraxias, + Agnosias, + Dyslexia, Dyscalculia, Dysgrafia, + Proprioceptive loss --------------------------------------- Agnosia = failure to appreciate the sensory impulses even when peripheral mechanisms intact, Can be: 1. Asterognosis/ Tactile: Cannot recognize familial object placed in Hand with eyes closed 2. Visual Agnosia: Cannot recognize object by sight but can by touch or handling it 3. Auditory agnosia: Can recognize the sound of familial object (phone ring, horn, voice, music) only when allowed to touch or see but not by hearing alone (dominant temporal lobe) 4. Autotopagnosia: Inability to recognize anything is wrong - happens in Left hemiplegia in a right handed person - Hemineglect.
112
Agnosias, a parietal lobe sign
Agnosia = failure to appreciate the sensory impulses even when peripheral mechanisms intact, Can be: 1. Asterognosis/ Tactile: Cannot recognize familial object placed in Hand with eyes closed 2. Visual Agnosia: Cannot recognize object by sight but can by touch or handling it 3. Auditory agnosia: Can recognize the sound of familial object (phone ring, horn, voice, music) only when allowed to touch or see but not by hearing alone (dominant temporal lobe) 4. Autotopagnosia: Inability to recognize anything is wrong - happens in Left hemiplegia in a right handed person - Hemineglect.
113
Apraxias, parietal lobe sign
Apraxia: cannot execute actions but can recognize the senses. Can be: 1. Dressing apraxia: Affects Upper limbs, cannot dress, use pen, comb or toothbrush 2. Gait apraxia: affects lower limbs, mimics ataxia - unable to lift one foot in front of the other. 3. Truncal apraxia: difficulty in sitting on a chair, turning in bed, getting onto bed 4. Facial Apraxia: cannot use face as in cannot whistle, cannot close eyes, cannot put out his tongue. LESIONS - can be in Parietal lobe, corpus callosum, pre motor cortex. 5. Constructional apraxia: usually seen with hepatic encephalopathy - person cannot construct simple figure like squares, triangle using match sticks.
114
Cerebellar Syndrome - Clinical
DANISH - Dysdidokokinesia - alternate rapid motions lost - Ataxia to one side or truncal in vermis - Nystagmus - Intention tremor, starts with action - Scanning speech - ipsilateral Hypotonia of limb with reduced power = Ipsilateral co-ordination clu)msy(do finger node and heel shin test ------------------------- - Pendular knee jerk - Overshooting of outstretched arm on down - Skew deviation of eyes: Ipsilateral goes down & in, contralateral up & Out - Rhomberg + with eyes open and closed in vermis lesions -
115
Cerebellar Syndrome, Causes
Causes of cerebellar lesions = Stroke, ischemic = Stroke, Haemorrhagic = Multiple sclerosis = Abscess (spread from Otitis, chest etc) = Alcoholic degeneration = Brainstem vascular lesions = Paraneoplastic = Friedrich ataxia, codon repeats = CP angle tumors = Arnold chairi malformations
116
Spastic paraparesis, Clinical
Spastic paraparesis clinical features = Lower limb spastic with raised tone = increased deep tendon reflexes = Disuse atrophy, contractures if long = Extensor plantars = Ankle, Patellar clonus = Limb weakness
117
Spastic parapresis, Causes
= Multiple sclerosis = Cord compression = Syringomyelia = Motor neuron disease = Traumatic cord injury = Birth injury-Cerebral palsy-Littles disease = Friedrich ataxia = ASA thrombosis = Hereditary spastic paraplegia = SACD, cord degeneration = Prasagital cranial meningioma = HTLV infection (Tropical SP) = AIDS Myelopathy = Tabes dorsalis
118
Clues for cause of Spastic paraparesis
Clues suggestive of cause of SpasticParaparesis - Cerebellar signs in MS - Pes cavus in Friedrich ataxia - Wasted Hand, inverted reflexes in Cx spondylosis - Horners syndrome in syringomyelia - Fasciculations with MND - Absent-ankles+extensor-plantar-SACD -
119
HTLV-1/2 associated diseases
Human Tcell Lymphotropic virus HTLV-1 associated Tropical Spastic paraparesis HTLV associated Leukemia/ Lymphoma = High WBC counts = unexplained hypercalcemia = Cutaneous lesions = High HTLV antibodies HTLV associated polymyositis HTLV -B-cell chronic lymphocytic leukemia HTLV associated Infective dermatitis HTLV-2 + Hairy T cell leukemia
120
Parkinson's disease, Causes Triad of Tremor+Rigidity+Bradykinesia
Causes of Parkinson's Disease = Idopathic, commonest = Drug induced (phenothiazines) = Post encephalitis Lethargica = Anoxic Brain damage, Post arrest = CO Poisoning brain damage = Manganese toxicity = Neurosyphillis = Cerebral tumors affecting basal ganglion ======================= Other causes with Parkinsonism: = Vascular Parkinsonism = Normal pressure Hydrocephalus = Progressive Supranuclear palsy = Multiple system atrophy, Striatonigral degeneration = Cortico-basal degeneration - asterognosis present - cannot recognize coin placed in Hand closed eyes = Alzeimer's disease = Wilson's disease = Jakob-Creutzfield disease = Hypoparathyroidism (BASAL GANGLION CALCIFICATION)
121
Stiff Person syndrome
Autoimmune association, DM1 Severe progressive muscle stiffness of the spine and lower extremities exacerbated by stress or external stimuli with tendency to fall. Starts 30-50 yr olds, Anti GAD antibodies for DM1 positive in 60%. Other autoimmunes possible as Hypo or hyperthyroidism, Pernicious anemia, Vitiligo.
122
Progressive Supranuclear Palsy
A supranuclear gaze palsy is an inability to look in a vertical direction as a result of cerebral impairment. There is a loss of the voluntary aspect of eye movements, but, as the brainstem is still intact, all the reflex conjugate eye movements are normal. = Axial rigidity = Tendency to fall backward = Subtle dementia = Frontal Lobe syndrome = Parkinsonism
123
Medications for Parkinson's disease
Levo dopa based: Carbidopa/Levodopa 25/100 TID Carbidopa/Levodopa/Entacapone: 25/100/200 BD ----------------- Dopamine Agonists: Non Egots: - Pramipexole 0.125 TID - Ropirinole 0.25 mg TID - Rotigotine 2 mg OD Ergots: - Bromocriptine 1.25 mg TID - Pergolide - Cabergoline S/q Apomorphine as recuse in crisis
124
Parkinson's: Tremor + Rigidity + Bradykinesia (BRT Parkinson)
Asymmetric in Parkinson’s disease think from head to toe - Unblinking expressionless face (Glabellar tap test) can’t blink as bradykinetic - Titubition (head nodding tremor) - Blepharoclonus(on gentle eye closure) - Monotonous speech - Resting Hand tremor - pill rolling - with synkinesis, increases when check with other action like foot tapping - bradykinesia: ask to tap thumb with index repeatedely, slow start with fatiguability - Wrist Cog wheel rigidity - Elbow Lead pipe rigidity - Micrographia on writing - Tendency to fall front or back, cannot balance if pushed - Stooped posture - Festinent shuffling gait
125
Peripheral neuropathy,
distal sensory loss, stocking and Glove, Foot Drop, Hand weakness Common Causes of PN: = Diabetes mellitus = Alcohol and B12 deficiency = Carcinomatous = Hereditary Motor Sensory: CMTooth = Drug induced: - INH, Ethambutol, Amiodarone - Metronidazole, Vincristine, Gold, - Nitrofurantoin, Phenytoin, - Hydralazine, Chloramphenicol, - Cyclosporine
126
Rare Causes of PN
Rare Causes of PN = Guillain Barre syndrome = Polyarteritis nodosa = Rheumatoid arthritis, CTD's = Amyloidosis, thick nerves = AIDS = Chronic IDP ---------------------------------------- = Myxoedema = Acromegaly = Sarcoidosis = Uremia = Lyme disease = Tetanus = Botulism = Paraproteinemia = Refsum's disease = Arsenic poisoning = -Tri-ortho-cresyl phosphate poisoning
127
Prominent Motor PN
Causes of Predominantly motor PN = Carcinomatous = Lead induced = Porphyria = Diphtheria = CMT disease
128
POEMS Syndrome (with myeloma)
Osteosclerotic myeloma associated - Polyneuropathy - Organomegaly - HepatoSplenomegaly, Lymphadenopathy - Endocrinopathy- DM, Hypothyroidism, Hypogonadism - Monoclonal gammopathy, plasma cell disorder, IgA, IgG - Skin changes - melanoderma,buccal fat pad atrophy, High levels of Vasc endothelial growth factor ( VEGF) Rx - Radiotherapy, Melphalan, Thalidomide
129
Multiple System Atrophy
Multiple system atrophy (MSA) is a rare condition of the nervous system that causes gradual damage to nerve cells in the brain. This affects balance, movement and the autonomic nervous system, which controls several basic functions, such as breathing, digestion and bladder control.
130
What is cortico-basal degeneration
Corticobasal degeneration (CBD) is a form of frontotemporal degeneration, a dementia that involves the loss of cognitive functions such as the ability to think, remember, or reason to the point that it interferes with a person's daily life and activities. CBD can affect: Movement. Balance
131
Painless Neck Weakness
= Myasthenia gravis = amyotic Dystrophy = Polymyositis = Motor neuron disease
132
Rinne test, Hearing
The Rinne test is performed by placing a 512 Hz vibrating tuning fork against the patient's mastoid bone and asking the patient to tell you when the sound is no longer heard. Once the patient signals they can't hear it, the still vibrating tuning fork is then placed 1–2 cm from the auditory canal. The Rinne test is used when conductive hearing loss is suspected and used in patients with otosclerosis to determine if a patient might benefit from stapes surgery. The patient should be able to hear the sound of the tuning fork adjacent to their ear, persist for approximately twice as long as the sound they heard over their mastoid process. This is considered a "positive test means normal (air > bone)
133
Weber test, Hearing
The Weber test has been mainly used to establish a diagnosis in patients with unilateral hearing loss to distinguish between conductive and sensorineural hearing loss Place the base of a struck tuning fork on the bridge of the forehead, nose, or teeth. In a normal test, there is no lateralization of sound. With unilateral conductive loss, sound lateralizes toward affected ear. With unilateral sensorineural loss, sound lateralizes to the normal or better-hearing side.
134
Neuromyelitis Optica Devic's disease
**Neuromyelitis optica** (NMO), (Devic's disease) is a rare **autoimmune**, more common in women > men, can have- = Pain in the eyes (Optic neuritis) = Loss of vision. = Weakness or numbness or paralysis in the arms and legs. = Difficulty controlling the bladder or bowels. = Uncontrollable vomiting and hiccups. to diagnose NMO is testing your blood for AQP4 or MOG antibodies. While blood testing can't always confirm NMO — because about 13.5% of cases don't involve identifiable antibodies. **MRI required**. **Eculizumab** is a complement inhibitor of protein C5, preventing the cleavage into C5a and C5b. Eculizumab was the first monoclonal antibody FDA approved for the treatment of NMO/NMOSD in adult patients who are seropositive for AQP-4 and may be considered a first-line treatment option.
135
**McDonald Criteria** for **Multiple Sclerosis** diagnosis, (MRI based) T2 MRI lesions are the white spots observed on MRI using the typical imaging sequences acquired to diagnose and monitor Multiple Sclerosis. The most popular of these sequences is a FLAIR image (Fluid Attenuated Inversion Recovery).
**Criteria** The diagnosis of multiple sclerosis can be made if there is fulfillment of any of these five categories of criteria, depending on how many clinical attacks have occurred: ≥2 clinical attacks with ≥2 lesions with objective clinical evidence with no additional data needed ≥2 clinical attacks with 1 lesion with objective clinical evidence and a clinical history suggestive of a previous lesion with no additional dta needed ≥2 clinical attacks with 1 lesion with objective clinical evidence and no clinical history suggestive of a previous lesion with dissemination in space evident on MRI 1 clinical attack (i.e. clinically isolated syndrome) with ≥2 lesions with objective clinical evidence with dissemination in time evident on MRI or demonstration of CSF-specific oligoclonal bands 1 clinical attack (i.e. clinically isolated syndrome) with 1 lesion with objective clinical evidence with dissemination in space evident on MRI with dissemination in time evident on MRI or demonstration of CSF-specific oligoclonal bands **Dissemination in space** Dissemination in space requires ≥1 T2-hyperintense lesions (≥3 mm in long axis), symptomatic and/or asymptomatic, that are characteristic of multiple sclerosis in two or more of the four following locations: periventricular (≥1 lesion, unless the patient is over the age of 50 in which case it is advised to seek a higher number of lesions) - cortical or juxtacortical (≥1 lesion) - infratentorial (≥1 lesion) - spinal cord (≥1 lesion) Notably, T2-hyperintense lesions of the optic nerve, such as those in a patient presenting with optic neuritis, cannot be used in fulfilling the 2017 revised McDonald criteria 5. **Dissemination in time** Dissemination in time can be established in one of two ways: 1. a new T2-hyperintense or gadolinium-enhancing lesion when compared to a previous baseline MRI scan (irrespective of timing) 2. simultaneous presence of a gadolinium-enhancing lesion and a non-enhancing T2-hyperintense lesion on any one MRI scan **Primary progressive multiple sclerosis** (PPMS)- In addition to the above criteria, the McDonald criteria also defines the diagnosis of primary progressive multiple sclerosis. The diagnosis now requires: ≥1 year of disability progression which can be determined either prospectively or retrospectively with two of the following: ≥1 T2-hyperintense lesions characteristic of multiple sclerosis in one or more of the following regions: periventricular, cortical or juxtacortical, or infratentorial ≥2 T2-hyperintense lesions in the spinal cord presence of CSF-specific oligoclonal bands
136
Multiple Sclerosis Treatment Options
**Acute Relapse:** to shorten it 1. High dose Methyl Prednisolone, 0.5 to 1 Gram IV OD for 3-5 Days 2. Plasma exchange, 5 times if above fails 3. IV IG **Prevention of New Lesions** or further relapse: = Interferon B1a = Interferon B1b = Natalizumab (Tysabri) infusion = Glatiramer acetate (copaxone) = Mitoxantrone (Novantrone) = Fingolimod (Gilyena), oral agent **Symptomatic therapies:** = for neurogenic bladder: 1. Anticholinergics: Oxybutinin, Tolterodine, 2. Intermittent Self Cathterization 3. Urology referral 4. Botulinum toxin injections = for spasticity: Baclofen, Valium, Tizanidine, Botulinum = Neuropathic analgesics = Diet, Peg Tubes = Anti depressants, Behavioral therapy = Offer MS society help
137
MS treatment 1. Interferon beta use criteria 2. Natalizumab use criteria
**Criteria for Use of Interferon Beta**: = > 2 clinical relapses in last 2 years = able to walk 10 m or more = not pregnant or attempting it = > 18 yrs and no contra indications to use of interferon **Criteria for Use of Natalizumab** for MS: = for rapidly evolving severe disease = > 2 relapses in 1 year = > 1 Gadolinum enhancing lesions MRI or significant increase in T2 lesion load as compared to past MRI Natalizumab, sold under the brand name Tysabri among others, is a medication used to treat multiple sclerosis and Crohn's disease. It is a humanized monoclonal antibody against the **cell adhesion molecule α4-integrin**. It is given by intravenous infusion every 28 days. (company- Biogen idec) - can cause PML and if stopped - immune reconstitution syndrome.
138
CTS differentials
= Brachial plexopathy = Peripheral neuropathy = Mononeuritis multiplex = Reflex sympathetic dystrophy, Post trauma to nerve = C6C7 or C8T1 root radiculopathy
139
CTS association, underlying cause
= Hypothyroidism = Amylodosis = Rheumatoid arthritis = Cushing's syndrome = Acromegaly = Distal radius bone fracture = Obesity = Dialysis patient = Pregnancy or Menopause = Vibrating tools Job
140
Anterior interosseus nerve, a branch of Median nerve in proximal forearm
The anterior interosseous nerve supplies flexor pollicis longus & radial part of flexor digitorum profundus. This branch runs on the interosseous membrane with anterior interosseous artery passes deep to the pronator quadratus & supplies it. It terminates by giving articular branches to the distal radio ulnar, radiocarpal & carpal joints. It also supplies to proximal part of the palm, via the palmar cutaneous branch. This branch does not enter the carpal tunnel and is hence spared in carpal tunnel syndrome. Loss of sensations over thenar skin implies proximal lesion in forearm. weakness of thumb and index finger to form a tight circles is found in pronator teres syndrome.
