Neurology Flashcards
Anton syndrome
Anton syndrome is due to bilateral parieto occipital infarcts causing cortical blindness with accompanying denial and confabulation.
Leber’s hereditary optic atrophy
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.
Uhthoff’s phenomenon
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.
Pulfrich phenomenon
It is seen in demyelination and optic neuritis. There is misperception of the trajectory of moving objects. Fading of vision might be there.
Cauda Equina Syndrome
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.
Tabes Dorsalis
tabes means wasting away
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
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D/d - Freidrich ataxia, MS
General paralysis of insane
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
Meningo-vascular syphillis
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
Circle of willis
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
Subclavian Steal syndrome
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
Parasympathetic Ganglions of Head - SPOC - 7793
SPOC-7793: Parasympathetic ganglion
Submandibular Ganglion:7
Preganglionic - 7th facial nerve (chordaTympani branch)
Post Ganglionic - directly to sublingual and submandibular glands
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Pterygopalatine Ganglion: 7
Preganglionic - 7th nerve, Greater petrosal branch
Post Gang - V2 branch of Trigeminal,
To - Nasopharynx, Lacrimal, Palate
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Otic Ganglion: 9
Preganglionic: 9th, Lesser petrosal N.
Post ganglionic: Auriculotemporal N. to parotid gland
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Ciliary Ganglion: 3
Preganglionic: 3rd, Oculomotor nerve
Postganglionic: Short ciliary nerves to sphincter pupillae and ciliary muscles
Superior Orbital fissure contents
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
Inferior Orbital Fissure contents
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)
Jugular foramen contents
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
Branches of External Carotid Artery -
Mnemonic:
Some Anatomists Like Freaking Out Poor Medical Students
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
Pterygoid venous plexus communications
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
branches of ICA - Internal carotid artery
A VIPS COMMA
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
Dystrophia myotonica - clinical features - ABCDEFGH
1 form of muscular dystrophy
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
Trigeminal nerve branches
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
Abducens nerve
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
Cervical Spondylitis
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
Leg Deep Tendon Reflexes and
Babinski/Chaddock/Oppenheim/Gordon signs
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
Foot movements-Muscles-Nerves
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
Lumbo-Sacral PLexus
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
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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
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
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.
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
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At Knee Joint:
Extension: by L3L4 - Quadriceps
Flexion: by L5S1 - Hamstrings
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At Ankle Joint:
Flexion by L4L5 - Dorsiflexion, Posteriaris tibialis extensor
Extension: S1S2 - Plantar flexion, Calf muscles
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
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
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
Ulnar claw Hand (Low Lesion)
Distal digits index, middle fingers flexes but ring + little fingers do not (on ulnar side) -when lesion is beyond wrist. Muscles innervated by the ulnar nerve include:
Abductor digiti minimi.
Flexor digitorum profundus.
Flexor digiti minimi.
Opponens digiti minimi.
Ring finger lumbricals.
Small finger lumbricals.
Dorsal and palmar interosseous muscles.
Adductor pollicis.
Primary Site of lesions: Ulnar tunnel bwetween prisiform and hook of hamate.
Or
at Guyaon canal at wrist
Medial epicondyle
Brachial plexus injury
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
Carpal Tunnel Median Neuropathy
Tingling numbness beyong wrist or thumb base
Thenar wasting
Numbness on lateral 3 & 1/2 fingers on all palm side and only distal on back side
Tinel Signs: Tap on wrist for symptoms
Phalen 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 and index extended + lateral forearm wasting
Anterior Interosseus Branch lesion: isolated weak pincer grip
Rx:
Spliting at wrist, specially at night
Steroid injection into wrist
Yoga techniques
NCV stury and decompression (open/ Endoscopic)
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
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
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
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.
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
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
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Divisions - Anterior and Posterior, 2x3=6
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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
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
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# 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)
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
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
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)
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.
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.
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.
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
Nothnagel’s syndrome,
(Midbrain Lesion)
Midbrain lesion
Ipsilateral 3rd nerve palsy
Cerebellar ataxia
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
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
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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
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
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.
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
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
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
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.
