Neurology Flashcards
(267 cards)
1
Q
Features of tuberous sclerosis
A
Skin
- Ash leaf spots
- Nail fibroma
- Freckling
- Adenoma sebaceum (angiofibromas on face in butterfly distribution)
Brain
- Tumour
- Seizures
- Developmental delays/retardation
Others:
- Renal cysts
- Teeth pitting
2
Q
Brainstem signs
A
- Cranial nerve abnormalities with contralateral UMN signs
(because cranial nerves III-XII arise from brainstem; I-II arise from cerebrum)
3
Q
Causes of decorticate vs decerebrate positioning
A
- Decorticate: cerebrum
- Decerebrate: brainstem (worse prognosis)
4
Q
Features of decorticate vs decerebrate positioning
A
Decorticate = flexor
- Arms flexed
- Fists clenched
- Legs extended and turned inwards
- Scores 3/6 in GCS
Decerebrate = extensor
- Elbows extended
- Arms and legs extended
- Head arched back
- Clenched teeth
- Scores 2/6 in GCS
5
Q
Upper motor neurone vs lower motor neurone- which anatomical area differentiates between them
A
- Anterior horn cell (UMN above, LMN below)
6
Q
Signs of UMN vs LMN
A
Upper motor neurone
- Increased tone (spasticity)
- Pyramidal pattern weakness (flexors stronger than extensors in arm, vice versa in legs)
- Increased weakness
- Up-going plantars
- Clonus
Lower motor neurone
- Decreased tone
- Wasting and weakness
- Reduced reflexes
- Fasciculations
7
Q
Signs of spinal cord lesion
A
- Bilateral upper motor neurone signs
- Sensory level
- Sphincter disturbance
8
Q
Signs of brainstem lesion
A
- Dysarthria
- Dysphagia
- Cerebellar signs
9
Q
Signs of nerve root lesion
A
- LMN signs in dermatomal/myotomal distribution
(nerve root = leave spinal cord at different levels e.g. C1, C2 etc– each nerve root has sensory component = dorsal nerve root, and motor component = ventral)
10
Q
Signs of polyneuropathy
A
- LMN signs
- Worse distally (legs worse than arms)
- Sensory signs
11
Q
Signs of neuromuscular junction lesion
A
- Weakness with normal sensation
- Usually proximal
- Fluctuates e.g. time of day/during examination
12
Q
Difference between spasticity and rigidity
A
- Spasticity = increased resistance initially but then gets better (velocity-dependent– clasp-knife) == pyramidal (cerebrum)
- Rigidity = increased resistances constantly == extrapyramidal (basal ganglia/cerebellum)
13
Q
Shoulder abduction - muscle, nerve root and nerve
A
- Deltoid
- C5-6
- Axillary nerve
14
Q
Shoulder adduction - muscle, nerve root and nerve
A
- Lat dorsi and pec major
- C6-8
- Thoracodorsal nerve
15
Q
Elbow flexion - muscle, nerve root and nerve
A
- Biceps
- C5-6
- Musculocutaneous nerve
16
Q
Elbow extension - muscle, nerve root and nerve
A
- Triceps
- C6-8
- Radial nerve
17
Q
Wrist extension - muscle, nerve root and nerve
A
- Extensor carpi radialis longus, extensor carpi ulnaris
- C5-8
- Radial and posterior interssseous nerves
18
Q
Wrist flexion - muscle, nerve root and nerve
A
- Flexor carpi radialis, fl exor carpi ulnaris
- C6-7
- Radial and ulnar nerves
19
Q
Finger extension - muscle, nerve root and nerve
A
- Extensor digitorum
- C7-8
- Posterior interosseous nerve (branch of radial nerve)
20
Q
Finger flexion - muscle, nerve root and nerve
A
- Flexor digitorum superficialis and profundus
- C8
- Median and ulnar nerves
21
Q
Thumb opposition, flexion, abduction - nerve root and nerve
A
- C8-T1
- Median nerve
22
Q
Spastic gait - features and cause
A
- Scissoring
- Narrow-based
- Stiff
- Toes-scuffing
Cause = spastic paraparesis
23
Q
Hemiparetic gait - features and cause
A
- Circumducting
- Scuffing of one foot
Cause = stroke
24
Q
Extrapyramidal gait - features and cause
A
- Shuffling/slow
- Festinant (rapid fall steps as if about to fall over)
- Poor arm swing
- Bradykinesia (ask pt to touch thumb and index finger repeatedly quickly)
Cause = Parkinson’s
25
Apraxic gait - features and cause
- Gait ignition failure
- Upright posture with short steps
Cause = fronto-parietal lesion (Vascular parkinsonism) or normal-pressure hydrocephalus
26
High-stepping gait - features and cause
- Foot-drop
Cause = neuropathy, myopathy, antalgic
27
Why is UMN lesion forehead sparing?
- The muscles of the upper half of the face (frontalis, corrugator and orbicularis) are innervated bilaterally by corticobulbar fibres
28
Fasciculations in tongue
- MND (tongue must be rested in floor of mouth)
29
Motor scoring for GCS
- 6: obeys commands
- 5: towards pain
- 4: away pain
- 3: decorticate (flexor)
- 2: decerebrate (extensor)
- 1: none
30
Reflex nerve roots
- Ankle: S1-2 (button my shoe)
- Knee: L3-4 (kick the door)
- Biceps: C5-6 (pick up the sticks)
- Triceps: C7-8 (open the gate)
1234567!
31
What is Hoffman sign
- Flick middle finger DIP --> see flexion of thumb/fingers = UMN
32
Causes of peripheral neuropathy
Metabolic/endocrine
- Diabetes
- Hypothyroidism
- Uraemia
Toxic
- Alcohol
- Chemotherapy
Inflammatory
- CIDP
- Vasculitis
Paraneoplastic
- Lung cancer
- Paraproteinaemia
Congenital--- only say if there are motor signs/wasting!
- Charcot-Marie-Tooth
33
Dorsal column tested by
- Light touch
- Vibration
- Proprioception
34
Spinothalamic tested by
- Pinprick
- Temperature
35
Hip flexion - muscle, nerve root and nerve
- Iliopsoas
- L1-3
- Femoral nerve
36
Hip extension - muscle, nerve root and nerve
- Gluteus maximus
- L5-S1
- Inferior gluteal nerve
37
Knee flexion - muscle, nerve root and nerve
- Hamstrings
- L5-S1
- Sciatic nerve
38
Knee extension - muscle, nerve root and nerve
- Quadriceps femoris
- L3-4
- Femoral nerve
39
Ankle dorsiflexion - muscle, nerve root and nerve
- Tibialis anterior
- L4-5
- Deep peroneal
40
Ankle plantarflexion - muscle, nerve root and nerve
- Gastrocnemius
- S1
- Tibial nerve
41
Ankle inversion - muscle, nerve root and nerve
- Tibialis posterior
- L4-S1
- Tibial nerve
42
Ankle eversion - muscle, nerve root and nerve
- Peroneus longus/brevis
- L5
- Superficial branch of common peroneal nerve
43
Contractures signify
- Long-standing spasticity
44
Waddling gait
- Proximal myopathy
45
Stamping gait
- Proprioceptive loss (patient figures out where their feet are by resulting sensory/auditory clues)
46
UMN signs with spastic scissoring gait- where is the lesion
- Above C4
47
Poor visual acuity in young patient
- Optic atrophy in MS?