141
Tremor characteristics
- Resting tremor - in Parkinson's - Tremor with changes in posture - postural tremor - Movement or while approaching target - intention tremor in Cerebellar disease - All time tremor - drugs/Metabolic/Physiological - Eased by Alcohol - essential tremor With Double vision - PSP with parkinson With Dysarthria/Salivation/Dysphagia With Hand cramps/ neck cramps - dystonia, torticollis With Postural Dizziness/ Sphinter disturbance/Palpitations (Autonomics) - seen in Shy Drager with parkinsonism With Dementia - Lewy Body dementia with Parkinsonism
142
Drugs causing Tremors
- beta agonists, Salbutamol/ Terbutaline/ Aminophylline etc - Thyroxine - SSRI, TCA's - Caffeine - Valproate, Lithium etc - Alcohol withdrawal - Valium withdrawal - Morphine withdrawal Manganese, Mercury exposure can cause neuro tremors, Parkinsons etc
143
Diplopia: Questions to ask anY RECENT FLU/GE followed by this- suspect Miller-Fischer variant of GBS with triad of Ataxia+Opthalmoplegia+Areflexia -------- Weight loss + Night sweats + Head ache increasing on cough - CNS SOL
= which eye, mono or binocular = Which direction of gaze, descending stairs (SO4), lateral gaze (LR6), all time (3rd nerve) = Blurring or loss of vision = Pain in and around eye = any past same episodes = Fatiguability: improves after resting/sleeping for few Hours? - MG/ inflammatory, GBS?? OR worse when trying to focus as watching TV. non eye symptoms: = any associated headache/ speech problems/ hearing issues/ ringing in ears/ vertigo/ nausea/ vomiting (brainstem) = if Headache: which part, lying more or standing? coughing/ sneezing ? = any sensory-motor limb issues?
144
Acute Diplopia Examination
Assess speech - 1 & 2 step commands Assess orientation in time/place/person All cranial nerves: = Visual acuity = Ptosis = Pupils, Light, Direct/Consensual = Visual Fields, each eye (CVA) = blind spot assessment = Eye movements for 3/4/6, fatiguability for Thyrotoxicosis/ MG/ Miller fischer/GBS/ Vascular = Saccades, palm/ fist: slow in Brainstem diseases = Nystagmus or INO with MS = Fundus: Papilloedema, DM changes ----------------------- TEMPORAL ARTERY TENDERNESS RHOMBERG TEST CEREBELLAR SIGNS PULSE FOR Afib Blood pressure both hands Carotid Bruit, Heart Murmurs Limb signs for stroke
145
Acute Diplopia, causes
Mostly vascular, inflammatory if acute Otherwise also - Autoimmune, SOL, Infiltrative **All cases - stroke work up required** **Autoimmune**: CTD's like RA, SLE, Sjogren's, Behcet;s, MG, GBS **Vascular:** microvascular (DM), thromboembolic (AF), Superior orbital fissure syndrome, Right Cavernous sinus lesions, MCA aneurysm **Infiltrative:** Amyloidosis, Sarcoidosis **Infections:** Lyme, TB, HIV, HTLV, **Other:** Thyroid, Wernickes, CNS tumors as CP angle, Lymphoma, Posterior fossa
146
Pakinson's disease Symptoms
**Atleast 3/5** = Bradykinesia, Tremor, Rigidity, Postural instabilty, Gait disorder **Early ones** - Day sleeping, Loss of smell - Asymmetrical tremor, difficulty with - hand tasks as buttoning - Walking/ turning difficulty (turns en bloc), Reduced speed, Initial freezing then festinant Gait, Loss of arm swing and Falls common - Micrographia - Poor facial expression -**Associated symptoms**: = constipation = Fatigue, poor sleep = Drooling of saliva think of parkinson's plus syndromes
147
Parkinson's **plus** syndrome These are ones with parkinsonian symptoms/ signs but usualy symmentrical and plus issues
? **Visual hallucinations**: Lewy body dementia with Parkinsonism (not PD) ? **Postural Hypotension** or Autonomic symptoms as palpitations, sweating, bladder dysfunction - MSA, Shy Drager ? **Difficulty in looking up**? Progressive supra nuclear palsy with Parkinsonism ? **Vascular risk factors** like smoking/ Strokes/ MI/ DM - Vascular Parkinsonism
148
Parkinson's Signs
Mask like, **expressionless face** Hypersalivation at angle of mouth Reduced blinking - glabellar tap test Unilateral **Hand tremor**, 4-6 hrtz, pill roll (pill rolling type) **Cog wheel rigidity** at wrist **Lead pipe rigidity** at elbow Check tone while patient tapping other hand on to bed (activation phenomenon) which exacerbates tone Ask to write - **micrographia** **Bradykinesia**: ask to tap thumb-index fingers onto each other rapidly and rapidly supinate, pronate hand, can’t do fast Ask to read some thing - soft voice and no tones, no up down **Postural instability** check: Retropulsion (pull suddenly towards you while standing back him, falls) Gait: small shuffling, festinate, can’t turn well and turning en block **Other for Plus syndromes**: - Supine and standing bp - Upwards gaze in pursuit lost in PSP but same vertical doll's eye is not lost - autonomic symptoms inquiry
149
Parkinson's Differential
- Idiopathic when called P. Disease - **Drug induced**: Antiemetics, Neuroleptics (phenothiazines, thioxanthenes, butyrophenones, Clozapine and risperidone) - **Vascular-Parkinson**: Sticky feet + - **PSP:** Vertical gaze palsy, truncal instability - **MSA** - Postural Hypotension (Autonomic failure, Cerebellar ataxia) - Diffuse **Lewy Body dementia:** with visual hallucinations - **Essential Tremor**, improves by Alcohol, intention tremor rather than resting
150
Pure Autonomic Failure
The main symptom of PAF is **orthostatic hypotension**, a sudden drop in blood pressure upon standing. This can cause dizziness, lightheadedness, blurry vision, and weakness. **Other symptoms** can include fatigue, bladder problems, constipation, abnormal sweating, and sleep disorders. Pure autonomic failure is caused by abnormal **accumulation of synuclein** in the brain. Blood pressure may decrease when people stand, and they may sweat less and may have eye problems, retain urine, become constipated, or lose control of bowel movements.
151
Stroke in Young Patients
- Antiphospholipid syndrome - Vasculitis: SLE, Sjogrens - VHD with emboiism or Afib - Aneurysm rupture - Hyperlipidemia - complex Hemiplegic migraine - Carotid dissection - neck extension at Hair dresser's or looking up or trauma, comes with neck pain and Horner's syndrome - Vertebral dissection - brainstem symptoms, same mechanism as carotid - Paradoxical embolism with ASD/ PFO OC pill use and Migraine are independant risk factors - suggest alternate contraception as IUD/Progrestogen only pill or implant, Barrier method
152
Migraine: clinical features
- Unilateral Headache, usually - Aura(visual/ sensory/ auditory/ motor), blurring of vision, **Fortification spectra** (Fortification illusion specifically refers to a visual hallucination where geometric lines arrange themselves in something that many say looks like a castle fortification) - Nausea, Photphobia - Hemiplegia, transient - **Common Triggers**: Chocolate, Cheese, Sleep deprivation, Starvation, Around menses, Caffeine, Alcohol, Exercise - Relief with sleeping in quiet dark room
153
Carotid Dissection Presentation
- sudden neck pain after hyper-extention as at hairdresser or trying to take some thing off top shelf or painting a ceiling looking up - Horner's: Meiosis+Ptosis+forehead sweating loss - Los of taste - Amaurosis fugax - Focal limb weakness some times - Pulsatile tinnitus or audible bruit - Neck swelling like hematoma - Migraine like symptoms - TIA or stroke like symptoms rarely due to broken off clot
154
Transient Loss of Vision, Differential
= TIA, Amaurosis fugax ( Greek "amaurosis," meaning dark, and the Latin "fugax," meaning fleeting) = Acephalgic Migraine, young women = Cardio-embolic, Afib, LV thrombi, IE = Local eye causes - vitreous H'rrhage = Optic neuritis = High Stroke risk factors (ABCD2 score)
155
**ABCD2 score** Admit/ Discharge in case of TIA Score > 4 = review within 24 Hrs Score 0-3 = TIA clinic within 7 days 4 weeks no driving
Age > 60 = 01 point BP @ presentation > 140/90 = 01 Clinical: # weakness = 02 # Speech disturbance no weakness 01 Duration of TIA # > 60 min = 02 # < 60 min =01 Diabetes mellitus = 01 Admit if: Crescendo TIA, 2 or more in 7 days Lateralizing signs on examination Symptoms still present on arrival Fluctuating symptoms Early specialist Review < 24 Hrs if: ABCD2 score > 4 Chronic or Paroxysmal Afib on Warfarin With Prosthetic valve Young, age < 50 yrs
156
Thunderclap Headache, Differentials
= **Subarachnoid headache**, mostly aneurysmal bleed in young patients = Cerebral sinus thrombosis = Arterial **dissection** = Colloid cyst of 3rd ventricle = Reversible Cerebral Vasoconstriction syndrome = Early meningitis (rash, neck stiffness, Photophobia) = **Blitz Migraine** = Cough Headache = Coital Cephalgia
157
Upper Limb Joint Nerve Roots
Shoulder: = Abduction C4C5 = Adduction C6C7 Elbow: = Flexion C5C6 = Extension C7C8 = Pronation C6 = Supination C6 Wrist: = Flexion C7C8 = Extension C7C8 Fingers: = Flexion C7C8 = Extension C7C8 = Adduction C7 = Abduction C7
158
Dementia Differential
= Alzeimer's disease = Fronto-Temporal dementia = Hutington's chorea = Dementia with Lewy bodies = Vascular Dementia (multiple strokes) = Leuco encephalopathies = Parkinson's disease associated
159
Astereognosis
Unable to recognize the objects by touch alone as a key in Hand. Happens with Parietal lobe SOL and cortico-basal degeneration.
160
Anosmia Causes
= Viral URTI, sinusitis, HEAD INJURY = Idiopathic Parkinsons disease = Alzeimer's disease = Olfactory groove meningioma = Superficial siderosis = Kallman's syndrome: Anosmia+Hypogonadism+Xlinked Recessive = Refsum's disease = Paraneoplastic disorders
161
Autonomic Storm
Massive catecholamine surge with rise of BP, Pulse and temperature. = Post CPR anoxia = Toxins and drugs can cause it: = PhenyL propanolamine = Cocaine = Amphetamines = TCA's = Botulism = Tetanus = Neuroleptics = SAH, Stroke, Tumors, Hydrocephalus: Rx: ICU, Supportive, Morphine, Labetolol
162
TESTS for Autonomic System
HRDB: Heart Rate Response to Deep Breath, Valsalva Ratio: persistent expiratory pressure 40 mmHgfor 10-15 seconds. Ratio is maximum tachycardia in phase 2 to maximum bradycardia in stage 4. QSART: Quantitative Sudomotor axon Reflex Test Beat to beat BP response to Valsalva HUT: Head up tilt test
163
Autonomic Neuropathies
Acute Auto-Neuropathy = Subacute Autoimmune = Paraneoplastic auto-neuropathy = GBS (AIDP) = Botulism = Porphyria = Drug induced AN = Toxic AN Chronic Auto-Neuropathy: = Distal small fiber AN = Combined sympathetic and paraSympathetic AN seen with: Amyloidosis DM associated Autoimmune Paraneoplastic Familial (Riley Day syn) Sensory Neuropathy
164
Gerstmann Syndrome
Dominant parietal lobe lesion Dysphasia: Receptive/Expressive/Global Dysgraphia: deficiency in ability to write coherently Dyslexia: trouble with reading despite normal intelligence Dyscalculia: difficulty with calculations Finger agnosia: difficult in recognizing finger names or on touch or name then own as well as of others
165
Chronic myelopathies
Chronic myelopathy of MS SACD, Tabes Dorsalis Familial spastic paraplegia Adrenomyeloneuropathy Primary Lateral Sclerosis Lathyrism: Grass Pea consumption NO inhalation associated Radiation associated Compressive or Metastasis related SLE, Sjogren's Sarcoidosis
166
Ring Enhancing Brain Lesions
Large Brain Metastasis Toxoplasmosis CNS lymphomas Brain abscesses Radiation necrosis Granulomas Tuberculosis lesions Sarcoidosis lesions Demyelination lesions Always consider Stroke, ICH, Trauma as differentials in all REL's.
167
One and Half syndrome
It presents a combination of ipsilateral conjugate horizontal gaze palsy (one) and ipsilateral internuclear ophthalmoplegia (INO) (a half) One-and-a-half syndrome is most often caused by multiple sclerosis (MS), brain stem stroke, brain stem tumors, and arteriovenous malformations.J The location of the lesion is the paramedian pontine reticular formation or VI nerve nucleus.
168
Eight-and-a-half syndrome
Eight-and-a-half (EHS) syndrome is a rare neuro-ophthalmologic condition which defines as one-and-a-half syndrome plus facial nerve palsy ipsilateral to the side of one eye which has no movement (1.5 + 7 = 8.5).
169
Cavernous sinus syndrome Both Cavernous sinuses connects each other.