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,
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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
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
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
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
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
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
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
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
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
Nystagmus Classification
Nystagmus Types:
- Physiological nystagmus
- Ocular nystagmus
- Vestibular nystagmus
- Cerebellar nystagmus
- Ataxic nystagmus
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
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
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
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
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)
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
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
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
MG associations
Associated conditions with Myasthenia
- Thyrotoxicosis, 5%
- Hypothyroidism
- Rheumatoid Arthritis
- Sarcoidosis
- Diabete mellitus
- Polymyositis, dermatomyositis
- SLE
- Pernicious anemia
- Sjogren’s syndrome
- Pemphigus
Myasthenic Crisis
Myesthenic crisis, Chrolinergic 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
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
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
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
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.
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
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,
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
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
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
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.
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
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
Spinal Cord-Root compression
Spinal Cord-Root compression causes
1. Vertebrae and disc lesions
2. Tumors
3. Vasular causes
4. Inflammatory disorders
Spinal Cord-Root Compression-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
Spinal Cord-Root Compression-2
- Tumors causing compression
= Extra-dural: Mets, Lymphoma, Primary Bone tumors
= Intra-dural but extra-medullary: Neurofibroma, Meningioma, Sarcoma
= Intra-medullary: Ependymoma, Astrocytoma, Secondaries
Spinal Cord-Root Compression-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
Spinal Cord-Root Compression-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
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.
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
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
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
Visual Field Defects
- Homonymous hemianopia - vision loss on same side in both eye- say right side - lesion behind optic chiasma
- Bitemporal Hemianopia - vision loss on lateral field of both eyes - lesion at chiasma - pituitary tumor, craniopharyngioma, suprasellar meningioma, aneurysm
- Tunnel vision - central only: RP, Extensive chorioRetinitis, Glaucoma, Hysteria
- Central scotoma -
= with pale optic disc(atrophy),
= swollen pink (papillitis) or
= normal (Retro-bulbar neuritis)
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.
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
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
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
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
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.
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
MND, Differentials
D/d for MND:
= Cervical cord compression
= Syphilitic amyotrophy
= CNS Lymphoma
= SMA of Juvenile type 3
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
= Trigemina 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
Types of Muscular dystrophy,
Genetic, AD, AR or X linked,
Do Muscle Biopsy, CK levels & Genetics
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
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.
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.
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.
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.
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.
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
-
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
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
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
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
-
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
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)
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.
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
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
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
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
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
Prominent Motor PN
Causes of Predominantly motor PN
= Carcinomatous
= Lead induced
= Porphyria
= Diphtheria
= CMT disease
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
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.
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
Painless Neck Weakness
= Myasthenia gravis
= amyotic Dystrophy
= Polymyositis
= Motor neuron disease
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)
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.
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.
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 (this stands for 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 5:
≥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 data 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 5:
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 5:
a new T2-hyperintense or gadolinium-enhancing lesion when compared to a previous baseline MRI scan (irrespective of timing)
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 define the diagnosis of primary progressive multiple sclerosis. The diagnosis now requires 5:
≥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
Multiple Sclerosis Treatment Options
Acute Relapse: to shorten it
1. High dose Methyl Prednisolone, 5.05 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
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.
CTS differentials
= Brachial plexopathy
= Peripheral neuropathy
= Mononeuritis multiplex
= Reflex sympathetic dystrophy, Post trauma to nerve
= C6C7 or C8T1 root radiculopathy
CTS association, underlying cause
= Hypothyroidism
= Amylodosis
= Rheumatoid arthritis
= Cushing’s syndrome
= Acramegaly
= Distal radius bone fracture
= Obesity
= Dialysis patient
= Pregnancy or Menopause
= Vibrating tools Job
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.
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
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
Diplopia: Questions to ask
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?
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
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
Pakinson’s disease Symptoms
3/5 = Bradykinesia, Tremor, Rigidity, Postural instabilty, Gait disorder
Early - Day sleeping, Loss of smell
- Asymmetrical tremor, difficulty with - hand tasks as buttoning
- Walking/ turning difficulty, Reduced speed, Initial freezing then festiant Gait, Loss of arm swing and Falls
- Micrographia
- Poor facial expression
- Associated symptoms:
= constipation
= Fatigue, poor sleep
= Drooling of saliva
think of parkinson’s plus syndromes
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
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
Cog wheel rigidity at risk
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
Ask to read some thing - soft voice and no tones
Postural inastabilty check:
Retropulsion (pull suddenly towards you)
Gait: small shuffling, 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
-
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
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.
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
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
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
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)
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
Spinal Cord Tracts