- If very poor acuity, think neuromyelitis optica (inflammation of optic nerve/spinal cord)
48
Elderly patient with brisk upper limb reflexes, wasting of the small muscles of the hands and a sensory neuropathy
- Could be dual pathology! Compressive cervical myelopathy with diabetes
49
Triad in normal pressure hydrocephalus
- Gait apraxia
- Urine incontinence
- Cognitive impairment
50
Causes of hemiparesis
Intracranial
- Stroke: MCA (arm > leg), ACA (leg > arm)
- SOL
- Demyelination/inflammation
Spinal cord = **Brown-Sequard**
- Trauma
- Tumour (neurofibroma)
- Abscess
Others
- Todd's paresis after seizure
- Hemiplegic migraine
51
Difference between paresis and plegia
- Paresis = weakness
- Plegia = paralysed
52
Causes of spastic paraparesis and a sensory level
- Cord compression (disc/tumour/abscess)
- Cord infarction
- Transverse myelitis (infection/autoimmune/paraneoplastic)
53
Causes of spastic paraparesis and dorsal column loss (joint position sense and vibration)
- Demyelination (MS)
- Subacute combined degeneration of cord (B12 deficiency)
- Friedrich's Ataxia (degeneration of spinal cord + demyelination)
54
Causes of spastic paraparesis and spinothalamic loss (pain and temperature)
- Syringomyelia (cysts in cord)
- Anterior spinal artery infarction (affects descending spinothalamic tracts)
55
Features of anterior spinal artery infarction
- Paralysis below lesion (corticospinal tracts)
- Loss of pain/temperature below lesion (spinothalamic tracts)
- Retained vibration/proprioception (dorsal columns not affected as they are posterior)
- Autonomic dysfunction (retention/incontinence etc)
56
Causes of spastic paraparesis and cerebellar signs
- Demyelination (MS)
- Friedreich’s ataxia
- Spinocerebellar ataxia (degeneration)
57
Causes of spastic paraparesis and bilateral UMN signs in arms
- Bilateral strokes
- Cervical lesion above C5
58
Features of flaccid paraparesis (LMN lesion)
- Wasting of upper limbs (hands): Charcot-Marie-Tooth/other neuropathy
- Pes cavus: peripheral neuropathy
- No sensory signs: GBS, CIDP (Chronic Inflammatory Demyelinating Polyradiculoneuropathy), MND
59
Features of flaccid paraparesis (LMN lesion)
- Wasting of upper limbs (hands): Charcot-Marie-Tooth/other neuropathy
- Pes cavus: peripheral neuropathy
- No sensory signs: GBS, CIDP (Chronic Inflammatory Demyelinating Polyradiculoneuropathy), MND
60
Causes of proximal myopathy
- Polyradiculopathy (reduced reflexes): Chronic Inflammatory Demyelinating Polyradiculoneuropathy and GBS
- Inflammatory: dermatomyositis
- Neuromuscular junction: Myasthenia Gravis/Lambert-Eaton Syndrome
- Endocrine: hypothyroidism
- Others: polymyalgia rheumatica/fibromyalgia
61
Cause of pronator drift
- UMN lesion affecting corticospinal tract: supinator muscles in the upper limb are weaker than the pronator muscles, and as a result, the arm drifts downward and the palm turns toward the floor
62
Causes of sensory loss
Central
- Cord: transection/hemisection/anterior artery syndrome
- Brain: parietal, thalamic, brainstem
Peripheral
- Radiculopathy: dermatomal
- Peripheral nerve: asymmetrical (mononeuropathy) and symmetrical (polyneuropathy)
63
Areas of brain which determine speech
- Broca's = expressive dysphasia (frontal lobe of dominant hemisphere)
- Wernicke's = receptive dysphasia (temporal lobe of dominant hemisphere)
64
Causes of dysphasia
Affects Broca's (expressive - frontal) and Wernicke's (receptive - temporal)
- MCA stroke
- SOL
- Degenerative (frontotemporal dementia)
65
Ptosis dilated vs ptosis constricted
- Ptosis dilated = surgical 3rd nerve palsy
- Ptosis constricted = Horner's Syndrome
66
Features of right 3rd nerve palsy
- Right eye down and out, cannot adduct (unopposed action of superior oblique IV and lateral rectus VI)
- Ptosis worse on adducting
Extra points: test for normal IV nerve by asking to look medially--> eye will intort (turn down towards nose)
67
Features of right 4th nerve palsy
- Right eye up and out, moves up further when abducts
- Diplopia downwards (6th nerve = diplopia on abduction)
68
Features of right 6th nerve palsy
- Right eye turned medially, cannot abduct
- Diplopia on abduction (4th nerve = diplopia downwards)
69
Causes of ptosis
- 3rd nerve palsy (dilated)
- Horner's (constricted)
- Myasthenia gravis (worse at the end of the day)
- Myotonic dystrophy
- Senile ptosis/eyelid lesion = commonest in real world!