Cavernous sinus syndrome is characterized by ophthalmoplegia and sensory deficits over the head due to combined deficits of the three cranial nerves (third, fourth, and sixth) responsible for eye movements and pupil function, and at least one branch of the trigeminal nerve. It is Life threatening and may have fever. Suspect if sudden peri orbital swelling and edema both sides. = CV cellulitis: sinus to facial to ophthalmic vein spread. = Mucormycosis in DM or on steroids = Carotid Cavernous fistula = Carotid Artery aneurysm = Meningioma = Nasopharyngeal cancer = Idiopathic granulomatous-Tolosa Hunt sydrome
170
Trigeminal Neuropathy, Causes and location of lesions
1. Nuclear Lesion - Brainstem, Upper cord: MS, Stroke, Syringobulbia, Gliomas, Lymphomas 2. Pre-Ganglionic Lesions: Acoustic neuroma, Meningioma, Mets, Chronic meningitis, Cavernous-Carotid aneurysm 3. Gasserian Ganglion Lesions: Trigeminal neuroma, Herpes zoster, Infection spread from Otitis media/ mastoiditis 4. Trigeminal nerve lesions: Ca nasopharynx, Trauma, GBS, Sjogrens, CTD's, Sarcoidosis, Idiopathic Certain drugs: Stillbamidine, Trichloroethyline
171
Glosso-pharyngeal neuralgia
- similar to trigeminal neuralgia - Shooting pain across throat, tonsils can spread to ears due to tympanic branch - Vagus involvement can cause cardiac arrhythmias, asystole, fainting attacks - Look for zoster lesions in ears (hidden) Treatment: - Pain control with Carbamazepine/ Pregabalin/ Gabapentin - Micro vascular decompression - Rhizotomy of 9, 10th n. in Jugular bulb
172
Palinopsia
Palinopsia: persistent images once the stimulus has been removed from visual field. Seen with occipital lobe SOLs Polyopia: seeing multiple images
173
P1 syndromes, P1 segment branch of PCA
affects: Midbrain, Subthalamic and thalamic areas Claude's syn: 3rd palsy + contralateral ataxia, red nucleus affected Webers: 3rd palsy + contra hemiplegia as cerebral peduncles affected Occlusion of Artery of Percheron: paresis of upward gaze + abulia ie slowness of response + drowsiness Dejerine Roussy syn: thalamic infarcts, contralateral sensory loss of pain + then later severe pain without stimulus or with minor one (neuropathic) Both PCA occlusion: Coma, Unreactive pupils, decerebrate rigidity, Bilateral pyramidal signs (extensor plantars, hemiplagia, spasticity, brisk DTR)
174
Cerebellar syndromes DASHING & PASTRIES
The arterial: Vertebro-basilar system: superior cerebellar artery (SCA), the anterior and inferior cerebellar artery (AICA), and the posterior and inferior cerebellar artery (PICA). Causes of Cerebellar Syndromes: P-araneoplastic- ca lung A-lcohol S-clerosis multiple-MS T-umor, SOLs R-are: Friedrich, ataxia telangiectasia I-diopathic I-atrogenic: drugs: Phenytoin E-ndocrines: Hypothyroidism S-trokes - PICA/SCA/AICA
175
Autonomic Disorders Brain - Spinal - Neuropathy
1. Autonomic-Brain origin: with MSA: 1. Prominant Autonomic: = MSA = Parkinson's + Autonomic failure = Dementia with Lewy bodies 2. Autonomic not prominant + MSA = Parkinson's disease = Progressive Supranuclear palsy = Cortico-basal degeneration = Machado-Josphine syndrome without MSA: 1. Mainly cortical - frontal, Parietal 2. Limbic/ ParaLimbic: = Shapiro Syndrome = Autonomic seizures 3. Hypothalamic disorders 4. Brainstem/Cerebellar: Posterior fossa SOLs, Arnold Chairi malformation, syringobulbia
176
Chorea, (means dance), causes ST VITUS DANCE
S= Sydenhams (in RHEUMATIC fever) V= Vascular strokes I= Increased RBC's (polycythemia) T= Toxins: CO, Mg, Hg U= Uremia S= SLE, MOST COMMON in adults S= enile chorea D= rugs A= Anti Phospho Lipid syndrome N= Neurodegenerative: Huntingtons, neuro-acanthocytosis, DRPLA C= ConceptionS: pregnancy, OCP's E= Endocrine: hyperthyroidism, hypo-, hyperglycemia
177
Neuro-Acanthocytosis
Neuroacanthocytosis refers to a group of genetic conditions that are characterized by movement disorders and acanthocytosis (abnormal, spiculated or star-shaped red blood cells). Four syndromes are classified as neuroacanthocytosis: 1. Chorea-acanthocytosis 2. McLeod syndrome 3. Huntington's disease-like 2 (HDL2) 4. Panthothenate kinase-associated neurodegeneration (PKAN) Symptoms and Signs include: Chorea (involuntary, dance-like) Parkinsonism (slowness of movement) Dystonia (abnormal body postures) Problems walking Muscle weakness Involuntary movements of the face and tongue Tongue/lip biting (which is mostly characteristic of chorea-acanthocytosis) Difficulty with speech and eating ​​​​​Cognitive impairment Psychiatric symptoms - self mutilation Seizures Cardiac problems in McLeods
178
Mini Mental Status Examination (MMSE), 30 point score
Orientation: - Name season/Day/Date/Month/Yr - Name Hospital/Town/State/Country Registration: Tell 3 common objects names and repeat them Attention Calculation: substract 7 from 100 upto 5 Recall: 3 objects told earlier at 5 min and 15 minute recall Language: Name pencil, watch (what is it?) Repeat: no ifs, ands or buts 3 step commands: ask to 1. take this paper 2. fold it in half and 3. put it on table Written commands: ask to read and do - close your eye now Write a sentence - ask to write a sentence by themselves Copy a design - intersecting pentagrams or a clock
179
Treatment of Dystonias
Levodopa Trihexphenydil Oral Baclofen Intra-thecal baclofen Tetrabenazine (12.5 to 200 mg/Day) Botulinum injections DBS, bilaterally for Parkinsons Dystonic storm: Focal or multi focal dystonia during stress - vocal cord dystonia, Airway obstruction and rhabdomyolysis
180
Dystonic Storm
Dystonic storm is a frightening hyperkinetic movement disorder emergency. Marked, rapid exacerbation of dystonia requires prompt intervention & ICU admission. Clinical features of dystonic storm include: = fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failure. Dystonic storm usually occurs in patients with known dystonia, such as DYT1 dystonia, Wilson’s disease and dystonic cerebral palsy. Triggers such as infection or medication adjustment are present in about one-third of all events. Rx = DBS, IV Baclofen, supportive otherwise
181
Dystonia Types
Dystonias are involuntary, sustained, repetitive muscle contractions. 1. Primary dystonia: = Idiopathic Torsional (Oppenheim's) - childhood onset, AutoDom, = Dopa Responsive (Segawa's): childhood, AutoDom, defective tyrosine hydroxylase, improves with sleep, foot dystonia on walking, = Focal Dystonias: Blephalospasm, Torticollis, Oromandibular, Spasmodic dysphonia, Limb dystonias (writers cramps, Musicians) 2. Secondary Dystonia: = Drug induced (metoclopramide) = Toxins : CO, Mg, Hg = Part of neurological illness
182
HIV associated Neuropathies
= Distal symmetric polyneuropathy: Nigh time painful paraesthesia, distal sensory loss, absent ankle DTRs = GBS, CIDP (demyelinating) = Mononeuropathy multiplex: multiple seperate nerves affected, foot drop, wrist drop, focal weakness (HCV, Cryoglobiulinemia) = Polyradiculopathy with CMV: flaccid paraparesis, saddle numbness, Urine retention = Toxic Neuropathy of HAART: with stavadine, didanosine, zalcitabione (need to stop them) = Focal with Zoster/ Lymphoma/ TB
183
Vitamin E deficiency
caused by irregularities in dietary fat absorption or metabolism. Vitamin E is a lipid-soluble nutrient. A normal level is usually with the range of 5.5–17 milligrams per liter (mg/L). Disorders that cause fat malabsorption, including abetalipoproteinemia (Bassen-Kornzweig syndrome, due to genetic absence of apolipoprotein B), chronic cholestatic hepatobiliary disease, pancreatitis, short bowel syndrome, and cystic fibrosis A rare genetic form of vitamin E deficiency without fat malabsorption results from defective liver metabolism. resembles friendrich ataxia ataxias, distal wasting, pes cavus, proximal weakness chair bound Vision loss Test Serum Tocopherol levels Test Serum Tcopherol: Lipid ratio Rx Alfa Tocopherol 400 mg BD
184
Dry Beri Beri (Thiamine low), B1
Thiamine was named by the Williams team as a portmanteau of "thio" (meaning sulfur-containing) and "vitamin". The term "vitamin" coming indirectly, by way of Funk, from the amine group of thiamine itself (although by this time, vitamins were known to not always be amines, for example, vitamin C). Thiamine was also synthesized by the Williams group in 1936. Stocking and glove sensory loss, Distal Leg weakness with ataxia, Lost ankle DTR. in Alcoholics, anorexics, Hyperemesis, Starvation, Do - thiamine check, NCV, Sural biopsy, RBC trans-kerolase activity low Rx Thiamine IV & oral 100 mg OD
185
Peripheral Neuropathy causes
Metabolic: DM, CKD, Porphyrias, Amyloidosis Infections: HIV, Lyme, Syphillis, CMV, Leprosy, Diphtheria Nutritional: B12, B1, B6 deficiency Immunological: GBS, CIDP, Multi-focal motor, Anti-MAG-neuropathy Paraneoplastic: Ca Lung Toxic: Alcohol, HAART, Platinum drugs, Amiodarone, Tick bites, INH, Phenytoin Hereditary: Charcot-Marie-Tooth (HMSN) Vasculitic: PAN, Churg-Strauss, Cryoglobulinemia in HCV Odd: Coeliac, Hypothyroidism, Fabry's disease
186
Neuropathies, Histolgical
1. Demyelinating: mostly proximal, acute or subacute, paraesthesias, weakness, vibration/Positon loss more than pain and temp, areflexia NCV shows velocity > amplitude, demyelination and then remyelination with rapid recovery = GBS, CIDP, Diphtheria, DM, Multifocal Motor neuropathy 2. Axonal : distal weakness more than proximal, slow progression, Pain/temp loss more than vibration/Position, paraesthesias, DTR loss, NCV shows: amplitude > velocity. Axon degenerates and regenerates Slow recovery, examples - toxic, metabolic, HIV related, DM, HMSN 3. Neuronal:non length dependant, rapid paraesthesia, ataxic gait, vibration/ position loss more than pain/temp, areflexia, proprioceptive weakness, rhomberg + NCV: sensory amplitude poor recovery, no regeneration examples: Cisplatin related, Sjogren's related
187
Peripheral neuropathies by Progression/ Time course
Acute: GBS, Toxic, Diphtheric, Thallium related, TriOrthocresyl phosphate (an Organophosphate flame retardant) Sub-Acute: Toxic (Hexacarbon, acrylamide, Angiopathic, Nutritional, Alcoholic Chronic: DM, CIDP, Paraneoplastic, Paraprotenemic, Long Standing: Hereditary (CMTD) Friedrich Recurrent: Relapsing CIDP, Porphyria, Refsum's disease, Hereditary neuropathy with pressure palsies Entrapment neuropathies as in pagets
188
Treatment of Polyneuropathies
General: Education, Foot care, Physiotherapy, offer Support as Social and occupation referral Pain control: Amitryptiline, Dulaxetine, Imipramine Gabapentin/Pregabalin/Mazetol as 2ndline Oral Morphine sulfate 3rd line Oxycontin 5 mg BD, Epidural analgesia Buprenorphine 35 mcg patch Topiramate 25 mg BD Clonazepam HS Treat Systemic Cause IVIG for CIDP, Mutifocal Motor neuropathy CST, Plasma Exchange with CIPD
189
Peripheral Neuropathy by Fiber type Large fiber neuropathy manifests with the loss of joint position and vibration sense and sensory ataxia, whereas small fiber neuropathy manifests with the impairment of pain, temperature and autonomic functions.
Small Fiber sensory: painful, dissociated sensory loss = HSMN, Lepromatous leprosy, DM, Amyloidosis, alfalipoproteinemia, Fabry's disease, HIV etc Large Fiber Sensory: ataxic, = Sjogren's syndrome, Friedrich ataxia, B12 deficiency, Cisplatin, Pyridoxine toxicity Small + Large Fibers: Global sensory loss, carcinomatous, Hereditary, DM, Xanthomatous neuropathy in PBC Vacor ingestion Motor Predominant: GBS, AIDP, Diphtheric, HSMN, Lead, Brachial neuritis, DM Amyotrophy Autonomic: Botulism, GBS, Porphyria, Vacor, Amiodarone, Vincristine, Chronically with DM, Amyloidosis, Sjogren's, HSAN, Chagas
190
Vacor Ingestion Neuropathy
Ingestion of Vacor (a rodenticide) results in a rapidly progressive, severe, distal axonopathy with accompanying diabetes mellitus, autonomic dysfunction, and encephalopathy. Diabetes is due to necrosis of pancreatic beta cells. A clinical syndrome, characterized by acute diabetic ketoacidosis associated with a toxic neuropathy, developed in five men who intentionally ingested a recently introduced rodenticide (Vacor) containing N-3-pyridylmethyl-N'-p-nitrophenyl urea (RH-787). A 7-yr-old boy, who accidentally ingested this poison, died within 14 h. Marked insulinopenia, without a reduction in glucagon levels, suggested a specific beta-cytotoxic effect, which was supported after autopsy in three cases by histopathologic evidence of extensive beta cell destruction. Rx - niacinamide
191
Dementia pugilistica
Punch Drunk Syndrome due to recurrent head injury as in Boxer's. Personality changes, Paranoia, Delusions, Dementia, Parkinsonian signs, Ataxia, Intention tremors
192
Dementia due to Metal exposure
Lead related: fatigue, depression, confusion, episodic abdominal pain, acute intermittent porphyria like pain attacks, raised urinary prophyrins, Anemia with basophillic RBC stippling Rx is with EDTA chelation Mercury related: dementia, ataxia, tremulousness, peripheral neuropathies, cerebellar signs, choreoathetosis Arsenic related: nausea, weight loss, peripheral neuropathy, Mees lines in nails, pigmentation and scaling of skin, Rx with Dimercaprol Aluminium related: progressive encephalopathy as accumulates in brain in ESRD, confusion, aphasia, memory loss, agitation, lethargy, stupor, speech arrest, myoclonic jerks
193
Seizure types:
1. Simple Partial - no LOC, a general strange feeling that's hard to describe = a "rising" feeling in your tummy = a feeling that events have happened before (déjà vu) unusual smells or tastes. tingling in your arms and legs. 2. Complex Partial: focal seizures that start in one hemisphere of the brain and are associated with impairment in consciousness = now preferably called "focal impaired awareness seizure" or "focal onset impaired awareness seizure." 3. Partial seizures with secondary generalization are focal seizures that spread to both sides of the brain causing a grand mal convulsion. Except for beginning locally and spreading, partial seizures look the same as the generalized tonic-clonic seizure, 4. Absence, petit mal seizures: multiple episodes of absence type, sudden freezing, = 3hz spikes in EEG 5. TONIC 6. Clonic 7. GTCS 8. Myoclonic - sudden brief muscle jerks un classified: - Infantile spasms, Neonatal seizures
194
Gullain Barre syndrome
acute inflammatory demyelinating neuropathy, ascending, autoimmune post Flu or campylobacter GE ascending symmetrical paralysis absent DTR ie areflexia Facial palsy possible Lower cranial nerve palsies possible Risk of respiratory failure autonomic features: Arrhythmias, Hypotension, Diarrhea present Look for other cause if bladder dysfunction is prominent as brainstem or cervical myelopathy
195
Normal Pressure Hydrocephalus
Triad of: Ataxia + Dementia + Urinary incontinence MRI: enlarged lateral ventricles without cortical atrophy and patent aqueduct of sylvius CSF shows - high opening pressures, otherwise normal. suspected delayed CSF absorption, slow from arachnoid vili into veins improves with VP shunt
196
GBS mimics
Botulism (descending paralysis) Hypokalemic PP Vasculitis neuropathy Toxins: Lead, Organophosphates, Thallium, Aresenic, Diphtheria Tick paralysis Shellfish Poisonins Porphyria Spinal cord syndromes - quadriparesis Acute form of CIDP Lyme polyradiculopathies
197
Sympathetic Effects Has survival benefits
(Fight or flight response) Tachycardia High Blood pressures Urinary sphincter tone rise (retention) Constipations reduced motility Bronchodiltation Sweating Pupillation dilatation Ejaculation, Orgasm
198
Parasympathetic Effects
Mostly through vagus Bradycardia Hypotension Urinary incontinence/ voiding Diarrhea Bronchoconstriction Pupillary constriction Erection Tearing of eyes Salivation OP poisoning activates it
199
Brachial Plexopathy
Upper Trunk, C5C6 (Birth injury) - Erb's Duschene palsy - jerking/pulling arm down can cause it Arm: internally rotated + elbow extended + wrist flexion and posteriorly directed (Policeman's tip) Weak shoulder abduction+weak external shoulder rotation+weak biceps Lower Trunk C8T1, (Birth Injury) Klumpke paralysis- when baby pulled out by arm from birth canal weak wasted hand, forearm wasting, claw hand, wrist drop Sensory loss on ulnar side may have Horner syndrome Other Causes: Cervical Rib, Thoracic Outlet syn, Tumors, Cx band,
200
Brachial Cord Lesions
Lateral Cord, C5C6: = Musculo-cutaneous, Median nerve = weak flexion & pronation = sensory loss on radial side of Hand/ forearm, damage due to trauma/ stretching of shoulder Medial Cord, C8T1: = Median, Ulnar nerves affected = weak wasted hand: claw = sensory loss ulnar side = Trauma Posterior Cord, C7: = Axillary, Radial nerve affected = Weak deltoid, elbow extensors = weak wrist extensors, weak finger extensors so flexor strong sensory loss on outer arm only - can be with shoulder dislocation/ humerus neck fracture
201
Leprosy neuritis
cause: mycobacterium leprae affects cooler part of body Types: Tuberculous, Lepromatous, & Borderline Tuberculoid: = single skin patch hypoasthetic, near by nerve thickened Lepromatous: = more widespread skin & nerves affected with anhydrosis - spares axilla, anterior chest, scalp, groins and affects: Ear pinna, dorsum hand + forearm + back + Legs deformities develop: claw hand, Finger loss, Foot drop, inability to close eyelids as weak orbicularis oculi.
202
ANS parts
Sympathetic ANS: = T1 to L2 = Superior Cx ganglion = Middle Cx ganglion = Inferior stellate Cx ganglion = Coeliac + other ganglion in abdomen = Lower abdomen sympathetic ganglion Para-sympathetics ANS: = Cranial 3, 7, 9, 10 = Sacral 2, 3 = Ciliary ganglion = Sphenopalatine ganglion = Submandibular ganglion = Otic ganglion = Vagal ganglion cells (Myocardiac) = Pelvic ganglion
203
Lacunar Stroke
Pure-Motor LS: affects posterior limb of internal capsule, focal weakness or arms/ Legs Pure Sensory LS: ventral/ thalamic stroke Ataxic Hemiparesis: infarct in ventral pons, int capsule Syndrome of Dysarthria + Clumsy Hand Infarct of ventral pons or genu of IC
204
ACA syndromes Anterior Cerebral artery
1. Contralateral Leg-foot palsy (branch to motor area for leg) 2. Contralateral arm palsies: arm area if cortex/ IC fibres 3. Cortical sensory loss over toes, foot leg 4. Urinary incontinence - sensory motor-paracentral block 5. Contralateral frontal release signs 6. Abulia, Akinetic mutism (medial FTP) 7. Gait apraxia - frontal cortex near Leg motor area 8. Dyspraxia-Left limbs, tactile apraxia left limbs (corpus callosum)
205
Causes of Ptosis+Ophthallmoplegia
1. Peripheral neuropathies: GBS, Miller fischer syndrome 2. NMJ disorders: MG, Botulism, LEMS, Congenital MG, 3. Thyrotoxicosis 4. Mitochondrial myopathies: Kearns-Sayre, CPEO 5. Congenital myopathy 6. Myotonic Dystrophy 7. Oculo-Pharyngeal dystrophy
206
Myopathy clues
Upper face: inability to close eyes forcibly # Lower face: impaired weak puckering # Neck Extensors: weak, dropped jaw, cannot raise head well #Neck Flexors: cannot raised head when lying down without support #Proximal Arm: unable to raise arms above head #Knee extensor: Genu recurvatum, back kneeling, unable to walk without hyper-extending knee #Shortening of achilles tendon - toe walking, cannot walk with heel flat on floor #Anterior Leg: High steppage Gait, foot drop #Hip Muscles: Waddling gait, gower's sign positive (climbs on himself), cannot get up sitting in chair with arms raised.
207
Causes of Enlarged Nerves
Neurofibromatosis Leprosy Amyloidosis Acromegaly Refsum disease CMTD
208
Hand wasting causes
Anterior Horn cell lesion: MND/ALS, Syringomyelia, Polio, Cord compression Brachial plexus: Cervical Rib, Pancoast tumor, Trauma, also will have wasted shoulder and winging of scapula Peripheral nerve combined median, ulnar lesions mononeuritis multiplex Muscle causea: disuse atrophy in RA
209
Hypothalamic disorders (ANS)
Horners syndrome Wernicke-Korsakoff syndrome Diencephalic syndrome Neuroleptic malignant syndrome Serotonin syndrome Fatal familial insomnia ADH related: DI-SIADH Hypo or Hyperthermia Sexual dysfunction appetite disorders: Bullimia, anorexia Disturbances of BP, HR, Gastric function
210
Acute Disseminated Encephalo-myelitis ADEM
mimics Multiple sclerosis Post vaccination, Measles, Rabies Post infections as Varicella widespread foci of demyelination Can follow: Rubella/ Mumps/ Influenza/ Infectious mononucleosis/ Mycoplasma infections Symptoms: Fever, Headache, Meningism, Lethargy, coma Seizures are common Neuro Signs: Hemiparesis, extensor plantars, DTR lost or brisk, Brainstem signs, Lost sensations, Cerebellar (basically depending on area affected) CSF: proteins high, wbc high, Oligoclonal bands MRI: extensive gadolinum enhanced plaques Rx - Glucocorticoids, Plasma exchange, IVIG
211
CSF in bacterial meningitis
Opening pressure > 18 cm H2O PCR testing for variety pathogens WBC count - 10-100, 000/uL RBC - nil if good tap CSF/Serum Glucose < 0.4 CSF Proteins - > 0.45 mg/dl Gram stain - positive > 60% Culture positve > 80% Latex agglutination - Strep pneumonie, Meningococcus, H influenza, E coli Limulus amebocyte lysate assay - detects gram negative endotoxins
212
Recurrent Falls without LOC
Gait problems: Joint issues, sensory ataxia, cerebellar CVA, Bradykinesia and Rigidity, Confusion Postural Hypotension: Parkinsons plus, Drugs (BB, Omnic, CCA etc), Autonomic, Addison's, Anemia, Cancer Vision Loss, Night blindness Acute causes - Low BP, Low Hb, low Glucose
213
LOC with Low BP
Addison's disease Autonomic failure Anemia, Cancer Medications: beta blockers, ACEi, Diuretics, CCA etc
214
Cranial Nerve Reflexes
Corneal reflex: V1 branch of Trigeminal to Facial Nerve efferent Pupillary reflex: 2nd Optic nerve to 3rd Oculomotor as efferent Jaw Jerk: V3 branch of Trigeminal to V3 branch of Trigeminal efferent Gag reflex: 9th nerve to 10th vagus Carotid Sinus Reflex: 9th nerve to 10 th vagus as efferent Lacrimation: V1 branch of Trigeminal to 7th facial as efferent
215
Restless Legs syndrome
4 criteria must be fullfilled: = Akathisia (an inability to remain still) = Wakeful motor restlessness = Rest symptoms also = More to end of day (evening) Triggers: Iron, Low B12. Low folate, CKD, Pregnancy, Hypothyroidism, DM, Steroids, Antidepressants, Caffeine Rx: correct whatever deficiencies avoid prolonged sitting as moving improves the restlessness Avoid trigger as caffeine LEVO-DOPA therapy
216
Frontal release Signs
appears in frontal lobe damage which removes inhibition/ control but present normally in babies 1 Grasp reflex - touching palm induces grasping of stimulus 2 Palmo-mental reflex - scratching palm causes mental contraction as chin 3. Suckling, Rooting reflex at touching cheek, lips 4. Glabellar Tap: keeps blinking repeatedly 5. Utilization behavior: Reaching out for and inappropriate use of objects: fiddling with your stetho or bedsheets (not seen with alzeimers disease)
217
Frontal Lobe Assessments
1. Verbal Fluency- words starting with F+A+S = shoulder able to say 15 words per letter in 60 seconds. 2. Abstraction - ask abstract meaning of phrases as "Too many cooks spoil the broth" or " Make hay while the sun shines" 3. Response inhibition-Set shifting - inability to shift from one cognitive task to another: (Luria 3 step Test: do Fist-Edge-Palm when told to show) Frontal Release Signs Paratonia-Gegenhalten - abnormal tone/ resistance in limb muscles on contralateral side of frontal lobe disease, (inability to relax)
218
Paratonia - Gegenhalten
Paratonia is the inability to relax muscles during muscle tone assessment. There are two types of paratonia: oppositional and facilitatory. Oppositional paratonia ("gegenhalten") occurs when subjects involuntarily resist to passive movements,[1] while facilitatory paratonia ("mitgehen") occurs when subjects involuntary assist passive movements.[2][3][4] Both types of paratonia have been associated with cognitive impairment or mental disorders, particularly in relation to frontal lobe dysfunction.
219
Hip Movement Muscles, Roots
Hip Flexion: Iliopsoas, L1L2(femoral) Hip Extend: Glutei, L4L5S1 (inf gluteal) Hip Adduct: Adductor longus/brevis/magnus/gracilis/ pectineus, L2L4 (Obturator) Hip Abduct: Glut max, L4L5 (inf gluteal) Internal Rotation: Glut minimus (Sup Gluteal) Ext Rotation: piriformis/ Gemellus superior & inferior, obturator internus & externus, quadratus femoris, gluteus maximus, medius, & minimus, Psoas major & minor, Sartorius.
220
Knee Movements: Muscles, Roots
Flexion: hamstrings, gracilis, sartorius, gastrocnemius, plantaris, and popliteus. L5S1 sciatic nerve (Hamstrings are: Semitendinosus. Semimembranosus, Biceps femoris) Extension: Quadriceps - Rectus femoris, Vastus intermedius/lateralis/medialis (L3L4 femoral nerve)
221
Superior Gluteal Nerve L4L5S1 Hip Abductor
- supplies gluteus max and medius - supplies Tensor fascia lata - supplies piriformis Weakness due to nerve lesion causes weak abduction of same hip and is known as Trendlenberg gait. The Trendelenburg sign is said to be positive if, when standing on one leg (the 'stance leg'), the pelvis severely drops on the side opposite to the stance leg (the 'swing limb'). The muscle weakness is present on the side of the stance leg. Present in paresis of Hip abductors due to superior gluteal nerve damage.
222
Hyperkinetic Movement Disorders
Athetosis: slow distal writhing involuntary movements of mostly hand Chorea: Rapid, semipurposeful, graceful, dance like, involuntary, distal or proximal movements Dystonia: sustained, involuntary muscle contraction, primary/secondary, focal or multiple Myoclonus: sudden, brief, shock like muscle jerks TiCS: brief repetitive, muscle contractions, can be motor/ sensory or vocal, simple or complex Tremor: rhythmic oscillation of body parts due to intermittent muscle contraction - essential/Parkinsons/ Cerebellar
223
CADASIL - Cerebral Autosomal Dominant Arteriopathy with Subcortical infarcts & LeucoEncephalopathy
- due to mutation in notch-3 gene - multiple family members affected with recurrent strokes, migraine without HTN, - progressive dementia in 5th to 7th decade of life - skin Biopsy: dense bodies in arteriolar media No treatment except risk factor control and support
224
Acute Causes of Transverse myelitis
Vascular: infarcts, Herniation of nucleus pulposus Inflammatory: SLE, Sjogrens, Mixed CTD, Behcet's disease, Vasculitis Post Infectious: EBV, HSV, HIV, CMV, Polio Infections: HSV, EBV, CMV, Rabies, Bacterial, TB, Schistosomiasis, Toxoplasmosis Sarcoid Myelopathy: Gadolinium enhancement Oligoclonal bands, Uveitis on Slit Lamp, ACE levels high, Chest lesions, Wheeze, lupus pernio Demyelination cause: MS, Devic's
225
Mononeuritis multiplex
Mononeuritis multiplex is a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body can be affected. As the condition worsens, it becomes less multifocal and more symmetrical. Deep aching pain, worse at night in back/Hip/Legs common in: DM, SLE, RA, PAN, Churg-Strauss, HIV, Dapsone therapy, Malignancy, Lyme/Leprosy/Sarcoidosis Typically in DM patients - appears as sudden acute unilateral severe thigh pain followed by anterior thigh muscle weakness & loss of knee DTR
226
P2 syndromes Distal PCA occlusion
infarction of medial temporal + occipital lobe. presents as contralteral hemianopia with macular sparing.
227
Anterior choroidal Artery Syndrome
branch of internal carotid supplies posterior limb of Internal capsule & white matter. occlusion of this artery presents as: = contralateral hemiplegia = Hemianaesthesia or Hypoesthesia = Homonymous hemianopia
228
MCA Occlusion syndromes Branch of ICA - four main branches, M1, M2, M3, and M4.
complete MCA occlusion: = contra hemiplegia = Hemianaesthesia = Gaze preferences, deviation = contra homonnymus anopia = contra Facial UMN palsy Dominant MCA: Global aphasia Non Dominant MCA: neglect, constructional apraxia, anosognomia
229
Pudendal nerve
pudendum means parts to be ashamed of (genitals) - S2S3S4, travels within pudendal canal, aka Alcock's canal. SensoryMotor - Sensory to ext genitalia, perineum anus - Motor to pelvic muscles(Bulbo-spongiosus, IschioCavernosus, Levator ani) external urethral sphincter, external anal sphincter - Pudendal block is given through inner wall of vagina. It can be damaged in cycling races, childbirth and leads to fecal incontinence
230
Onuf's nucleus
Onuf's nucleus is a small group of neurons located in the ventral horns of the sacral spinal cord. The motor neurons (MNs) of Onuf's nucleus innervate striated voluntary muscles of the pelvic floor and are histologically and biochemically comparable to the other somatic spinal MNs. Pudendal nerve carries neurons from Onuf's nucleus.
231
Sciatic nerve Sciatic means pertaining to hip also known as Ischiadic nerve
L4L5S1S2S3, TRAVELS BEHIND HIP 2 branches: Tibial and Common peroneal. Supplies in: Thigh: Hamstrings Calf: Superficial & deep groups Sole: Muscles, peronei (anterior compartment & deep) Sensory: entire sole, dorsum of foot except 1st webspace MT (by deep peroneal) , Lateral side if Leg and calf. Sural supplies skin lateral edge of foot Saphenous nerve supplies medial leg and mid dorsum of foot. Lesion: Gluteal numbness, foot drop, Weak Hamstrings (knee flexion) Weak eversion of foot DD. -L5S1 radiculopathy
232
Obturator Nerve obturator means a device to close body opening - Ob nerve do this by hip adduction.
L2L3L4, sensory to meal thigh, femoral to front Motor to Hip adductors = Adductor magnus = Adductor brevis = Adductor longus = Gracilis Has 3 branches: Anterior, Posterior & cutaneous
233
Femoral Nerve
L2L3L4 (Motor 04, sensory 02) Enters scarpa's triangle by passing beneath inguinal ligament lateral to femoral artery. Sensory: Front of thigh, Medial Leg, medial foot Motor: Iliopsoas, Quadriceps, Sartorius, Pectineus (below inguinal ligament) Testing Femoral Nerve: = Hip flexion against resistance = Knee extension against resistance = Knee jerk L3L4 = Femoral Stretch Test: Mackiewicz Sign Lesion: Weak Hip flexion and weak knee extension, numbness along anterior thigh and medial calf D/D ;L3L4 raduclopathy, DM amyotrophy
234
l3l4B12 deficiency - SACD-Clinical
similar to Hypocupic Myelopathy due to low calcium levels. Subacute paraesthesias in Hand, feet Loss of position & vibration sense Rhomberg test positive Progressive spastic-ataxic weakness Loss of DTR's + Extensor plantars (LMN + UMN signs mixed) Optic atrophy in advanced Symmetric and diffuse damage Predominant & lateral spino-thalamic and posterior column degeneration Labs: Macrocytosis, Low B12, High Homocysteine levels, High Methyl Malonic acid levels, + Schilling test (dont using radiolabelled B12) Rx = 1000 mg of B12 IM then periodic injections and then oral
235
Hakimov's Triad
Hakimov triad (gait apraxia, urinary incontinence, dementia) in normotensive hydrocephalus (NPH), although it may be a symptom of some other conditions. Apraxia of gait is a unique disorder of locomotion characterized by inability in lifting the feet from the floor despite alternating stepping action (frozen gait), and disequilibrium. Responsible site of lesions are in the frontal lobe and/or the basal ganglia. Gait apraxia is a motor planning deficit and, thus, has a cerebral localization. Patients with gait apraxia have a hard time getting started with walking and may have a “magnetic” or shuffling gait. Gait apraxia is commonly seen in dementia (especially vascular dementia) and in NPH. Apraxia results in a person's inability to carry out a familiar purposeful movement, while in ataxia they can carry out the movement with little coordination.
236
Grading of DTRs
--- Absent (minus sign) + present only with re-inforcement + diminished ++ NORMAL +++ Hyperactive ++ ++ Clonus Inverted Reflex: absent reflex at a level and brisk reflexes below that level: indicates cord lesion at level where its absent.
237
Grading of Muscle Power
05 = normal power 04 = active movement against resistance but not full power 03 = Active movements against gravity but not against resistance 02 = Active movements with gravity eliminated ie side to side only 01 = flicker only 00 = no visible movement
238
Charcot-Marie-Tooth Disease Hereditary Sensory Motor Neuropathy
Autosomal Dominant, PPM22 mutation 1. Demyelinating, or 2. Axonal type gradual progression over decades clinical signs: - Pes cavus, foot drop + High steppage gait, inverted champagne bottle appearance as legs atrophic and thighs hypertrophic, variable stocking sensory loss to vibration/proprioseption - rhomberg + Hand muscle wasting Palpable lateral popliteal nerve @ fibular head Diagnosis - Nerve biopsy Rx - mainly supportive care
239
UMN Signs The nerves in the CNS which carry the impulses for movement are known as upper motor neurons (UMN). The primary tract which carries signals for voluntary movement is known as the pyramidal tract. The pyramidal tract divides further into the corticospinal tract and the corticobulbar tract.
Tone - increased, goes onto develop spasticity, clasp-knife spasticity-initial resistance to elbow extension and then suddenly give away, pyramidal pattern of weakness - UL extensors more weak so they develop flexor spasticity and Leg flexors more weak so they remains in extension when spastic. DTR's - brisk, rapid, hyper Hoffman sign - present, flicking middle finger distal phalanx flexes thumb & index finger Plantars - extensor Most common causes of UMN lesions stroke. multiple sclerosis (MS) B12 vitamin deficiency. Brown-Sequard syndrome (spinal hemiplegia) trauma to the brain or spinal cord. tumors. severe brain infection. dementia.
240
Motor Neuron Disorders:
Acute: Poliomyelitis Herpes zoster Coxsackie virus Chronic: UMN + LMN both - ALS UMN mainly - Primary LS LMN mainly: - multifocal motor neuropathy with conduction block - motor neuropathy with cancer/paraproteinemia - motor predominant + peripheral neuropathy Other: MND with other neurodegeneratives Secondary motor neuron disorders
241
Multiple system Atrophy (MSA) Shy Drager syndrome
Multiple system atrophy (MSA) is a rare condition of the nervous system that causes gradual damage to nerve cells in the brain. This affects balance, movement and the autonomic nervous system, which controls several basic functions, such as breathing, digestion and bladder control. can be of 4 types 1. Strianigral degeneration 2. Olivopontocerebellar degeneration 3. MND with Parkinsonism 4. Shy Dragger syndrome = Postural Hypertension = Cerebellar ataxia = Bladder incontinence = Parkinsonian symptoms-bradykinesia, rigidity, tremor = Dystonias = Constipation, Cold hand/Feet, Depression, dementia etc
242
Toxins/Drugs causing Parkinsonism
Manganese Carbon monoxide chronic exposure MPTP ((1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine) Manganese Rotenone, an insecticide Cyanide exposure Alfa methyl dopa Neuroleptics, Anti emetics Lithium can cause essential tremor Reserpine Fluoxetine Valproic acid
243
M-P-T-P
MPTP - is a chemical precursor to the neurotoxin MPP+, which causes permanent symptoms of Parkinson's disease by destroying dopaminergic neurons in the substantia nigra of the brain) MPTP was a by-product of the synthesis; it has no narcotic activity. The difference in structure between MPPP and meperidine is slight, and their pharmacological effects are similar. However, esterolysis of MPPP produces a compound that can undergo dehydration to form MPTP. While MPTP itself has no psychoactive effects, the compound may be accidentally produced during the manufacture of MPPP, a synthetic opioid drug with effects similar to those of morphine and pethidine (meperidine). The Parkinson-inducing effects of MPTP were first discovered following accidental injection as a result of contaminated MPPP.