70
Weakness of eye movements in multiple directions
- "Complex ophthalmoplegia" (myasthenia, thyroid, ocular myopathy)
71
Features of internuclear ophthalmoplegia
- Eye on affected side cannot adduct
- Eye on opposite side has nystagmus when abduct
- (Damage to medial longitudinal fasciculus in the brainstem)
72
How to check for ocular myasthenia
- Look upwards-- will complain of diplopia after a few seconds
- Icepack to eyes for 2 mins- ptosis will improve
73
Can't move both eyes up
- Supranuclear Palsy
74
Can't move both eyes laterally
- Lateral gaze palsy (pontine lesion)
75
In resting position
- Lateral nystagmus
- Upbeat nystagmus
- Downbeat nystagmus
- Lateral nystagmus = unilateral vestibular lesion
- Upbeat nystagmus = cerebellar/brainstem
- Downbeat nystagmus = craniocervical junction lesion (Arnold-Chiari = cerebellar tonsils move down into foramen magnum; demyelination; syringobulbia)
76
When looking at examiner's finger and then nose, affected eye is slow to adduct and left behind
- Mild Internuclear Ophthalmoplegia
77
Cause of small vessel (lacunar) vs large vessel occlusion in stroke
- Small vessel = atherosclerosis
- Large vessel = embolus
78
Areas of brain affected by lacunar strokes
- Deep white matter: corona radiata, internal capsule (corticospinal tracts), basal ganglia
- Does not affect cortex so no cortical deficits (no dysphasia)
79
Types of lacunar stroke
- Pure motor
- Pure sensory
- Sensorimotor
- Ataxic hemiparesis
80
ACA supplies what areas of brain
- Frontal and medial-parietal areas
- Includes motor/sensory areas for lower limbs
81
MCA supplies what areas of brain
- Whole lateral surface of the frontal, parietal and temporal lobes + auditory cortex
82
PCA supplies what areas of brain
- Inferior, occipital, cerebellum
83
Features of ACA stroke
- Leg weakness/sensory loss (worse than arm)
- Urine incontinence
- Face spared
84
Features of MCA stroke
- Arm weakness/sensory loss (worse than legs)
- Homonymous hemianopia (optic radiations in parietal and temporal lobes)
- Dysphasia if dominant hemisphere involved (Broca's = frontal and Wernicke's = temporal)
85
Components of posterior circulation
Vessels supplied by the 2 vertebral arteries
- Basilar artery (pons and superior/inferior cerebellum)
- Posterior inferior cerebellar artery (PICA)
- Anterior inferior cerebellar artery (AICA)
- Superior cerebellar artery (SCA)
- Posterior cerebellar artery (PCA)
86
Features of PICA occlusion (Wallenberg's Syndrome = Lateral Medullary)
- Ipsilateral pinprick loss in face (trigeminal nerve)
- Ipsilateral Horner's (descending sympathetic tract)
- Ipsilateral cerebellar signs (inferior cerebellar peduncle)
- Contralateral pinprick loss in trunk/limbs (spinothalamic)
87
Features of AICA occlusion (Lateral Pontine Syndrome)
- Ipsilateral sensory and motor loss in face (**LMN facial droop** as affects CN 7 which is in pons)
- Contralateral pinprick loss in trunk/limbs
88
Features of PCA stroke
- Homonymous hemianopia with macular sparing = central vision intact (occipital pole at site of rich anastomosis between MCA/PCA)
- Cortical blindness
- Contralateral pain/temperature loss (thalamic damage)
- Weber's Syndrome (medial midbrain): ipsilateral CNIII palsy and contralateral hemiplegia
89
Most common site for spinal cord infarction
- Anterior spinal artery (anterior 2/3 of cord)-- at upper thoracic cord (watershed area)
90
Features of spinal cord infarction
- Acute flaccid paraparesis (becomes spastic later on)
- Loss of pain/temperature (spinothalamic) but preserved vibration/proprioception (dorsal column)
- Loss of sphincter control
91
Cause of basal ganglia haemorrhage (deep) vs lobar haemorrhage
- Basal ganglia = HTN
- Lobar = structural abnormality (AVM/tumour), vascular abnormality (vasculitis/amyloid angiopathy)
92
Causes of subarachnoid haemorrhage
- Genentic: PCKD, Marfan's, Ehlers-Danlos, pseudoxanthoma elasticum
- Smoking
- HTN
- Drugs: cocaine, meth
93
Features of hydrocephalus in ICH
- New 6th nerve palsy (false-localising- nerve is stretched)
- Cushing's triad (rise in BP, bradycardia, wide pulse pressure)
94
Differential diagnosis for stroke
- Viral encephalitis
- Migraine (aphasia/hemiparesis)
- Epilepsy (Todd's paresis post-seizure) = instant symptoms (stroke takes a few mins)
- SOL
- Functional (weakness unlikely to be pyramidal i.e. flexors won't be weaker than extensors)
95
Investigation if CT head normal but still suspect subarachnoid haemorrhage
- LP 12hrs after symptoms to let xanthochromia to form
96
Treatment for subarachnoid haemorrhage
- Nimodipine 60mg PO every 4hrs for 2 weeks = prevents cerebral vasospasm
- Neurosurgery: endovascular coiling/clipping
97
Treatment of TIA/minor ischaemic stroke as per 2023 guidelines
- Clopidogrel and aspirin 300mg loading then DAPT 75mg for 3 weeks --> clopidogrel 75mg OD
(If not appropriate for DAPT, just give clopidogrel 300mg loading dose --> 75mg OD)
98
Criteria for referral for carotid endarterectomy
- Symptomatic stenosis 50% or more
(symptomatic stenosis = acute focal neurology ipsilateral to stenosis)
99
Stroke-specific MRI sequences
- DWI with SWI (susceptibility-weighted images)
- T2-weighted
100
Thrombolysis criteria if stroke occurred >4.5hrs ago (2023 guidelines)
- Can thrombolyse within 9hrs of onset/9hrs of midpoint of sleep if wake-up symptoms
AND
- CT/MRI perfusion scan shows salvageable tissue (mismatch ratio greater than 1.2, a mismatch volume greater than 10 mL, and an ischaemic core volume <70 mL)
100
Thrombolysis criteria if stroke occurred >4.5hrs ago (2023 guidelines)
- Can thrombolyse within 9hrs of onset/9hrs of midpoint of sleep if wake-up symptoms
AND
- CT/MRI perfusion scan shows salvageable tissue (mismatch ratio greater than 1.2, a mismatch volume greater than 10 mL, and an ischaemic core volume <70 mL)
101
Thrombectomy criteria if stroke occurred 6hrs ago- anterior circulation strokes (ACA+MCA) (2023 guidelines)
ICA/M1 occlusion with NIHSS 6+ and pre-stroke MRS 0/1
AND
- 6-12hrs: ASPECTS 3+, irrespective of the core infarct size
- 12-24hrs: ASPECTS 3+ and CT or MRI perfusion mismatch of greater than 15 mL, irrespective of the core infarct size.
102
Thrombectomy criteria if stroke occurred 6hrs ago- posterior circulation strokes (PCA) (2023 guidelines)
- Within 12hrs
- Vertebral/basilar occlusion
- NIHSS 10+
103
What is valvular AF
- Mitral stenosis/artificial valves
104
Cause of Multiple Sclerosis
- Genetic susceptibility
- EBV/HHV6 increases risk (?)
105
Features of Multiple Sclerosis
2 episodes separated in time and space
- Optic neuritis = often presenting symptom (pain behind eye on movement --> reduced acuity/red-green colour blind/RAPD)
- Diplopia (INO or 6th nerve palsy)
- Cord involvement: sensory changes/paraparesis (legs)/urine and bowel dysfunction
- Lhermitte's sign: neck flexion --> electric shock from neck to limbs (cervical cord lesions)
- Uhthoff's sign: symptoms worse when body temp rises (hot shower)
106
Types of Multiple Sclerosis
- Clinically isolated syndrome: 1 clinical episode --> gadolinium-enhancing/T2 weighted lesion 30 days later = relapsing-remitting MS
- Relapsing-remitting MS: usually 1 relapse per year (85% present like this)
- Secondary-progressive MS: gradual decline without clear relapses (80% of RRMS develop this after 20yrs)
- Primary-progressive MS: 15% present with this gradually progressive disorder
107
Investigations in Multiple Sclerosis
- Exclude mimics: autoimmune screen, B12, treponemal serology (syphilis)
- MRI with gadolinium: hyperintense lesions on T2 = periventricular and corpus callosum white matter demyelination (corpus callosum joins 2 hemispheres)- can see old and new lesions
- LP: unmatched oligoclonal bands (in CSF but not in serum) and mildly raised WBCs
- Visual evoked potentials: delayed conduction but normal amplitude = optic neuritis
108
Management of Multiple Sclerosis- acute relapse
- IV methylprednisolone 1g OD for 3 days (5days if severe)
- Screen for precipitating infection before starting methylpred!