244
Manganism, due to Manganese
Manganese overexposure is most frequently associated with manganism, a rare neurological disorder associated with excessive manganese ingestion or inhalation. Historically, persons employed in the production or processing of manganese alloys have been at risk for developing manganism; however, current health and safety regulations protect workers in developed nations. The disorder was first described in 1837 by British academic John Couper, who studied two patients who were manganese grinders. Manganism is a biphasic disorder. In its early stages, an intoxicated person may experience depression, mood swings, compulsive behaviors, and psychosis. Early neurological symptoms give way to late-stage manganism, which resembles Parkinson's disease. Symptoms include weakness, monotone and slowed speech, an expressionless face, tremor, forward-leaning gait, inability to walk backwards without falling, rigidity, and general problems with dexterity, gait and balance. Unlike Parkinson's disease, manganism is not associated with loss of the sense of smell and patients are typically unresponsive to treatment with L-DOPA. Symptoms of late-stage manganism become more severe over time even if the source of exposure is removed and brain manganese levels return to normal.
245
Superior Orbital Fissure Syndrome
Superior Orbital Fissure Syndrome: SOFS may involve any of the structures coursing through the superior orbital fissure anterior to the orbital apex which include CNIII, CNIV, CNV1, CNVI, and the ophthalmic veins. While superior orbital fissure syndrome may manifest with dysfunction of multiple oculomotor cranial neuropathies, it is dysfunction of the optic nerve function that differentiates orbital apex syndrome from SOFS.
246
Transient Global amnesia
Short term memory loss: appears confused to due to memory loss of recent ones but long term memories are well preserved. Can tell long term stuff. Asks repeated questions as what is time/place/date etc as forgets. Lasts around 1-8 hrs and recovered within 24 Hrs which he forgets (day lost) Usually doesnt recur again. Association with DM, HTN, Migraine Suspected Hippocampal ischemia Rule out - Hypoglycemia, TIA, Stroke, etc
247
Supportive Criteria for Primary Parkinson's disease
- unilateral onset (asymmentrical) - Persistent asymmentry - Resting tremor (not intentional) - Progressive symptoms - Good response to Levo DOPA - Levo DOPA induced chorea after about 10 yrs of therapy - Response to L-DOPA > 5 yrs at least - Clinical course > 10 yrs
248
Exclusion Criteria for Parkinson's disease if present - suspect secondary cause
- Repeated strokes + stepwise progress - Repeat Head injury as in Boxers - on AntiPsychotics - on Dopamine depleting drugs (Olanzapine, Quetiapine, Risperidone) - Encephalitis or Oculogyric crisis while on no treatment - More than 1 affected relative - Sustained remissions, early severe demendia with praxis (loss of skilled functions) - Negative response to large levo-DOPA - Strictly unilateral after 3 yrs - Exposure to known neurotoxin (?mptp) - Communicating Hydrocephalus + Plus symptoms: - Supranuclear Gaze palsy, Autonomic disturbance, Cerebellar ataxia, Postural Hypotension
249
Prevost Sign
conjugate eye and head deviation to side of stroke at rest. Example - in a right MCA/ACA infarct stroke -disrupted signals to left eye with normal signals to right eye causes as imbalance in neural tone that causes RiGHT EYE to move to right at rest due to no counter pull from left and left eye follows RE due to intact MLF mechanism between 3/6 nerve nucleus. So both eyes deviates to side of stroke.
250
Top of Basilar Syndrome
distal end - top of basilar artery OCCLUSION= affects Upper cerebellum, Midbrain, Thalamus, PCA territories. = Somnolence = No new memory formation = Small pupils = Vertical gaze palsy: cannot look up (3rd nerve partially affected as in midbrain lesion)
251
PCA infarct
Single PCA infarct: affects midbrain, Occipital lobes, visual cortex, Posterior temporal lobe and thalamus. = contralateral hemiplegia = contra Limb - Motor, Sensory loss = Left side - reading, naming, color affected = Right side: Hemineglect to left Double PCA occlusion: Cortical blindness or bilateral visual field defects. most common causes are still atherosclerosis, embolism, and small artery disease.
252
Cerebello-Pontine angle Lesion
Affects- 5, 7, 8 cranial nerves Common causes - acoustic neuroma, meningioma Clinical: - Loss of corneal relfex, keratitis risk - Facial numbness, weak jaw (5th) - Facial UMN Palsy, same side - loss of anterior tongue tase - Hearing loss, tinnitus - Vertigo - Cerebellar dysfunction - Trigeminal neuropathy symptoms
253
Cerebral Cavernous Malformation, Familial type
Familial CCMs are inherited through a mutation in one of these genes: KRIT1, also called CCM1. CCM2. PDCD10, also called CCM3. AVMs are high-flow lesions with rapid blood movement, while CCM lesions are low-flow lesions. The low-flow nature of CCMs makes them angiographically occult and they are only reliably visually detectable using magnetic resonance imaging (MRI). Cerebral cavernous malformations are removed in a surgery called a craniotomy.
254
Syncope analysis
True syncope - transient global hypoperfusion: complete transient LOC with rapid spontaneous recovery with no further sequelae or damage. 3 key questions: 1. It is syncopal episode or not? - qualifies above definition. 2. Has the aetiological diagnosis been determined? 3. Is there data suggestive of high risk cardiovascular event or death? Red flags: - ECG abnormality - Heart failure - Transient lOC or exertion - Family H/o sudden death < 40 yr old - Family H/o inherited cardiac diseases - Age > 65 yrs with no prodrome ie sudden - a heart murmur - new or unexplained breathlessness Get info from Bystanders, paramedic Eyes usually open during vasovagal/Epilepsy but closed in functional attack
255
Neuromuscular causes of Ophthalmoplegia
Peripheral neuropathy: GBS, MFS Neuromuscular Disorders: = Botulism = Lambert Eaton myasthenia syn = Congenital myasthenia = Myasthenia gravis Myopathic causes: = Mitrochondrial myopathy: Kearns-Sayre syndrome, CPEO, Oculopharyngeal, Myotonic dystrophy (ptosis), congenital myopathy (Nemaline, Myotubular), Hyperthyroidism, Grave's disease
256
Stroke Mimics
SDH, EDH AVM bleed Normal pressure hydrocephalus Multiple sclerosis Mass lesions: Tumors, Abscess Convulsions: Todd's paralysis Metabolic: Hypoglycemia, Hyponatremia Hemiplegic Migraine Functional Psychological Hemiparesis
257
Genetic screening for: Huntington's: cytosine, adenine, and guanine (CAG) to repeat Myotonic dystrophy: mutations in the DMPK gene CCTG repeats
No need to rush, routine type Send for genetic counselling Consent is required: confidential Discuss result implications on patients: - family planning, Career Plans, Insurance plans Offer better discussion with Genetic counsellors as he can explain better Family planning options as autosomal dominant: = IVF and ovum or embryo selection or = early prenatal selection by amniocentesis, chorionic villous sampling, = adoption
258
Parkinson's features
3 in Face: Expressionless face, Blink reflex cannot stop blinking with glabellar tap, Drooling at mouth 3 in Hands: Pill Rolling tremor + Resting hands in lap + cog wheel rigidity due to tremor + bradykinesia 3 in Actions: Shuffling festinant gait + micrographia + monotonous speech 3 Plus syndrome: PSP -upgaze palsy Postural Hypotension in MSA Cerebellar signs Dementia
259
Tremors: DoCtoR Wilson HElP
D - drugs C- Caffeine, Cerebellar Renal - uremic Wilson's disease: copper accumulates H - Hepatic encephalopathy, hyperthyroidism E - Essential tremors - improves with alcohol, parkinsons P - Parkinsonism: pill rolling, Resting
260
Myotonic Dystrotphy
Autosomal dominan, Mutation in DMPK gene causing CCTG repeats = myotonic repeats, sad long = frontal balding = Bilateral ptosis = reduced facial expressions = Testicular atrophy = Foot drop with High stepping gait = Hand grip myotonia = Percussion myotonia = close eyes tightly, cant open fast = LIMB WASTING, sensations intact = Slurred speech Cataracts early Pacemakers need Cardiomyopathy, Heart blocks Glucosuria, DM Family history
261
Normal Pressure Hydrocephalus
Triad of Dementia + Urinary incontinence + Ataxic gait Gait: glued to one place (frozen), unstable posture, tendency to fall back (retropulsion) Upper limb apraxia, Mouth apraxia Dementia: late sign Do CT/ MRI Remove CSF 30-50 ml initially then do cognition check: = Walking Test = Reaction time Test = Memory Test: MMSE
262
Dysphonia
impaired voice production or phonation due to lesions of vocal cord or larynx or vagal nerve Step 01: Ask to cough: = cough normal and speech altered = Local laryngeal lesion = Bovine cough - Vocal cord palsy Step 02: Ask to sa long eeeee: should say it without up/down if unable to - abnormal = Monotonous: Parkinson's (weak bradykinetic muscles = Laboured: Bulbar weakness Ortner Phenomenon: Recurrent laryngeal nerve compression by enlarged Left atrium (LA) causing hoarse voice.
263
Nystagmus Analysis
Central cause: - Demyelination, stroke - may be towards side of lesions - vertical or rotatory - Vertigo rare - Cerebellar signs may be present - doesnt improve with rest - slow recovery Peripheral causes: - vestibular neuronitis - fast phase away from lesion - vertigo common - tinnitus common - vomiting common - improves with rest - quick recovery due to central adaptation
264
Dysphasia analysis
confirm dysphasia: 1. Can you tell me ur name and DOB 2. Receptive check: Close your eyes - then touch your left ear with right hand - then touch your nose (3 steps) 3. Motor check: tell me a little about where you live? if cannot do both above - GLOBAL type aphasia 4. Nominal check: Name some common objects - show 3-4 common objects like key, pen etc 5. Word finding ability: Name as many animals as you can 6. Repetition: Ask to repeat: " A giraffe is a tall graceful animal" 7. Ask to read some thing, write some thing.
265
Dysphasia Types
Definition: a disorder of language with inability to understand or find words. Wernicke Aphasia: Lesion in sensory ie parietal cortex: cannot understand language but speaks fluently, glibly meaningless jargon (sensory, receptive) Broca's Aphasia: (Lesion in motor ie frontal lobe) understands words, commands but unable to express and highly frustrated (Motor, Expressive) Conductive Aphasia: intact comprehension and expression but repetition is lost Trans-cortical Sensory Dysphasia: same as sensory type but repetition is preserved, lesion in parieto-occipital concept area Trans-cortical Motor Dysphasia: same as motor but repetition preserved, incomplete lesion in Broca's area Nominal Dysphasia: cannot name objects, angular gyrus lesion.
266
Causes 3,4, 6 cranials lesions
Brainstem Level: Vascular strokes Demyelination MS Compression by SOL, Tumor Skull Base: Infections: Bacterial, Fungal, TB Carcinomatous meningitis Neurosarcoid CNS Lymphoma Bulbar palsy - 10, 11, 12 mostly PCA aneurysm - painful 3rd palsy Temporal Lobe ICH Petrous temporal tumor in Posterior fossa stretches 6th nerve Cavernous Sinus: Thrombosis, Sepsis, Carotid aneurysm, Wegner's
267
Spinal Cord Lesions - Levels
T2-L1: all lower limb muscles spastic T8-T12: Abdominal superficial and tickling lost Hemisection: CST cut - ipsilateral UMN weakness DC cut - ipsilateral position, vibration lost STT - contralateral pain, temp lost Central Cord Lesion: syrinx Bilateral loss of pain and temp below level of lesion and other modalities preserved - risk of finger burns, charcot Joints Dorsal Columns: affected in tabes dorsalis, long standing syphilis with loss of position and vibration senses, deep pain on pressing achilles tendon lost Anterior Spinal infarct, (ASA): Bilateral loss of pain and temperature below lesion. Preserved position and vibration.
268
Post Spinal Cord trauma problems
High Quadriplegia:, C1-C4 needs respiratory support completely dependant Low Quadriplegia, C5-C8 partial dependancy Paraplegia, below T1 Autonomic dysreflexia, above T6 Headache Palpitations Hypertension and tachycardia Quadriplegic fever: Recurrent fever with no other cause Bladder Troubles: Detrusor spasticity (Rx Oxybutinin) Urinary dyssynnergia: retention when emptying: Rx with Terazocin Bladder areflexia Other issues: DVT Pulmonary embolism Decubitus ulcers Spasticity Rx: stretching exercises, Baclofen, Tizanidine, Dantrolene, Botulism, Rhizotomy
269
Lumbo-Sacral Lesions
Upper Lumbo-sacral Lesions: L2L3L4, femoral, Lat femoral = weak thigh flexion-adduction = weak knee extension = Hip in extension + Knee flexed = Sensory Loss: Ant thigh, Medial leg = DTR's: absent knee jerk Causes: Diabetic amyotrophy, abdominal Surgery, Lumbo-Sacral plexitis Lower Lumbo-sacral Lesions L4L5S1S2, sciatic nerve weak thigh extension Weak knee flexion Weak foot dorsiflexion Weak Plantar flexion also Position: Foot drop, Knee extended Sensory Loss: Posterior thigh, lateral Leg and all of foot DTR's : absent ankle jerk Causes: Plexitis, Surgical; trauma, Cancer, Radiation, infiltrative
270
LMN Sign
lesion of peripheral nerves beyond anterior horn cells: Wasting - poor mass Weakness - reduced tone & power Hypotonia Hyporeflexia Sensory loss
271
Benign Intracranial Hypertension (PseudoTumor Cerebri)
Cause of secondary head ache Usual Case - young woman with obesity in her 30's. Migraine like head ache with diplopia, pulsatile tinnitus, loss of peripheral vision (tunneling) Association with MS, SLE, OC pills, Cerebral sinus thrombosis in pregnancy. (need to exclude those) Symptoms can include: Headaches. Tinnitus (ringing in the ears) Temporary blindness. Double vision. Blind spots. Neck and shoulder pain. Peripheral (side) vision loss. The diagnosis is based on the triad of: (1) papilledema, (2) elevated intracranial pressure with a normal cerebrospinal fluid constituency, and (3) normal central nervous system imaging studies. Treatment involves various maneuvers to lower the intracranial pressure, including medicine and surgery.
272
Orbital Apex Syndrome
Vision loss from optic neuropathy + ophthalmoplegia Causes can be: 1. Inflammatory = Sarcoidosis, SLE, Churg-Strauss syndrome, Granulomatosis with polyangiitis , Tolosa Hunt Syndrome, Giant cell arteritis, Orbital inflammatory pseudotumor, thyroid orbitopathy, IgG4 related orbital myositis 2. Infectious – invasive fungal sinusitis or post-septal bacterial orbital cellulitis, aggressive bacterial sinusitis without cellulitis causing an OAS. Fungi: aspergillosis, Mucormycosis Bacteria: streptococcus, staphylococcus, actinomyces, gram-negative bacilli, anaerobes, mycobacterium tuberculosis Spirochetes: treponema pallidum Viruses: Herpes Zoster 3. Neoplastic = nasopharyngeal carcinoma with extension into the orbit, Primary orbital adenoid cystic carcinoma with extension to the orbital apex Neural tumors: neurofibroma, meningioma, glioma, ciliary neurinoma, schwannoma Metastatic lesions: lung, breast, renal cell, melanoma 4. Hematologic: Burkitt lymphoma, non-Hodgkin lymphoma, leukemia Perineural invasion of cutaneous malignancy: squamous cell cancer 5. Iatrogenic/Traumatic –Direct injury typically results from displaced bony fragments leading to anatomic compression of the contiguous nerves and vessels. Indirect injuries result from shearing forces to the anatomic structures of the superior orbital fissure and/or optic nerve from high impact forces. Iatrogenic: sinonasal surgery, orbital/facial surgery Traumatic: penetrating, nonpenetrating, orbital apex fracture 6. Vascular = Carotid cavernous aneurysm, carotid cavernous fistula, cavernous sinus thrombosis 7. Other: Mucocele, fibrous dysplasia, neurofibromatosis
273
Lumbo-Sacral root Pain areas
L3L4 - anterior thigh L4L5 - Lateral leg, dorsal foot, great toe L5S1 - posterior calf, heel, little toe Sciatica - posterior buttocks, thigh Cauda equina - bilateral thigh-legs
274
Superficial reflexes
Cremasteric L1L2 Anal wink - S4S5 Abdominals around T10 Plantars - S1 tibial nerve Enmass reflex: S1 sensory L5 motor - incontinence on flexion of hip and knee Corneal Reflex - afferent Cranial 5(Trigeminal) and motor cranial 7 (facial)
275
Peripheral Neuropathy: Common causes
A - Alcohol B - B12 deficiency, Vegan C - Cancers, CTDs D - Drugs, DM E - Encephalopathy Hereditary -CMTd
276
Parkinsonism: Red Flags
suggestive of some thing more than just parkinsons disease Symmetrical symptoms Lower Body involvement Absent/Poor response to L-DOPA Absence of dyskinesias or fluctuant symptoms after long use of L-DOPA Early postural instability and falls (suspect Shy Drager syndrome) Early Autonomic disturbance (Sexual, Bowel, bladder, postural hypotension) Early pre dominant Upgaze restriction Early dementia Early non drug related Hallucinations (suspect Lewy body dementia) Associated jerks, myoclonus (suspect Multisystem Atrophy)
277
Sensory neglect: Inattention
Intact cortical vision but when right parietal lobe is damaged patient ignores left side of body which is paralysed and not recognised as existing. if told to raise left side - will not do it. Needs rehabilitation attempts to stimulate left side movements for example - talk to patient from his left side only
278
Mirror Agnosia: Looking Glass Syndrome
Patients with sensor neglect due to damage to right parietal lobe when a mirror is placed on their right = showing them there left side body - tries to reach an object through the mirror but not directly if placed on left side as totally neglects left side.