- Check glucose
- Plasma exchange if very severe
109
Management of Multiple Sclerosis- chronic
- MDT approach: physio, SLT, OT, incontinence nurses
- DMARDs: interferon beta-1a, glatiramer, natalizumab (risk of PML)
- Urine frequency: oxybutynin (anticholinergic)
- Sensory symptoms: gabapentin/duloxetine
- Muscle tone/spasms: baclofen
110
Differential for Multiple Sclerosis
- Autoimmune: SLE, Sjogren's, Behcet's, sarcoid
- Vascular: recurrent TIA, antiphospholipid
- Metabolic: B12
- Infectious: HIV encephalitis, syphilis, PML, Lyme disease
- Mitochondrial: MELAS (Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes )
111
What is Neuromyelitis Optica and treatment
- Demyelination of cord/optic nerves (antibody directed against aquaporin-4 = can be found in serum)
- Severe optic neuritis
- Treatment: IV immunoglobulin and plasma exchange in the acute setting --> steroids and immunosuppressants
112
Features of Parkinson's Disease tremor
- UNILATERAL pill-rolling
- Worsening by distraction (count from 100 to 1) or repetitive movements of other hand
- Disappears on movement
113
Causes of drug-induced Parkinsonism
- Dopamine antagonist antipsychotics (haloperidol)
- Anti-emetics (metoclopramide = dopamine antagonist)
- Phenothiazines (prochlorperazine)
- Valproate
114
Features of Progressive Supranuclear Palsy (PSP)
- Can't look up
- Falls (backwards)
- Frontalis overactivation
- Axial rigidity (neck and trunk)
115
Features of Multi-System Atrophy
Autonomic disturbance
- Incontinence
- Postural hypotension
- Impotence
Cerebellar ataxia
116
Features of corticobasal degeneration
- Unilateral apraxia/pyramidal signs
- Myoclonus (brief muscle twitching)
- Dystonic posturing
117
Features of essential tremor
- "Yes-yes" head movements
- On movement
- Relieved by alcohol/beta-blockers
118
Features of demyelinating tremor
- Present in posture (holding arms out) or action
- Absent in rest
119
Causes of bilateral cerebellar disorder
- Demyelination
- Bilateral strokes/SOL in posterior fossa
- Paraneoplastic (onconeural antibodies)
Remember PASTRIES
- Paraneoplastic
- Alcohol
- Sclerosis (MS)
- Tumour (posterior fossa)
- Rare (Friedrich's Ataxia = young, spinocerebellar ataxia = old)
- Iatrogenic (phenytoin, lithium)
- Endocrine (hypothyroidism, Wilson's)
- Stroke (brainstem)
120
Features of spinocerebellar ataxia (apart from cerebellar signs)
**May look like well patient with mild cerebellar signs**
- Varied modes of inheritance, degenerative
- Old (Friedrich's ataxia will be young)
- UMN signs
- Peripheral neuropathy
- Ophthalmoplegia
121
Features of Friedrich's Ataxia (apart from cerebellar signs)- including pathogenesis and mode of inheritance
- Degeneration of spinal cord + demyelination
- Autosomal recessive deficiency of frataxin protein- onset 8-15yrs so will be wheelchair-bound (whereas spinocerebellar ataxia patient will be old)
- Spastic paraparesis
- Pes cavus
- Leg wasting with absent reflexes and upgoing plantars
- Dorsal column signs (loss of vibration/proprioception)
- HOCM risk (need regular echos)
122
Features of Miller-Fisher Syndrome
- Ophthalmoplegia
- Ataxia
- Absent lower limb reflexes
(Auto-immune post-infectious- anti-GQ1b ganglioside
antibody)
123
Signs of spinal cord disorders
- Spastic paraperetic gait (scissoring narrow toes scuffing)
- Pyramidal weakness
- Hyper-reflexia with upgoing plantars
- Sensory level about L1
- Catheter
124
Predominant sensory signs vs predominant motor signs in spinal cord disorder
- Sensory: inflammatory myelitis (HIV/syphilis)- also B12 deficiency as affects posterior cord
- Motor: cord compression
125
Asymmetrical spinal cord signs
- Cord compression
- Myelitis
126
Causes of inflammatory cord lesions
- MS: commonest transverse myelitis in young
- Post-infectious acute disseminated encephalomyelitis (ADEM)- MMR, varicella
- Autoimmune: SLE, Sjogren's, vasculitis
- Neuromyelitis optica (optic neuritis with long segments of myelitis)
Get sensory level with mostly sensory signs
127
Features of subacute combined degeneration of cord (SCD)
- Bilateral dysaesthesiae (unpleasant sensation when touched)
- Loss of posterior column modalities = dorsal column (light touch, vibration, proprioception)
- Brisk knee jerks + absent ankle jerks + upgoing plantars
- Spastic paraparesis lower limbs
Causes: B12 deficiency (also copper/Vitamin E)
128
Features of hereditary spastic paraparesis
Heterogenous group of disorders (autosomal dominant/recessive/X-linked)
- Slowly progressive gait disturbance
- Spasticity worse than weakness
- Hyper-reflexia with upgoing plantars
- Complicated form: neuropathy, retinopathy, cognitive issues
129
Causes and features of bulbar palsy
LMN lesion of cranial nerves 9-12
- MND
- Myasthenia
- Neuropathy (GBS)
Features
- Nasal speech (palatal weakness)
- Tongue wasting/fasciculations
- Can't handle secretions
- Dysphagia
130
Causes and features of pseudobulbar palsy
UMN lesion of cranial nerves 9-12 (affecting supply to tongue/oropharynx)
- MS
- Brainstem stroke
- MND
Features
- Hot potato speech (tongue doesn't move as spastic)
- Slow tongue movements
- Brisk jaw jerk
- Emotional labile (sudden laughter/crying)
131
Causes of mixed UMN and LMN signs
- Dual pathology (cervical myelopathy and peripheral neuropathy)
- Cervical radiculomyelopathy (affects nerve root and cord)
- MND
- Syringomyelia
- Subacute combined degeneration of cord
132
Signs of cervical myelopathy with peripheral neuropathy
Mixed UMN and LMN in old person
- Surgical scars on neck
- Brisk reflexes if severe myelopathy with mild neuropathy
- No signs above neck
133
Features of cervical radiculomyelopathy
Degeneration of c-spine --> compression of the exiting nerve roots (radiculopathy) and cord (myelopathy)
Get mixed UMN and LMN signs
- Wasting and pyramidal weakness
--- C5-6: flaccid weakness biceps/supinators with loss of these reflexes
--- C7-T1: wasting of the triceps, forearm or intrinsic hand muscles
- Brisk reflexes (clasp knife)
- Spasticity
- Sensory loss in radicular distribution
134
Types of MND
- Amyotrophic Lateral Sclerosis (ALS): UMN and LMN (corticospinal tracts mainly --> spastic paraparesis)
- Primary Lateral Sclerosis (PLS): UMNs- best prognosis
- Progressive Muscular Atrophy (PMA): LMNs (speech/swallowing issues --> worse prognosis)
135
Treatment for MND
- MDT: SLT/dietitian for feeding
- Riluzole increases survival by 2-3 months (causes nausea/deranged LFTs)
- Baclofen for muscle spasms
- Monitor respiratory function (may need NIV)
- Screen for frontotemporal dementia
136
Features of syringomyelia
Cyst in central spinal canal (syringobulbia is when brainstem affected)
- Loss of pain/temperature but preserved light touch/vibration/proprioception (affects decussating spinothalamic fibres in the central grey matter with preservation of the dorsal columns)
- "Hanging/cape-like" sensory loss in upper body but not legs
- Wasting/weakness intrinsic hand muscles (C8-T1)
- Charcot joints
137
Features of conus medullaris vs cauda equina lesion
Conus medullaris (end of spinal cord) = central disc herniation, demyelination, tumour
- Early sphincter loss
- Saddle anaesthesia
- Upgoing plantars (conus contains UMNs)
Cauda equina = extrinsic compression (prolapsed intervertebral disc), inflammation, infection (HSV)
- Radicular pain
- Unilateral LMN signs (flaccid weakness, absent reflexes)
138
Causes of unilateral LMN facial droop
- Bell's Palsy
- Ramsey-Hunt: herpes zoster reactivation in the geniculate ganglion--> vesicles over the external auditory meatus/in ear canal
- SOL: cerebellar pontine angle (CPA) lesion e.g acoustic neuroma in NF2
139
Features of T1 radiculopathy
- Horner's Syndrome (disrupts sympathetic pathway to the
cervical sympathetic ganglia)
140
Causes of radiculopathy (nerve root damage)
- Disc herniation (usually L5-S1 = sciatica)
- Cervical/lumbar spondylosis (degeneration) + osteophytes
- Compression: schwannoma, abscess, met
- Inflammation: ADEM, GBS
141
Causes of brachial plexopathy
- Brachial neuritis: inflammatory- sudden pain--> patchy sensory loss and winged scapula
- Cancer: Pancoast tumour
- Thoracic outlet syndrome: cervical rib/structural abnormality --> slowly progressive unilateral atrophic weakness of intrinsic hand muscles and numbness in the distribution of the ulnar nerve
142
Features of brachial plexopathy
- Muscle weakness and atrophy.
- Reduced reflexes in weak muscles.
- Sensory loss which commonly involves the axillary nerve distribution (regimental patch C7)
143
Causes of lumbar plexopathy
- Diabetic amyotrophy (L2-4): wasted quadriceps (may be asymmetrical), severe proximal leg/hip pain and weakness, not much sensory symptoms
- Compression (haematoma/abscess/tumour)
- Radiotherapy: slowly progressive weakness and sensory loss of leg
144
Features of median nerve palsy
Affects LOAF muscles of hand (T1)
- Lateral 2 lumbricals: index/middle finger
- Opponens pollicus: make "O" with thumb to fingers
- Abductor pollicis brevis: thumbs up
- Flexor pollicis brevis: thumb flexion
Sensation to lateral 2/3 of palm
145
Tests for carpal tunnel (median nerve)
- Tinel's: tap flexor retinaculum --> tingling along median nerve
- Phalen's: keep this position for 1min
146
Causes of carpal tunnel
- Work-related
- Pregnant
- Diabetes
- Hypothyroid
- Acromegaly
147
Signs of ulnar nerve palsy
- Claw hand
- Sensory loss of little finger and ulnar half of ring finger
148
Signs of radial nerve palsy
- Wrist drop: weakness of wrist extension and elbow extension
- Can't straighten fingers
149
Signs of common peroneal nerve palsy (L4/5)
- Foot-drop (high-stepping gait)
- Weakness of ankle dorsiflexion and eversion of the foot.
- Reduced sensation anterior shin and dorsum foot
150
Causes of peripheral neuropathy (sensori-motor)
- Charcot-Marie-Tooth
- GBS/CIDP
- Drugs: chemotherapy, isoniazid, amiodarone
- Critical illness neuropathy
- Vasculitis
151
Causes of peripheral neuropathy (motor)
- Inflammatory: GBS
- Diabetic amyotrophy
- Critical illness neuropathy
- Heavy metals (lead)
- Congenital: spinal muscular atrophy
152
Causes of peripheral neuropathy (sensory)
- Alcohol
- B12/folate deficiency
- Diabetes
- Uraemia
- Drug: isoniazid, chemotherapy, amiodarone
153
Causes of peripheral neuropathy (mononeuritis multiplex)
- Vasculitis: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), Granulomatosis with Polyangiitis (Wegener's)
- Sarcoid
- Sjogren's
154
Drug-induced peripheral neuropathy (sensori-motor)
- Anti-tuberculosis drugs (isoniazid, ethambutol)
- Antibiotics (metronidazole, nitrofurantoin)
- Anti-cancer (cisplatin, vincristine)
- Anti-arrhythmics (amiodarone)
- Anti-convulsants (phenytoin)
155
Differential diagnosis for motor peripheral neuropathy
- NMJ disorder: myasthenia, Lambert-Eaton
- Distal myopathy: myotonic dystrophy (frontal balding/cataracts/facial wasting); inclusion body myositis (loss of wrist/finger flexion > extension)
156
Myasthenia gravis age of onset
- Bimodal (women in 20s, men in 60s)
157
Myasthenia gravis antibodies
- Anti-acetylcholine receptor
- Anti-muscle specific kinase
158
Myasthenia gravis- other conditions to co-exist
- Thymoma in 15%
- Pernicious anaemia
- SLE
- Vitiligo
- RA
159
Myasthenia gravis features
- Proximal muscle weakness- fatigable
- Ptosis and diplopia - worse at end of day/when eyes kept in same position (hold upward gaze to see if ptosis develops)
- Bulbar weakness (dysphagia, jaw weakness, slow hoarse voice)
- Paradoxical breathing = respiratory muscle weakness- check FVC!!!
160
Myasthenia gravis investigations
- Anti-acetylcholine receptor and anti-muscle specific kinase antibodies
- Single-fibre EMG on repetitive stimulation (will have decrementally reduced response - opposite occurs in Lambert-Eaton)
- CT chest
- FVC <1.5L = HDU, <1.2L = ITU
161
Drugs which worsen myasthenia gravis
- Aminoglycosides
- Quinine
- Beta-blockers
- Phenytoin
- Neuromuscular blockade anaesthetic agents (rocuronium)
162
Myasthenia gravis management (acute and chronic)
- Pyridostigmine (cholinesterase inhibitor) - can add glycopyrrolate to reduce muscarinic effects (oral secretions, bradycardia)
- Prednisolone --> azathioprine if pyridostigmine not effective (anti-muscle specific kinase antibodies)--- steroids can initially worsen myasthenia so severe patients need admission for steroid titration
Acute:
- IV immunoglobulin + plasma exchange
- High-dose pred (1mg/kg)
163
Differential for myasthenia gravis
Ophthalmoplegia
- Thyroid eye disease
- Cranial nerve palsy
- GBS
Bulbar and respiratory weakness
- MND
Proximal limb weakness
- Lambert-Eaton
- Myopathy
- Inflammatory myositis
164
Lambert-Eaton Syndrome cause and mechanism
- Anti-voltage gated calcium channel antibodies --> reduced release of ACh from the pre-synaptic terminal
- 50% cancer (small cell, lymphoproliferative), 50% autoimmune (thyroid, RA)
165
Lambert-Eaton Syndrome features
- >40yrs
- Pelvic girdle weakness (unlike Myasthenia)
- Reduced reflexes, which temporarily improve after contraction of relevant muscle (do reflexes before and after testing power!)