279
ABCD2 score
prognostic score for TIA patients predicts their risk of stroke in near future A, Age > 60 = 1 point B, BP > 140/90 = 1 point C, clinical weakness, dysarthria without weakness = 1 point D, Duration of TIA > 60 min = 2 points and if 10-59 minutes = 1 point D, Diabetic = 1 point > 4 points = Do CT/ MRI, Admit, Carotid endarterectomy < 2 weeks
280
Tremor Types
by Amplitude: Coarse vs Fine by Frequency: waveforms per second (Hz) by Anatomy: affected part & symmetry - head, hand, tongue by voluntary: - Resting - Action: Intention, Postural - Others: Holmes, Dystonic, Primary Orthostatic
281
Secondary Parkinsonism
Vascular parkinsonism Drug - MPTP, Manganese Structural lesions Huntington's chorea Wilson's disease Neuroacanthocytosis
282
Achromotopsia
cortical color blindness due to damage to V4 area, visual in parietal lobe bilaterally, things looks gray MT area Lesion: middle temporal lobe visual area is involved in sensing movements and in damage here, person might be terrified to cross street but can read books, can recognize faces and see color
283
Blind Sight
condition with blindness in one visual field due to damage to visual cortex in another cortex. Person cannot see that side but if asked to touch with finger in the field, can do so.
284
Anosagnosia
denial of left paralysis when right parietal lobe is damaged, sensory cortex
285
Sensory Neuropathy: Causes
Central Lesions: = Multiple sclerosis = Myelopathy = Stroke, TIA = Epilepsy = Tumors Peripheral Lesions: = mononeuropathy, Ex median nerve = Mononeuritis multiplex = Polyneuropathy = Radiculopathy Other causes: = Migraine with aura = Panic attacks = Hypoglycemia
286
Stroke MIMICs
Seizures with Todd's palsy Delirium Flare up of old stroke Syncope Tumor with acute presentation Psychiatric-Functional-Conversion Alcohol or Drug related symptom Hypoglycemia
287
Binocular Diplopia
Orbital causes: Acute orbitopathy, fractures, cellulitis, tumors Muscle lesions: MG, Orbital myositis, Kearns Sayre syndrome (CPEO+RP+Young age 20s) NMJ lesions - MG, Botulism Nerve Lesions: 3,4, 6 palsies, Orbital apex syndrome, Cavernous sinus syndrome, Multiple Cranial palsies as in meningitis, Wernickes ophthalmoplegia, Miller Fisher Syndrome variant of GBS Brain Lesions: Internuclear ophthalmoplegia in MS, Stroke, Tumors etc Skew deviation: brainstem lesions Other causes: Decompensated phoria (heterophoria) Childhood strabismus with failed compensation
288
Stroke BP Management
When to lower Blood pressure: - ICH with BP > 200, MAP > 110 - Hypertensive encephalopathy - CKD with stroke with High BP - Stroke with Heart failure - Aortic dissection - Pre eclampsia Use: Labetolol, GTN, Nitroprusside
289
Secondary Prevention measures for Stroke
ACE inhibitors for BP control Antiplatelets Anticoagulants in Afib Carotid endarterctomy if > 70% Statins Life style changes
290
ICH management
Reverse anticoagulation: Factor 8 concentrates, Vitamin K, Stopd Drug, FFP Avoid vasidilators Anti epileptics but not routine Protect ABC Surgical approach if: = Cerebellar bleed > 3 cm = Young healthy patient with ICH = Superficial cortical bleed = Lobar ICH with hydrocephalus = Lobar ICH with neurodeficits
291
Cord versus Cauda Equina
Cord compression: = Spasticity, Hypertonia, Bilateral pyramidal weakness, Brisk reflexes = Sensory level with loss of all modalities below lesion = Sphincter disturbance = Autonomic features Cauda equina: = hypotonic flaccid = absent DTR's = Dermatomal loss = Saddle numbness = Sensory UB with retention and incontinence
292
Total Anterior Circulation Stroke
TACS: Proximal MCA occlusion Has all 3 of features: 1. contralateral Hemiparesis OR weakness of 2 of 3 - Face/ arms/ Legs UMN features 2. Contralateral Homonymous Hemianopia 3. Higher cortical Function abnormality: Dysphagia with left sided TACS, Dyspraxia, Sensory Hemineglect with right MCA stroke if drowsy but hemiparetic then all 1/2/3 assumed to be present (ACA is protected by anterior communicating artery which feeds ir from other side MCA)
293
Lacunar Strokes (LACS)
Lipohyalinolysis of penetrating lenticulo-striate arteries and can affect anterior, posterior circulation areas , basal ganglion or pons 1. Pure motor stroke: anterior limb of internal capsule 2. Pure Sensory stroke: posterior limb of internal capsule 3. Sensory motor stroke 4. Ataxic Hemiparesis: contralateral hemiparesis with ipsilateral cerebellar signs due to small infarcts in basal ganglion or pons.
294
Partial Anterior Circulation Stroke
due to MCA branch occlusion Has 2 of 3 features 1. contralateral weakness or Arm/Leg/Hand/Face. Monoparesis may be sole manifestation. 2. Higher cortical dysfunction alone as dysphasia, dyspraxia (inattention or neglect) may be sole manifestation MCA upper division lesion = hemianopia is less common MCA lower vision lesions = sensory motor deficits uncommon
295
Caused of ICH
1 Microaneurysm, fibrinoid necrosis leads to Charcot Bouchard aneurysms in cerebral arteries which might bleed 2. Cerebral amyloid angiopathy: elderly, cortical bleed not deep 3. Tumor with bleed inside 4. AV Malformations: Lobar bleed from single AV malformations is common in young people leading to SAH mostly, seen with HHT. A large AVM can cause stroke by stealing blood from other terrotories. 5. Saccular aneurysms in young people, also berry aneurysms common in ADPKD, Tuberous sclerosis 6. rare Causes: Vasculitis, Venous thrombosis, Infective endocarditis, Alcohol, Amphetamines, Cocaine, Sympathomimetics, anticoagulations, Thrombolysis 7 Traumatic
296
Imaging for Stroke - for vessles + Parenchyma and to see reversible ischemia
1. Vascular imaging: CT angio to see occlusion as hyperdense or white vessel, not commonly done for strokes MIMICS - High Hematocrit, Microcalcification, Hypodence parenchyma might make vessel falsly dense 2. Imaging acute Parenchymal Infarctions: MR diffusion weighted imaging within 1 hr : CSF looks black in T1 weighted MRI and white in T2 weighted MRI. Infarct looks white in both. CT will show: Loss of white/gray differentiation, Obscuration of deep nuclei, Loss of insular ribbon 3. Imaging Reversible Ischemia: DWI or perfusion MRI
297
Red flags with Unsteady Gait
Spinal cord signs Stroke Guillaine Barre syndrome Normal pressure Hydrocephalus
298
Spastic Gait
Pattern: stiff knees with hyperadducted hips close to body, mild plantar flexion at ankles and walks like scissors seen with UMN damage: = Multiple sclerosis = Cerebral palsy = Transverse myelitis = Motor neuron disease = Cord compression = Syringomyelia = Anterior spinal artery syndrome = Hereditary spastic paraparesis = Parasagital meningioma = Bilateral cerebral infarcts = Friedrich ataxia = B1 B12 Deficiency
299
Spastic Parapresis
walking through mud like gait bilateral UMN lesions required UMN Signs: Bilateral Hypertonia, Hyperreflexia, ankle clonus, extensor plantars - symmetry suggests a complete lesion, Sensory level will localize lesion - UMN signs in Hand means lesion is above C8 LEVEL - Examine cranial nerves for evidence of MS or MND - LMNS signs at level of lesion suggest damage to anterior Horn cells at that level - if LMN signs or widespread, consider MND (hypotonic rather than spastic) Mixed signs seen in - cord compression, MND, dural AVM at L1L2
300
Spastic parapresis: Causes
presents as bilateral leg weakness with UMN signs - parasagital meningioma - Degenerative disc disease - Syringomyelia - Intra dural or Extra dural AVM - Spinal AVM - Multiple sclerosis - Sarcoidosis - Anterior spinal artery syndrome - Heredeitary spastic parapresis - HIV, HTLV-1, B12 DEFICIENCY, - Cerebral palsy
301
Evaluation: Acute Neuro-Event
Inquire like this: 1. Before Event: Doing what, mechanism, past events, any other triggers 2. Suring Event: Any sugar levels checked? Any incontinence? stool more serious Ask as Bed wetting and bed soiling Ask about any change of face color as went pale(vasovagal) ot went blue(CNS as epilepsy) 3. Recovery: After event: How did you recover? Any Sugar given, CPR done, Spontaneous? Duration of full recovery, short, long? 4. Medication history to take including recreational drugs, OTC, Herbals, Drining 5. Family history to ask as well
302
Pure Motor Neuropathy
Pure motor Neuropathy: causes = GBS, CIDP, Multi focal = Brachial neuropathy = Diabetic Lumbosacral Radiculoplexus neuropathy = LMN form of ALS = Lambert-Eaton myasthenic syn = Tick bite paralysis = Poliomyelitis = Drugs: Dapsone, Lead, = Acute intermittent porphyria associated = Diphtheria Motor Neuropathy
303
Pure Sensory Neuropathy
= Friedrich ataxia = Idiopathic sensory neuropathy = Sjogren's associated = B12 deficiency neuropathy = Pyridoxin toxicity = Cisplatin neuropathy = Hereditary sensory neuropathy = Lepromatous leprosy = DM small fiber neuropathy = Fabry's disease = Vacor associated
304
Alzeimer's disease
neurodegenerative disease with progressive cerebral atrophy characterized histopathologically by NEUROFIBRILLARY TANGLES containing TAU protein and BETA AMYLOID plaques in brain matter with apolipoprotein isomer APO-E4 in > 60% above 65 yr old. = pathology progresses from back to front in brain to memory decline occurs before personality change = Initial atrophy occurs in Hippocampus, medial temporal lobe
305
CADASIL: Cerebral Autosomal dominant arteriopathy with Subcortical infarcts and Leucoencephalopathy
CADASIL: multiple small lacunar infarcts in basal ganglion often with diffuse white matter lesions, mostly caused by: = Hypertensive microangiopathy = Low perfusion = CADASIL or = amyloid angiopathy of chronic systemic diseases as RA
306
Creutzfield-Jacob disease (Transmissible spongiform Encephalopathy)
Mutation of Prior prP genes of chromosome 20 turns them into transmissible and change leads to plaque formations. 1. Sporadic type: 85% 2. Inherited, Familial, Auto Dom 3. Acquired CJD: use of cadaveric Growth hormone, consumption of affected peoples brain (Kuru) 4. New variant CJD Features: Rapid dementia within 2 yrs + myoclonus + Ataxia + Visual disturbances, early death
307
Charles Bonnet Syndrome
Syndrome of new visual hallucinations in the absence of delirium, dementia or neuropsychiatric disorder - Mean age of onset 57 yrs - Hallucination are usually complex, detailed like dwarves wearing silly hats, bright clothing and in occurs broad daylight (no sundowning feature) - common in macular degeneration.
308
Encephalopathies causing delirium
MISTIV: 1. Metabolic: Alcohol, drugs, NH3, CO2, O2, Uremia 2. Inflammatory: Lupus, Sarcoid, Behcets, MS 3. Structural: Hydrocephalus 4. Tumors: Primary SOLs, Mets, paraneoplastic( limbic encephalitis) 5. Infections: HSV, HIV, Lyme, CJD 6. Vascular: Venous sinus thrombosis, Vasculitis, SDH, CADASIL
309
Delirium: VIIT-D-PM
VIIIT-DPM: Vascular- HTN Infections: UTI, Pneumonia Infarctions: STEMI, Strokes Impactions: U retention, constipation Traumatic: Head injury edema Tumors: SOL, Metastasis Drugs: Alcholol, sedatives Post ictal Metabolic, Hyponatremia, uremia, Ammonia, myxedema, Hypercalcemia
310
AMTS: Abbreviated Mental Test Score
Age Time of day Year Name of place Recognize 2 people as dr, nurse Name Ruler of country Count back from 20 Five minute recall - an address
311
Neurological causes of Ataxia
Frontal lobe disease Normal pressure Hydrocephalus Dementia Cerebellar ataxia Spasticity-Hemiparesis, paraplegia Peripheral sensory Ataxia: Sensory, Visual or Vestibular cause Peripheral Motor: Myopathy, motor neuropathy
312
Non-neurological causes Ataxia
Musculo-skeletal: arthritis Intermittent claudication Postural hypotension Fear of falling Alcohol effect Medications - sedatives, Antidepressants, Antipsychotics, Phenytoin
313
Chronic Headache: MOIST-CO
MORE THAN 8 WEEKS headache M: Medication overuse O: OSA associated I: Idiopathic intracranial HTN S: SOL: Cancers, Prolactinoma, Mets T: Tension Headache Co: CO poisoning (wakes up flushed, other roommates affected)
314
Cluster Headache
primary type headache also called "Histamine Headache" - ? due to inhibition of nociceptive pathways in hypothalamus Smoker 80% cases, 50% Auto-dom May H/o head injury/ Alcoholism Lasts minutes or Hours, multiple attacks of headache always unilateral, retro orbital associated redness eye + tearing associated runny nose, sweating, associated ptosis can be- episodic or chronic Abortive Rx: = O2 15 Lit/min for 15 min = Triptans (contra if IHD, Stroke) = Ergots Preventive Rx: = Calcium channel blockers = Anticonvulsants = Galacanezumab/ month
315
Ischemic stroke: Uncommon causes
Carotid-vertebral dissection Cerebral vasculitis Giant cell arteritis Bacterial endocarditis Thrombophilia, young pts Migraine Patent foramen ovale Hereditary: Fabry's disease Fibro muscular dysplasia, young pt with reno vascular HTN + involves ICA, angio shows beads of strings appearance of artery
316
Posterior Circulation stroke
Vertebro-basilar, Brainstem Dizziness, Vertigo, Headache, Diplopia, Loss of vision, Ataxia, Limb Numbness, Limb weakness Ocular palsies Oro-pharyngeal dysfunction
317
Stroke Thrombolysis: Exclusions
exclude from thrombolysis if: = Age < 18 or > 80 = Time window > 4.5 Hrs = GTCS on arrival = GTCS within 6 months = LP within one week = Arterial puncture, non compressible = GIT or GU bleeding < 21 days = Head injury, stroke, < 3 months = Past IHC, AVM known, aneurysms Other possible exclusions: = Comatose = Hemiplegia with Fixed head, eye deviation = Minor symptoms = Rapidly improving symptoms = Sx: SAH, even if CT normal = HTN Bp> 185, DBP > 110 = INR > 1.7, aPTT > 1.2 = Glucose < 2.8 > 22 mmols = Platelet count < 100k CT shows ICH or SOL
318
FAST tool for stroke recognition
Facial weakness Arm weakness Speech disturbance Test all 3 present
319
Dementia with Lewy bodies
1. Fluctuating cognition: reduced attention and reduced alertness, past H/o recurrent UTI or pneumonias 2. Recurrent visual hallucination of animals, people 3. Sun Downing phenomenon: twilight visits of children, cats as hallucination 4. Parkinsonism: bradykinesia, rigidity, festinant gait, monotonus speech, paucity of expression, micrographia Antipsychotics are contra indicated
320
Vascular dementias
1. Classic multi infarct Vascular dementias - large vessel territories 2. Micro-Angiopathic small Vessel disease: = A. Strategic infarct Dementia: Thalamic, Temporal, Hippocampal = B. Sub-cortical Arteriosclerotic LeucoEncephalopathy (Binswangers disease) = C. Multilacunar state = D. Mixed cortical-Subcortical = E. Granular cortical Atrophy 3. Post Ischemic Encephalopathy: CPR 4. Cerebral Haemorrhage sequalae 5. cerebroVascular+Alzeimer's: Mixed
321
Delirium Causes
Age < 65 yrs: = illicit drug use = illicit drug withdrawal = Meningitis = Encephalitis = HIV-AIDS Age > 65 yrs: = Polypharmacy = Stroke = SDH = UTI, Pneumonia = Urinary retention = Fecal impaction All age groups: = Electrolyte disturbance = Hepatic encephalopathy = Uremic encephalopathy = Thyroid associated: Hypo/Hyper = CNS tumors, Mets = MI, PE associated = Delirium tremens (alcoholics) = Wernicke's encephalopathy
322
Proptosis: Causes
Grave's disease Cavernous sinus thrombosis Carotid Cavernous fistula Orbital cellulitis Retro Orbital tumors: Lymphoma, leukemia, Meningioma, Metastasis Retro Orbital granuloma: Wegner's, Histiocytosis-X, Sarcoidosis Traumatic hematoma
323
Bamford Stroke Classification
TACS: Total Anterior Circulation Stroke, MCA-ACA, needs to have all features: Hemiplegia + Homonymous Hemianopia + HIgher cortical dysfunction as Dysphasia, Dyspraxia, Neglect PACS Partial Anterior circulation stroke, branch of MCA occluded, 2/3 as above LACS Lacunar Anterior circulation strokes pure motor/ sensory or mixed but small deficit as only hand weakness
324
Partial Motor Seizures
no LOC if simple partial Jacksonian March: seizure starts in fingers then marches up along limb Todd's paralysis: Localaized palsy after convulsions, recovers completely later Epilepsia Partialis continua - may continue to have partial seizures for Hours. Repetitive flexion/extension movements ie clonic Frequency: 2-3 Hz in EEG Pure tonic posturing sometimes seen Hand movements + Facial movements (Twitches & fasciculations) EEG can be normal if focus is deep seated in brain
325
Intermittent Myopathic weakness