166
Lambert-Eaton Syndrome management
- Treat underlying cause
- Amifampridine +/- pyridostigmine
167
Indication of thymectomy in Myasthenia Gravis
- Anti-AchR receptor +ve patients with generalized disease under the age of 60 (ocular myasthenia may not need thymectomy)
168
Causes of muscle disorder
- Inflammatory (autoimmune)
- Dystrophy: congenital defect in components of muscle fibre
- Metabolic
- Secondary: drugs (steroids/statins), endocrine (hypothyroid), systemic (ICU illness)
169
Red urine/+ve erythrocytes with muscle weakness
- Myoglobinuria = McArdle's Syndrome, a glycogen storage disease (deficiency of myophosphorylase --> glycogen not broken to glucose for use in muscles)
170
Causes of distal > proximal weakness in myopathy
- Inclusion body myositis: flexors of forearm and quadriceps.
- Facioscapulohumeral dystrophy: scapular winging and sparing of deltoid, involvement of anterior tibial
compartment may cause foot drop.
- Myotonic dystrophy: frontal balding, hatchet facies, ptosis
171
Cause of facial weakness with external ocular muscle weakness in myopathy
- Grave’s disease.
- Mitochondrial disorders –look for ptosis, deafness, retinitis pigmentosa
- Myasthenia Gravis
172
Causes of facial weakness without external muscle weakness in myopathy
- Myotonic dystrophy: frontal balding, hatchet facies, ptosis
- Facioscapulohumeral dystrophy –winging, wasting of upper arm, foot drop
173
Features of myotonic dystrophy (including mode of inheritance)
- Most common adult dystrophy (autosomal dominant with genetic anticipation)
- Myotonia: fail to relax after voluntary contraction (after shaking hand)
- Percussion myotonia: percuss thenar eminence--> thumb flexion
- Frontal balding
- Bilateral ptosis
- Wasting of the masseters--> narrowing of lower half of face (‘hatchet face’)
- Distal > proximal weakness and areflexia (first affects hand/extensor elbow)
- **Complications**: conduction block, cardiomyopathy, diabetes
174
Complications of myotonic dystrophy
- Cardiac involvement, with bradycardia, AV block, or heart failure –this is a frequent cause of death
- Low IQ
- Cataracts.
- Diabetes mellitus
- Testicular atrophy.
175
Cause of myotonic dystrophy
- Autosomal dominant
- Expansion of an unstable trinucleotide repeat on chromosome 19q (type 1) or chr 3 (type 2)
- Genetic anticipation = earlier onset and more severe with subsequent generations
- PROM = Proximal myotonic dystrophy (milder, proximal weakness mainly, no cardiac/IQ issues)
176
Cause of Duchenne's vs Becker's muscular dystrophy (including mode of inheritance)
- X-linked mutation in dystrophin gene
- Always male as X-linked
- Duchenne's = no dystrophin; Becker's = abnormal dystrophin
177
Features of Duchenne's vs Becker's muscular dystrophy
Duchenne's
- Onset 3-10yrs
- Proximal weakness legs and lower trunk
- Calf pseudohypertrophy
- Dilated cardiomyopathy/low IQ
- Wheelchair by 12yrs --> dead by 20s
Becker's
- Onset 10-40yrs
- Milder, often still walking as adults
- Fewer IQ and cardiac issues than Duchenne's
- Diagnosis: raised CK, EMG consistent with myopathy,
muscle biopsy and dystrophin gene testing
178
Features of facioscapulohumeral dystrophy (including mode of inheritance)
- Autosomal dominant, onset 6-20yrs
- Facial weakness
- Cannot raise arms above head
- Winged scaplulae (scapulae are pushed off the back due to weakness of serratus anterior)
179
Features of dermatomyositis
- Proximal myopathy legs > arms
- Myalgia
- Heliotrope rash rash and upper trunk
- Gottron's papules
- Periorbital oedema
- Associated with adenocarcinoma/gynae tumour
- Antibody mediated
180
Features of polymyositis
- Proximal symmetrical weakness
- Older people
- No skin lesions
- Cell-mediated (CD8)
181
Features of inclusion body myositis
- Older white males
- Distal muscle weakness (flexion worse than extensors)
182
Management of myotonic dystrophy
- MDT approach!
- Phenytoin may help myotonia
- No anaesthetic as too high risk of resp distress
- Treat complications (heart block, diabetes)
183
Causes of ptosis
Unilateral
- 3rd nerve palsy
- Horner's - look for ipsilateral scars = trauma (endarterectomy) or Pancoast tumour
Bilateral
- Myotonic dystrophy
- Myasthenia gravis
184
Cerebellar lesion - ipsilateral or contralateral signs?
- Ipsilateral (tracts do not decussate in cerebrum)
185
Nystagmus in cerebellar lesion vs VIII nerve palsy- which direction?
Cerebellar
- Fast phase TOWARDS lesion and maximal when looking TOWARDS lesion
VIII nerve palsy
- Fast phase AWAY lesion and maximal when looking AWAY lesion
186
Causes of hand muscle wasting
Anterior horn cell (motor neurone which projects from anterior grey matter of cord to muscle)
- MDN
- Syringomyelia
- Cervical cord compression (C7-C8)
Brachial plexus
- Cervical rib (extra rib above 1st rib)
- Pancoast tumour
- Trauma
Peripheral nerve
- Median and ulcer nerve palsies combined
- Peripheral neuropathy
Muscle
- Disuse atrophy (RA)
187
Management of Parkinson's Disease
***MDT approach and manage non-motor symptoms! Involve Parkinson's Nurse/support groups***
Medical
- Dopamine agonist (pergolide): use for younger patients as less side-effects (nausea/hallucinations) and save L-Dopa until later
- L-Dopa with Dopa-decarboxylase inhibitor (co-careldopa = carbidopa/levodopa): nausea/dyskinesia and effect wears off after a few years
- MAO-B inhibitor (selegiline): stops dopamine breakdown
Surgical
- Deep Brain Stimulation (neurostimulator inserted onto brain to fire impulses to globus pallidus)
188
Features of Charcot-Marie-Tooth (a.k.a Hereditary Sensory Motor Neuropathy)- including differences between 2 types and mode of inheritance
- Type 1 = demyelinating; Type 2 = axonal-- so do nerve conduction studies!