Evaluation:Check Myoglobinuria If Present: clinically normal between attacks Proximal >> distal weakness Do Forearm Exercise Test: Lactate *Normal Lactate - CPT deficiency *Low Lactate - Glycolytic defects Do muscle Biopsy -------------------------- No Myoglobinuria: Weakness variable, Ophthalmoplegia +, Ptosis +, Bulbar weakness + Limb weakness + Do Repetitive NCV test Do Ach receptor antibody testing - Acquired Myasthenia gravis - congenital MG ------------------------ No Myoglobinuria: Normal between attacks ie no weak Proximal >> distal Paradoxical MYOTONIA on examine Normal K = Hyperkalemic periodic paralysis Low K = Hypokalemic periodic paralysis
326
Persistent Weakness Evaluation
Look for pattern of weakness Proximal> Distal = Polymyositis, Dermatomyositis, MD's Ptosis, Ophthalmoplegia = Oculopharyngeal MD, Mitochondrial myopathy, Myotubular myopathy Facio-Scapulo-Humural MD, winging Facial+Distal quadriceps weakness +Hand Grip myotonia = Myotonic MD Proximal weakness + Hand grip myotonia + Quadriceps weakness = Inclusion body myopathy (IBM) Distal weakness only = distal myopathy Droopy head - MG, PM, ALS Myopathic EMG confirms muscle disease, Repetitive NCV for MG and other tests as CK levels, DNA testing, Muscle biopsy to do
327
Muscle Tone assessment
Normal, Hypo or Hypertonic Rigidity & Spasticity- Hypertonia Clasp-Knife Spasticity: if resistance increases when you are moving a joint then suddenly gives away. Example- Heel goes off bed when suddenly flexed passively OR passive flexion at elbow initially resisted then gives way. Lead Pipe Rigidity: tone is rigid throughout movement seen in Parkinson's disease Cog Wheel Rigidity: jerky irregular breaking movements like a cog wheel, best assessed by slow movements, seen in Parkinsons Paratonia-Gegenhalten: refers to resistance due to frontal lobe damage, involuntary resistance to passive limb movements, increases with degree of dementia
328
AEIOU-THRIPS: Delirium causes
Definitiion: acute confusional status of sudden onset with diurnal fluctuation, inattention, reduces focus, disorganized thinking, cloudy consciousness. Hypoactive or seen restless plucking at bed sheets. A- Alcohol E- Epilepsy I- Insulin ie Hypo/Hyperglycemia O- Oxygen ie hypoxia U- Uremia T- Trauma SAH, SDH, EDH H- Heart issues: CCF, Arrhythmias R- Retention of Urine I- Infections, UTI, Pneumonias P- Poisons: Oversodes, Sedatives S- SLE cerebral vasculitis, Autoimmun
329
Dementia
Impairment of memory to new learnings with any one of = Aphasia, Agnosia, apraxia or Executive function Irreversible Causes of Dementia: = Alzeimer's disease = Vascular dementia = Fronto-temporal Dementia = Dementia with Lewy bodies Potentially Reversible Causes: VVIT-A-Hypothyroidism V- Vasculitis V - Vitamin B12 deficiency I - Infections: HIV, Syphilis T - Tumors, frontal lobe SOL A - Alcohol excess H - Hypothyroidism H - Hydrocephalus (+_ depression)
330
Genetics of MND
5-10 % cases hereditary/ Genetical 1. UMN + LMN MND: AutoDom - Superoxide dismutase mutation AutoRecessive - Alsin mutation Mitochondrial: Cytochrome oxidase 2. LMN type MND: Spinal muscular atrophies X Linked Spinobulbar muscular atrophy GM2 Gangliosidosis: Sandhoff disease, AB variant, Adult Tay-Sach's disease 3. UMN type MND: Selected Familial Spastic paraplegia AutoDom - Spastin AutoRec - Spartin X Linked Adrenomyeloneuropathy 4. ALS Plus Syndromes: ALS with Fronto-temporal dementia ALS with Parkinsonism ALS with Tau protein
331
Seizures: Other Differentials
Syncopes: Vasovagal, Cardiac, situations Psychogenic Seizures, Hyperventilation, Panic attacks Metabolic: Hypoglycemia, Hyponatremia, Hypernatremia, Hypoxia Drugs: Hallucinogens, Ethanol withdrawal Migraine: confusional, Basilar TIA of basilar artery Sleep Disorders: Narcolepsy, Cataplexy, Benign sleep myoclonus Movement Disorders: Tics, Non Epileptic myoclonus, Choreoathetosis Pediatric Group: Breath Holding spells, BPPV, Migraine with recurrent tummy pain, Vertigo, Apneic episodes, Night tremors, Sleep walking
332
Medial Temporal Lobe Epilepsy
Febrile Seizures - familial - Early onset - Remit and reappear, Aura common, behavior arrest, stare, complex automatisms, Unilateral posturing, Post ictal confusion, Memory loss of event # Work up: EEG-Anterior temporal spikes (Uni/Bi) PET scan - hypometabolism in inter ictal phase SPECT - hypoperfusion interictal WADA testing: intracranial amo-barbital-material specific memory deficits (tests each side of brain differently for memory issues) MRI scan: small hippocampus and temporal lobe, enlarged temporal horn Pathology: Highly selective loss of specific cells in hippocampus
333
Convulsions in Neonates
in Neonates: = Perinatal Hypoxia = Intracranial bleeding = Trauma to head = Acute CNS infections = Metabolic: Hypocalcemia, Hypoglycemia, Hyponatremia, Hypomagnesemia, pyridoxine deficiency Drug withdrawals - cocaine, Alcohol (mother abusing them)
334
Convulsions in Children
Febrile convulsions Genetic, Hereditary CNS infections Head injury Developmental disorders Idiopathic Drugs induced
335
Convulsions in Young and adults
Tumors Alcohol withdrawal CNS infections Drugs induced-Amphetamines Drug overdoses, Poisoning Hypoxia associated Stroke sequelae Metabolic: Na, Glucose, Ca, Mg, TSH Idiopathic
336
Movement Disorders
Akinetic Rigid (Parkinsonism) Tremors Dystonia Chorea Myoclonus Tics Drugs induced movements disorders Dyskinesia: Drug induced choreas, dystonias, (Metoclopramide) Acute Dystonia: Haloperidol, Metoclopramide, Prochlorperazine, Antipsychotics Tardive Dyskinesia: continuous involuntary movements, due to anti-psychotics (tardive means late occurring, months or yrs after Rx)
337
Epilepsy associated Genes
1. Classic Lissencephaly with Severe mental retardation + Seizures, Xq21-24 2. ADNFLE - AutoDom nocturnal frontal lobe epilepsy, childhood onset, 20q13.2 3. BFNC - Benign familial nocturnal convulsions, AutoDom, KCN62, 20q 13.3, starts first week of life 4. GEFS - Generalized epilepsy with febrile seizures, 19q12.1, SCN1B, AutoDom, febrile convulsions at 1 yr 5. ADPEAF - AutoDom partial epilepsy with auditory features, 10-25 yrs, Lateral temporal lobe involves, LG11, 10q24, AUDITORY APHASIA 6. PME - Progressive myoclonus disease, CSTB, 21q22.3, AutoREC 7. Lafora disease: myoclonic epilepsy, EPM2A, 6q24 early dementia, myoclonus
338
Advice to Epileptics
Stop: = driving, swimming, recreational drug use, Reduce: = Alcohol < 14 U/Wk, late night awake, OC pill might be less effective so take barrier precautions as well If female, planning pregnancy, need to be aware about teratogenic effects and need to plan conception
339
Gait analysis
Reduced arm swing: Corticospinal tracts, Parkinsons Stooped posture, Short, shuffling = Parkinson's Broad based, feet apart, unstable gait = Ataxia - sensory/ cerebellar Scissoring gait, crossed steps = Spastic High stepped, slapping - foot drops due to neuropathy, Posterior column disease Stuck in, Glued to spot - apraxia with frontal lobe disease
340
Genetic Testing
done to diagnose disease or delay or prevent complications. indicated early if symptomatic, bad family history, high risk jobs as pilot, military HOCM = AutoDom, as soon as possible - then ICD insertion = HOCM ECHO positive: repeat ECHO - if repeat also positive - do genetic studies = HOCM ECHO negative - repeat later - if still negative - genetic studies if family history = HOCM ECHO in children: do at 10 yrs then every 3 yrsly upto puberty - then yearly until 18 yr old - if negative by then, every 5 yearly ATAT deficiency: AutoRec, as soon as possible, to prevent or minimize complications of COPD, cirrhosis ADPKD: AutoDom, reoutine after age 20, only USG enough for watch and screening Huntington disease: AutoDom, routine as early diagnosis might be depressive Myotonic dystrophy: AutoDom, routine as clinical diagnosis enough and early diagnosis offers no benefit
341
Headache Differential:
Primary Headaches - Migraine, Tension, Cluster Headache, Secondary Headaches: Meningitis, SAH, Cerebral Sinus thrombosis, Anti-phospho-lipid syndrome, Posterior Circulation Ischaemia, Analgesic Overuse Headache, Sinusitis, Otitis media, Trigeminal neuralgia, SOL Combined Oral contraceptive pills
342
Headache Red Flags
= Headache with head injury – a fall or accident = Sudden headache & extremely painful Severe Headache with: = sudden problems speaking or remembering things = loss of vision = feel drowsy or confused = very high temperature and symptoms of meningitis = the white part of the eye is red a headache with vision problems or difficulty speaking, swallowing, balancing or walking a headache with drowsiness or a persistent lack of energy a headache that starts within 5 days of a head injury
343
Tension Headache
Common symptoms of tension headaches include: = pain on both sides of your head, face or neck = feeling like something is pressing on your head or being tightened around it = the affected area may feel tender and your head may hurt more when touched = You should be able to continue doing daily activities without making the headache worse. Tension headaches last at least 30 minutes but they can last much longer, sometimes for several days. Causes of tension headaches = stress = sleep problems = caffeine = Overuse or rebound headaches. Tension headaches are not a sign of an underlying condition.
344
CNS Radiation Toxicity
Early delayed Radiation Toxicity: < 4 months - somnolence in children, Lhermitte phenomenon after cord radiation Rx is with Glucocorticoids Late Delayed Radiation Toxicity: > 4 months after: Radiation necrosis (low glucose uptake in PET-SPECT, contrast enhancing mass noted MRI Spectroscopy required-high lactate, low choline develops - dementia, hypo-pituitarism, new cancer like lesions but low glucose uptake
345
GBS Subtypes
AIDP: adults 90%, Anti GM1 antibodies+ first attacks schwann cell surface, widespread myeline loss AMAN - Acute Motor Axonal neuropathy: in children, Anti GP1 antibodies, axonal damage starting at nodes or Ranvier (no sensory) AMSAN - Acute Motor-Sensory neuropathy, Adults, axonal damage, sensory loss also seen Miller fischer type: uncommon, adult & children, Ataxia + Ophthalmoplegia + Areflexia. Anti GQ1 antibodies +, demyelination present Rx - IVIG, Plasma pheresis
346
GBS diagnostic Criteria
Must present: = progressive weakness or 2/ more limbs = Areflexia = Less than 4 weeks duration of start Exclude: Vasculitis/ Toxic/ SLE/ Churd strauss Supportive features: - relatively symmetric - Mild sensory mostly motor - facial, lower cranials involved - no fever - CSF: Acellular with High proteins - Demyelination in NCV studie may have Back pain, Hip Pain
347
Tension Headache
Common symptoms of tension headaches include: = pain on both sides of your head, face or neck = feeling like something is pressing on your head or being tightened around it = the affected area may feel tender and your head may hurt more when touched = You should be able to continue doing daily activities without making the headache worse. Tension headaches last at least 30 minutes but they can last much longer, sometimes for several days. Causes of tension headaches = stress = sleep problems = caffeine = Overuse or rebound headaches. Tension headaches are not a sign of an underlying condition.
348
Cluster Headache
quickly and without warning, pain very severe, sharp, burning or piercing sensation on one side of the head. It's often felt around the eye, temple and sometimes face. It tends to affect the same side for each attack. People often feel restless and agitated during an attack because the pain is so intense, and they may react by rocking, pacing or banging their head against the wall. May Have: = a red and watering eye = drooping and swelling of 1 eyelid = a smaller pupil in 1 eye = a sweaty face = a blocked or runny nostril The attacks generally last between 15 minutes and 3 hours, and typically occur between 1 and 8 times a day. = Usually same time every day = Cause unclear, link to Hypothalamus = More in smokers = Genetic link suggested = Aggrevated by Alcohol, Strong Smells, Petrol Pattern of attacks= usually happen every day, in bouts lasting several weeks or months at a time (typically 4 to 12 weeks), before they subside. A symptom-free period (remission) will often follow, which sometimes lasts months or years before the headaches start again. They'll often get cluster headaches every year for many years and they may be lifelong. They tend to happen at similar times of the year, commonly in the spring and autumn.
349
Nerve Conduction Velocity (NCV)
involves electrical stimulation of a peripheral nerve recording latency and amplitude along it. From latency, conduction velocity can be calculated. Normal Conduction Velocity is: = 50- 60 m/Sec in arms = 40-50 m/Sec in Legs Amplitude is small & large Nerve size #Demyelination: Velocity is slow, amplitude is preserved. #Axonal neuropathy: amplitude is low, velocity is normal. Reduction of amplitude of recorded responses generally indicates a loss of axons. Acute neuropathy - upto 4 weeks Chronic Neuropathy: more than 8 wks Neuropraxia - focal of segmental demyelination with preservation of the axon, recovers in 2/12 weeks Axonotmesis: axon is divided but the epineurium remains intact and regrowth occurs at 1 mm/day from injury site
350
Autonomic Chemical Tests
1. Serum Nor-epinephrine: Supine NE- reduced in postganglionic lesions = Standing NE at 5 minutes - fails to rise in pre-ganglionic lesions 2. Tyramine administration - causes NE to be release at post-ganglionic ends and is reduced when lesion is post-ganglionic. 3. Phenyl Ephrine (an alfa agonist) administration - shows excessive NE response even at low levels 4. Trimethaphan (a nicotinic antagonist) causes ganglionic blockade and can induce hypotension if lesion is pre-ganglionic. production of controlled hypotension during surgery to reduce bleeding into the surgical field and also for rapid reduction of blood pressure in the treatment of hypertensive emergencies, especially in patients with acute dissecting aneurysm, and in the emergency treatment of pulmonary edema in patients with pulmonary hypertension associated with systemic hypertension.
351
Dementia, Reversible Causes
Thiamine deficiency, early Hypothyroidism Vitamin B12 deficiency Normal pressure Hydrocephalus Subdural Haematoma Chronic infections Brain tumors Drug intoxications
352
Periodic Paralysis (non dystrophic myotonia)
1. Ca channel related: HypoK-PP The most common familial form, type 1 HypoKPP, has a mutation in the dihydropyridine-sensitive, skeletal muscle calcium channel gene, CACNA1S. Type 1 Hypokalemic periodic paralysis - Autosomal dominant, starts in adolescence, no myotonia, attacks daily or yrly, K low during weakness, doesnt change with K infusion. 2. K channel - Anderson Syndrome: AutoDom, Early childhood onset, No myotonia, episodic weakness, Daily to yrly attacks last 2-24 Hrs, No change with K loading or muscle cooling. 3. Na channel related Hyperkalemic periodic paralysis Paramyotonia congenita
353
Hypokalemic Periodic Paralysis, Acute Treatment
Acute Treatment: The goal is to normalize the serum potassium level by administering oral potassium chloride. Oral potassium chloride is administered in incremental dose, starting initially with 0.5 to 1 mEq/kg (i.e., 60 to 120 mEq of potassium for a 60 kg individual) is reasonable. If they do not respond to the initial dose, then 30% of the initial dose (i.e., 0.3 mEq/kg) is repeated every 30 min. If the patient requires the addition of more than 100 mEq of oral potassium, then close monitoring of serum potassium is needed, and the total dose of oral potassium should not be more than 200 mEq within the 24 hours of starting of the treatment. do ECG monitoring, and muscle strength should be examined periodically. Serum potassium level should be monitored for 24 hours after treatment as the post-treatment rise in serum potassium level can have an adverse effect on patients. IV potassium is not preferred initially and is reserved for arrhythmias due to hypokalemia or if the patient has swallowing difficulties or respiratory muscle paralysis. IV potassium is preferentially administered with the mannitol, not with dextrose or saline as both carbohydrate and salt can itself trigger the muscle paralysis and thus may worsen the weakness. IV potassium therapy requires inpatient, continuous ECG monitoring. 40 mEq/L in 5% of mannitol solution of IV potassium is infused at a rate not more than 20 mEq/hour, not exceeding 200 mEq in 24 hours. Individuals having a milder form of attacks can also benefit from low-level exercise.