- Autosomal dominant-- mention FH when asked what you would do after examination
- Stocking sensory loss
- Inverted champagne bottle legs
- Pes cavus
- Hand wasting (dorsal interossei and thenar eminences)
- Weakness of ankle dorsi-flexion and toe extension
- Gait: high-stepping (foot drop) and stamping (reduced proprioception)
189
Associating features with Freidrich's Ataxia
- Sensorineural deafness
- HOCM
- Optic atrophy
190
Causes of upgoing plantars and absent knee jerks
- Combined UMN/LMN: cervical spondylosis with peripheral neuropathy
- Friedrich's ataxia
- Subacute combined degeneration of cord
- MND
191
Cause of Bell's Palsy
- Swelling/compression of facial nerve --> demyelination and temporary conduction block (?due to HSV-1)
192
Management of Bell's Palsy
- If within 3days of onset: pred 60mg for 5days, then taper by 10mg every day
- Eye protection: artificial tears, tape eye closed at night
- Ophthalmology if persistent eye symptoms
- ENT referral if no improvement after 3 weeks
193
Mode of inheritance in tuberous sclerosis and neurofibromatosis
- Autosomal dominant
194
Causes of Horner's pupil
Follow sympathetic tract's anatomical course:
Brainstem
- Stroke (Wallenberg's)
- MS
Spinal cord
- Syrinx
Neck
- Pancoast's
- Trauma (endarterectomy)
- Aneurysm
195
Causes of 3rd nerve palsy
Medical = Ms -- normal pupil
- Mononeuritis multiplex (diabetes)
- Midbrain stroke (Weber's)
- Midbrain demyelination (MS)
- Migraine
Surgical = Cs -- dilated pupil (affects superficial pupillary fibres)
- Communicating artery aneurysm (posterior)
- Cavernous sinus pathology (affects III-VI): tumour
- Cerebral uncus herniation
196
How to categorise embolic causes of stroke
Cardiac
- AF
- Mural thrombus
- Valvular
Vascular
- Carotid stenosis (ant. circulation)
- Vertebral disease (post. circulation)
197
Diagnostic criteria for neurofibromatosis type 1
2 or more of:
- 6+ café au lait macules (>15mm diameter)
- 2+ neurofibromas
- Freckling in axillary or inguinal regions.
- 2+ Lisch nodules.
- Optic glioma.
- Distinctive osseous lesion, such as sphenoid dysplasia or thinning of long bone cortex
- 1st -degree relative with NF-1.
- Also scoliosis
198
Diagnostic criteria for neurofibromatosis type 2
- Bilateral 8th cranial nerve masses
- 1st-degree relative with NF-2 and either a unilateral 8th nerve mass or 2 of: neurofibroma, meningioma, glioma, schwannoma or juvenile
posterior subcapsular lenticular opacity.
Also get:
- Renal artery stenosis
- UIP fibrosis
199
Differences between neurofibromatosis type 1 and type 2
- Type 2 less skin: fewer cafe-au-laits, no axillary freckling/Lisch nodules
- Type 2 presents with more central issues: hearing loss, tinnitus, ataxia (acoustic neuromas)
200
3 causes of HTN in neurofibromatosis
- Renal artery stenosis
- Phaeochromocytoma
- Coarctation of the aorta
201
TIA affecting ACA: features
- Emotional changes (frontal)
- Dysphasia (frontal - Broca's area)
- Contralateral weakness (frontal)
202
TIA affecting MCA: features
- Dysphasia (frontal- supplied by ACA and MCA)
- Contralateral weakness (frontal)
- Sensory loss (parietal)
- Visual field loss and neglect (parietal and temporal lobes)
203
TIA affecting PCA: features
- Contralateral visual field loss and neglect (occipital)
- Weakness and sensory loss
204
TIA affecting vertebrobasilar arterial system: features
- Nausea, vomiting, vertigo, visual disturbance, dysarthria, ataxia, weakness and/or sensory disturbance.
205
Risk of stroke post-TIA
- 2-day stroke risk of ~4 %
- 7-day stroke risk of ~5.5 %
- 30-day stroke risk of ~7.5 %
- 90-day stroke risk of ~9 %
206
If CT head is performed within this timeframe, a LP is not needed to confirm subarachnoid haemorrhage
- 6 hours (if done after 6 hours, need to do LP after 12 hours to exclude xanthochromia)
207
Signs of Turner Syndrome
- Webbed neck
- Shortened 4th/5th metacarpals
- Absent sexual characteristics
- Cubitus valgus
- Widely spaced nipples
208
Signs of DiGeorge Syndrome
- Long face
- Low-set ears
- Cleft palate
209
Cause of Brown-Sequard Syndrome
- Unilateral cord lesion
210
Features of Brown-Sequard Syndrome
- Ipsilateral motor loss below (corticospinal injury)
- Ipsilateral vibration/proprioception loss below (dorsal column)
- Contralateral pain/temperature loss below (spinothalamic)
Because spinothalamic decussates at level of cord, whereas others decussate in brainstem!
211
Chromosomes affected in Neurofibromatosis Type 1 vs Type 2
- Type 1: chromosome 17
- Type 2: chromosome 22
212
Acronym for Parkinson's Disease symptoms
TRAP CDS
- Tremor
- Rigidity
- Akinesia
- Postural instability
- Cognitive (hallucinations)
- Digestive (constipation/retention)
- Sleep (REM sleep disorder = muscles don't relax = act out dreams)
213
Treatment for cervical myelopathy
- MDT approach
- Decompressive surgery (neurosurgery)
214
Examination special tests for meningitis
- Neck stiffness must be checked while supine!
- Brudzinksi: you lifting their head off bed will cause involuntary knee/hip flexion
- Kernig: lift up knee bent at right angle--> extending the knee would cause pain
215
When need CT head before doing LP in meningitis
- Focal neurology
- Altered consciousness
- Papilloedema
- Known cancer
- HIV
216
Features of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
- Symmetrical
- Loss of reflexes
- Loss of vibration sense
- Proximal and distal muscle weakness
217
Purpose of nerve conduction studies in peripheral neuropathy
Differentiate between axonal and demyelinating
- Axonal: reduced amplitude and length dependent (occur distally first)
- Demyelinating: reduced conduction velocity and non-length dependent
218
Examination tests which check for small nerve fibres vs large nerve fibres
- Small = pinprick
- Large = vibration
219
Heel-to-toe walking tests for
- "Midline gait ataxia"
220
Horizonal saccades vs vertical saccades
- Horizontal = internuclear ophthalmoplegia
- Vertical = supranuclear palsy
221
Complications of methylprednisolone
- Insomnia
- Personality change/mania
- Rise in BMs
- GI ulceration
- Avascular necrosis of hip
222
Causes of spastic paraparesis
Demyelinating
- MS = most common
Trauma
- Cord compression
Vascular
- Spinal stroke (cord infarction)
- Bilateral cerebral strokes
Congenital
- Motor neurone disease
- Cerebral palsy
- Spinocerebellar ataxia
Infective
- Transverse myelitis
- Abscess causing cord compression
223
Important distinction you need to make with ataxic patient
Cerebellar vs sensory
- Cerebellar: dysarthria and nystagmus
- Sensory: sensation and proprioception loss--- sensory is central (dorsal column damage) or peripheral (neuropathy)... or both (B12)
224
Areas in brain which control speech
- Broca’s (expressive - frontal)
- Wernicke’s (receptive - temporal)
225
Differentials for Motor Neurone Disease
- Multifocal motor neuropathy with conduction block (inflammatory-- no UMN/sensory signs-- needs IVIG)
- Spinal muscular atrophy (congenital loss of motor neurones)
- Kennedy Disease (X-linked recessive, peri-orbital fasciculations)
226
Complications of Down's Syndrome
- Atrial-septal defect/mitral regurgitation
- Acute leukaemia
- Early Alzheimer's
- Duodenal atresia
- Cataracts
227
Bedside investigations for diabetic peripheral neuropathy
- L/S BP (autonomic dysfunction)
- Urine dip
- Fundoscopy
228
How to classify lower motor neurone conditions affecting motor function
Anterior horn cell disorder
- MND
Motor nerve disorder
- Multifocal motor neuropathy
229
Pathophysiology of poliomyelitis
- Destruction of motor neurones --> inflammation around destroyed neurones
230
Features of post-polio syndrome
- Unilateral wasted shortened leg (shortening may be masked by foot drop, so straighten the foot!)