354
Periodic Paralysis, prevention of attack
Pharmacologic interventions include medications like chronic potassium supplementation, carbonic anhydrase inhibitors (CAIs), potassium-sparing diuretics that are used when lifestyle modifications become insufficient in reducing attack rates. The favored approach is to add one of the diuretics with the chronic potassium supplementation. The initial choice of diuretics is carbonic anhydrase inhibitor acetazolamide.
355
D/d for Primary HypoK Periodic paralysis
Hyperkalemic or normokalemic periodic paralysis Thyrotoxic periodic paralysis Andersen-Tawil syndrome Secondary hypokalemia Myasthenia gravis Paramyotonia congenita
356
Autonomic disorders due to spinal level causes
Traumatic quadriplegia Syringomyelia SACD Multiple sclerosis Tetanus Amyotrophic Lateral sclerosis, ALS Stiff Man syndrome (alternating rigidity and spasm in their muscles. Muscles can be so rigid that they feel like a board. And the muscle spasms, which are extremely painful and can be triggered by stress or other external factors, can create enough force to break bones.F)
357
Headaches Differentials
Exclude if acute: SAH, meningitis, Carotid-Vertebral dissection, Cavernous sinus thrombosis, Pituitary apoplexy Primary Headaches: 1. Migraine with or without aura 2. Tension Headache 3. Trigeminal autonomic cephalgia-Cluster Headchae 4. Medical Oversuse Headache 5. Exertional Headache Secondary Headache: Infections - meningitis, abscess Neoplastic: Brain or metastatic Vascular: SAH, GCA, Venous thrombosis, Extracranial dissection Traumatic: Head or neck injury Referred pain - Otitis, Glaucoma, Sinusitis
358
Headache Features
Tension Headache: usually end of day, band around head, bilateral and across forehead, improves with rest Migraine Headche: unilateral, throbbing, with or without aura, frontoparietal, can have transient hemiplegia, prefers dark quiet room Cluster Headache: always unilateral, retro orbital, eye watering, redness, many clusters in a day Temporal Arteritis: temporal headache with tender scalp, eye pain, risk of blindness, red desaturation, RAPD, pale optic disc, needs urgent steroids SAH, meningitis - occipital, stiff neck, photophobia, sudden thunderclap in SAH, fever, rash, contact history Drugs induced
359
Optic neuropathies
Demyelination as in MS Vascular - AION (GCA) , Non arteritic AION in vasculitis, anemia, Hypotension, CTDs Compressive - meningioma, Glioma, metastasis Nutritional: Thiamine, B12, Folate def Drugs/ Toxins: Ethambutol, Amiodarone, INH, Lead, CO Orbital trauma, Syphilis, Lyme Hereditary: Congenital Lebers Hereditary Optic neuropathy
360
Headache analysis
Onset/Site/ Duration/ Progression/ Aggravating factors/ Relieved by/ Associated features Triggers: Exercise, certain foods as chocolates, Cheese, pre-menstruation, Bright lights, Sounds, Lack of sleep, Analgesic overuse headache > 12 days per month use of painkillers for headache. Risk factors for serious causes: - Past malignancy, Arthritis (? SLE), HIV risk factors cause odd CNS infections, Smoking, Alcohol, Illicit drugs, Contact with possible Meningitis, Local Pain Sources: TM joint pain, Temporal arteritis, Herpes rash, C SPINE tenderness, Greater occipital nerve tenderness, eye problems, ear, mastoid
361
Absent Ankle DTR + Extensor Plantar conditions
combination of UMN (extensor plantar) and LMN (absent ankle reflex) seen in some conditions as: 1. Cervical myelopathy with DM sensory neuropathy 2. MND with lesion at conus medullaris 3. SACD with B12 deficiency (also can cause pure sensory neuropathy, dementia) 4. Tabes Dorsalis due to tertiary syphilis after about 20 yrs of untreated primary syphilis - cellular reaction in dorsal root ganglion causes retrograde sensory tract loss, initial lancinating pain attacks then loss 5. Friedrich ataxia: GAA repeats, AutoRecessive, degeneration of spinocerebellar, Doral column, corticospinal tracts causing mixed signs. Progressive ataxia below 25 yr old, EMG+NCV suggestive of axonal neuropathy,
362
SACD vs Tabes Dorsalis vs Friedrich ataxia
Dorsal columns degeneration in all 3 Rhomberg test positive in all 3 Spinothalamic tracts spared in all 3 Absent ankles reflex(LMN) in all 3 Extensor plantar reflex (UMN sign) in all 3 SACD: Optic atrophy with centro-caecal scotoma, Dementia, Pernicious anemia Tabes dorsalis: Charcot Joints, Argyl-Robertson pupils, Autonomic degeneration, Neurgogenic bladder, constipation Friedrich Ataxia: Pes cavus + Kyphoscoliosis as long standing neuropathy, Cerebellar signs, DM, Cardiomyopathy
363
Chronic Demyelinating Polyneuropathy
Genetic/ Hereditary: = CMT type 1,4, X linked = Hereditary Liability to pressure palsies = Metachromatic Leucodystrophy: Lipid build up = Globoid cell Leukodystrophy: Krabbe's disease = Refsum disease: Phytanic acid build up (Peroxisome deficiency) Acquired CDP: = CIDP: Autoimmune, High CSF proteins = Multifocal Motor Polyneuropathy = Paraproteinemic demyelinating polyneuropathy = Monoclonal Gammopathy of Uncertain Significance, HIgh Globulins = Osteosclerotic myeloma
364
Progression of Distal Symmetrical Polyneuropathy
Predominant Axonal DSP: DM, Vasculitis, Toxins Predominant Motor DSP: Charcot-Marie-Tooth Initial distal symmetrical sensory symptoms Ascending sensory symptoms Weakness of toe, foot dorsiflexion (cant stand on toe or heels) Loss of ankle reflexes Relatively preserved plantar flexion (cant stand on toes ie weak dorsal flexion but good plantar flexion) Shin & Finger tip paraesthesia Gait Disorder: Lost proprioception, weak extensors Mid thigh, upper forearm & Lower abdomen Hypoesthesia Advanced: Stock & Glove sensory loss, distal wasting and weakness, Absent reflexes
365
Syringomyelia
Syrinx is an empty cavity which starts in center of brainstem or upper cord and expands which damages neural tracts with effects Dissociated sensory loss ie loss of pain, temperature but preserved vibration and position sense and differentiates it from DM/ Tabes dorsalis. Clinical: = Horner's syndrome = Loss of hot/cold discrimination & pain in cape like distribution & both UL (C2) = T1 root signs: Wasting of small hand muscles = Loss of Upper Limb DTRs = Flaccid Tone in Upper Limbs = Spared dorsal columns (no Rhomberg) = Late: affects CST: Spastic paraparesis = Charcot Joints (neuropathic) = Cervical Rib might be present = Kyphoscoliosis La Main Succulemente: Cold, swollen, dystrophic hands that may occur in syringomyelia
366
Spastic Paraparesis
above C4 causes tetraparesis Clinical: Spastic limb tone with weakness, clonus, hyperreflexia, extensor plantars, root signs might be present (UMN lesion) in Brown-Sequard Syndrome: contralateral pain and temperature loss, ipsilateral position vibration, spastic paraparesis Compressive Causes of SP: = Cervical spondylosis = Disc prolapse = Tumors, Trauma to cord = Meningioma, Astrocytoma, Neurofibromas Non Compressive Causes of SP: = Multiple sclerosis = Viral infections = Autoimmune as SLE = Infarction, Bleed in cord = Friedrich ataxia (degenration) = Spino-Muscular atrophy = Hereditary Spastic Paraparesis = West Nile virus, HIV, Syphilis, Lyme = SACD, Vitamin E deficiency = Lathyrism, Konzo disease in Cortical Lesions with Quadriplegia: Bilateral cortical
367
Konzo disease
Konzo is an epidemic paralytic disease occurring among hunger-stricken rural populations in Africa where a diet dominated by insufficiently processed cassava results in simultaneous malnutrition and high dietary cyanide intake.
368
Myelopathy Work up and Treatment
Work up: Early MRI spine: High signal on T2 CSF analysis: including Oligoclonal bands Treatment: - Surgical decompression - IV Acyclovir 10 mg /Kg/TID if HSV myelitis?? - Pulses Methyl Pred 0.5-1 GM OD x 3 days - Hydroxycobalaime 1000 mcg OD x 5 days then every month once - HAART therapy for HIV Pain control Intensive Physiotherapy, Air Bed
369
Small Hand Muscle Testing
Abductor Pollicis brevis (APB): thumb Abduction, test by Palm, thumb to ceiling, dont let me push down your thumb to palm First Dorsal Interossei: index finger: Test as palm up and push index out against resistance Abductor digiti minimi, little finger abduction tests by pushing out little finger against resistance APB weak + 1st DI weak = T1 radiculopathy APB weak + 1st DI spared = Median nerve lesion APB intact + 1st DI weak = Ulnar nerve lesion
370
Causes of C8/T1 root lesions
= cervical spondylosis = Syringomyelia = MND = Pancost tumor = Cervical Rib
371
Weak Hand Muscle Causes
C8T1 root lesions Polyneuropathies Disuse atrophy as in Long Rheumatism Ulnar + medial lesions
372
Obturator nerve Lesions
due to plevic tumors or surgery Weak Hip adduction & sensory loss over medial thigh Same can be seen with Lumbo-sacral plexopathy
373
Lateral Spinal Lesion at L2L3L4
Neurofibroma at L2L3L4 Anterior thigh pain Quadriceps wasting, weak knee extension ankle inversion (L4) weakness Absent ankle DTR or Brisk ankle + extensor plantar if terminal cord compressed Sphincter dysfunction, incontinence
374
Midline Lesions in Cauda Region
Intrinsic Lesions: Dermoid tumor, Lipoma, Ependymoma Extrinsic Lesions: Disc protrusion, Sacral tumors, Bone metastasis Root damaged in to out, S5-S4-S3 Ankle reflex Lost (LMN sign) S1L5 Genital Pain, rectal pain without local pathology Perianal numbness Saddle numbness, area sits on horse saddle Anal wink reflex weak or lost Rectal tone weak or lost
375
Acute Flaccid Paralysis Differentials
Brainstem Causes: Bleed, Stroke, Encephalitis Myelopathic: SOL, Acute transverse myelitis Anterior Horn Cell: Poliomyelitis, Other virals NMJ disease: MG, Toxins Myopathies: Hypokalemia, HypoPhosphatemia, Inflammatory myopathies, Acute rhabdomyolysis, Periodic paralysis Peripheral Neuropathies: GBS, Diphtheria, Botulism, Heavy metals, Toxins, Drug induced, Vasculitis, Acute intermittent porphyria, Critical illness, Lymphadenopathies, Post Rabies vaccination AFP
376
Neuropraxia
Focal or segmental demyelination with axon preservation and recovery occurs in 2-12 weeks Axonotmesis: Axon divided but epineurium intact and regrowth occurs at 1 mm/day Neurotmesis: complete division of neuron and no recovery or regrowth possible
377
Allodynia:
Pain after a non painful stimulus ie pain after light touch
378
Paraesthesia
Abnormal sensation evoked or spontaneously and not necessarily unpleasant Dysesthesia: Unpleasatn paraesthesia Hyperesthesia: increased sensitivity to a stimulus
379
Autonomic Symptoms
Postural Hypotension Paroxysmal Hypertension Tachycardia or bradycardia episodes Atonic bladder Constipation Erectile dysfunction Dry eyes and dry mouth Anhidrosis ie loss of sweating Hyperhidrosis rarely Diarrhea rarely
380
Meralgia paraesthetica
Pain and tingling at lateral mid thigh due to damage to lateral cutaneous nerve of thigh damaged at inguinal ligament Common causes: = Obesity = Pregnancy = Surgery
381
Volkman's Ischemic contracture
due to brachial artery tear in supra condylar fracture of humerus
382
Foot Drop Causes
Common peroneal nerve palsy Sciatic nerve palsy L4L5S1 root lesions Polyneuropathy as CMT disease UMN lesions involving cord and cortex
383
Diabetic amyotrophy
pain, weakness and wasting of quadriceps and thigh due to lumbo-sacral plexus micro vasculitis
384
Shoulder Movement Muscles
Flexion: forward movement: Pectoralis major, Deltoid (anterior 1/3), coracobrachialis Extension: Backward movement: Deltoid (posterior 1/3rd), Lattisimus dorsi, Pectoralis major, Teres major if shoulder already outstretched to side Ab-duction: Supra spinatus for first 15 degrees then deltoid Ad-duction: Pectoralis major, Deltoid middle 1/3rd, Subscapularis Medial Rotation: Pectoralis major, Deltoid middle rd, Lattisimus dorsi, Teres major, Subscapularis Lateral Rotation: Teres minor, Infraspinatus
385
Anterior Cord Syndrome
Loss of power and pain below injury but preserved touch & Proprioception. Injury from front of cord.
386
Spinal Cord Tracts
387
Posterior Cord Syndrome
Loss of sensation but power preserved power below lesions.
388
Central Cord Syndrome
usual mechanism is in elderly people who fall on their chin and cause extension injuries. Incomplete tetraparesis with upper limb paresis more than lower limb. Lower limb power may be normal. Sensory deficits are variable.
389
8 D’s of stroke care
Detection Dispatch Delivery Door ie ED care Data ie CT, assessment Decision to do what Drug or device ie thrombectomy Disposition
390
Stroke care targets
Door to needle < 60 minutes in 85% Door to device in 90 minutes for direct arrivals to ED or 60 minutes for transfer in
391
Stroke care targets
Door to needle < 60 minutes in 85% Door to device in 90 minutes for direct arrivals to ED or 60 minutes for transfer in
392
4D’s of delay for STEMI care
Door to data (ECG) Data to Decision Decision to Drug or device 1. Door 2. Data 3. Decision 4. Drug
393
Time targets for stroke
ED assessment < 10 minutes Neuro assessment < 20 minutes CT/MRI done & reported < 45 minutes Fibrinolysis < 45 min from arrival Or Thrombectomy < 90 minutes for direct arrivals & < 60 min for transfer in Admission to stroke unit < 3 Hours
394
IV Alteplase
Plaminogen activator, human Dose 9 mg iv bolus in 1 minute and 81 mg infusion over 1 hour - for > 18 and also > 80 years - for severe stroke indicated - for mild stroke recommended - if BP <180/110 or when lowered to this -
395
Alteplase contraindications
- ICH - Recent head injury < 3 months - Severe ischemic large stroke with clear hypo attenuation - ischemic stroke < 3 months - mild disabling stroke 3-4.5 hours - intra spinal surgery < 3 months - structural GI malignancy - recent bleeding event < 21 days - coagulopathy: INR >1.7, aPTT>40, PT > 15, platelets < 100,000 - full dose LMWH taken last 24 hours - pts on thrombin inhibitors or Factor Xa inhibitors with coagulopathy - along with Abciximab
396
L5 and S1 root lesion signs
**L5 Root lesion:** - weak Extensor hallucis longus - weak ankle dorsiflexion - weak ankle eversion - altered sense in L5 dermatome **S1 Root lesion:** - weak ankle plantar flexion - weak ankle eversion - weak or lost ankle jerk - altered S1 dermatome
397
Glabellar Tap Sign
The stimulus for the glabellar reflex is light taps with the finger or soft rubber reflex hammer, about two times per second, over the patient's glabella. Although most normal persons respond to this by blinking bilaterally, the blinking stops after the first few taps in normal individuals. Persistent blinking is a positive response, although there is no consensus whether habituation should be indefinite or just beyond a certain number of blinks (e.g., >4 successive blinks). The glabellar reflex is sometimes called the “blink reflex” or “Myerson's reflex,” although the original description was by Overend in 1896.
398
Flaccid dysarthria
399
Oculogyric crisis
This neurological phenomenon is characterized by a sustained dystonic, conjugate, involuntary upward deviation of both eyes lasting seconds to hours. The term oculogyric is applied in reference to the **simultaneous upward movement of both eyes**. The reaction is not life-threatening. Other features that are noted during attacks include mutism, palilalia, eye blinking, lacrimation, pupil dilation, drooling, respiratory dyskinesia, increased blood pressure and heart rate, facial flushing, headache, vertigo, anxiety, agitation, compulsive thinking, paranoia, depression, recurrent fixed ideas, depersonalization, violence, and obscene language.