- Progressive weakness
- Fatigue
- Cramping
231
Side effects of Parkinson's medications
- Impulsivity
- On-off phenomenon (esp Levodopa)
- Dose-related dyskinesia
232
Differentiate between neuromuscular junction disorder and myopathy using special investigations
- NMJ: nerve conduction study shows reduced action potentials with repetitive stimulation
- Myopathy: EMG shows denervation
233
Causes of unilateral foot drop
Common peroneal nerve palsy
- Weak ankle dorsiflexion/inversion/eversion
- Cause: fracture fibula neck
L4/5 root lesion
- Cause: disc prolapse
Peripheral neuropathy
Sciatic nerve lesion
- Cause: hip trauma/surgery
234
Causes of flaccid paresis
Anterior horn cell disease
- MND
- Poliomyelitis
Cauda Equina
- Disc herniation/abscess/mets
Lumbrosacral plexoplathy (usually unilateral)
Motor neuropathy
- Inflammatory (CIDP, GBS)
- Infectious (HIV, HTLV-1)
- Congenital (Charcot-Marie Tooth)
Neuromuscular Junction disorder
- Myasthenia/Lambert-Eaton/botulinism
Myopathy
235
Causes of myopathy
Inflammatory
- Polymyositis
- Dermatomyositis
Connective tissue disease
- SLE
- Vasculitis
Endocrine
- Cushing's
- Diabetic amyotrophy
Drugs
- Statins
- Steroids
Paraneoplastic
- Small cell lung cancer
236
Pathogenesis of GBS (inc triggers)
- Resp (M. pneumoniae) or GI (campylobacter)
- Acute inflammatory demyelinating polyneuropathy
237
Clinical features of GBS
- Ascending flaccid paresis
- Distal reduced reflexes
- Distal paraesthesia
- Patchy sensory loss (non length-dependent)
Also:
- Cranial nerves (ptosis, opthalmoplegia, bulbar weakness = CN IX to XII)
- Autonomic dysfunction
- Respiratory!
238
Investigations for GBS
- CSF (raised protein)
- Anti-GM1 antibodies
- Nerve conduction study: reduced velocity
- FVC (<1.2L = ITU!)
239
Management of GBS
- IVIG +/- methylprednisolone
- Plasma exchange
240
Differential for pes cavus
- Charcot-Marie-Tooth
- Friedrich's ataxia
- Polio
- Myotonic dystrophy
- Cerebral palsy
241
Pathogenesis for Motor Neurone Disease
- Progressive degenerative disorder of motor neurons in the motor cortex and corticospinal tract + anterior horn cells of the spinal cord and brainstem.
- Hence affects upper and lower motor neurones
242
Investigations for Motor Neurone Disease
- Bloods: paraneoplastic screen, syphilis serology, B12
- MRI: exclude cord compression
- Nerve conduction studies: exclude multifocal motor neuropathy
- EMG: denervation/fibrillations.
- Lumbar puncture may be indicated to exclude demyelination if unclear clinical picture
243
Paraneoplastic antibodies
- Anti-Hu, Yo, Ri, Ma
- Urine bence-jones protein!
244
Important complications of Friedrich's ataxia
- HOCM
- Diabetes
245
Myotonic dystrophy investigations
- EMG: dive bomber (waxing and waning of potentials)
- Muscle biopsy: fibre atrophy, no inflammation
- Genetic testing (unstable trinucleotide repeat on chromosome 19q)
- ECG: long QT/heart block
- Echo: cardiomyopathy
246
Proximal myopathy investigations
- Bloods: including anti-Jo for myositis
- Cancer screen if myositis
- EMG: low amplitude
- MRI muscle and biopsy: inflammation
247
Origin of cranial nerves
1-2: cerebrum
3-4: midbrain
5-8: pons
9-12: medulla
248
Features of Cavernous Sinus Syndrome
- Complex ophthalmoplegia (CNs 3,4,6)
- Fixed dilated pupil (CN 2)
- (Super Orbital Fissure Syndrome is similar, but also has ptosis/exophthalmos)
249
Features of Jugular Foramen Syndrome
- Deficit of CN 9-11 (and maybe 12)
- Due to tumour (such as glomus tumour)
250
Special tests to do in Parkinson's examination
- Gait
- Bradykinesia: thumb and index finger together
- Writing (if no micrograthia then draw spiral - should be irregular contours)
THEN do following if time
- Supranuclear palsy: test upgaze
- Multisystem Atrophy: cerebellar signs and L/S BP
- Lewy-Body Dementia: mini-mental state examination
251
Special tests to do in Myotonic Dystrophy
- Close eyelids tightly --> delay in opening
- Percussion myotonia of thenar eminence
- CN exam to check for ptosis/ophthalmoplegia/dysarthria
- Cardio including PPM
- Testicular atrophy
- Diabetes (urine dip)
252
Horizontal vs vertical diplopia - cranial nerve palsies
- Horizontal = 6th nerve palsy
- Vertical = 4th nerve palsy
253
Important cause of 6th nerve palsy
- Raised ICP (SOL) = false-localising sign (because 6th is longest cranial nerve)
254
Eyes cannot move in any direction
Complex Progressive External Ophthalmoplegia = mitochondrial
255
Unilateral foot drop = UMN or LMN?
- UMN as plantarflexion is extensor!
256
Drug-induced cerebellar disorder
- Lithium
- Phenytoin
- Carbamazepine
257
Differential for unilateral wasted leg (apart from polio)
- Anterior horn cell: Progressive Muscular Atrophy
- Plexopathy (sensory loss)
- Trauma
258
Cause and features of diabetic amyotrophy
Lumbar plexopathy (L2-4)
- Wasted quadriceps (may be asymmetrical)
- Severe proximal leg/hip pain and weakness
- Reduced reflexes
- Not much sensory symptoms
259
Seizure comes up in Comms Skills scenario- what 2 things must you ask about
- Driving
- Contraception- condoms not good enough!
- (Also counsel about shower not bath, do not cook alone, no open water swimming)
260
Causes of bilateral ptosis
- Myasthenia
- Myotonic dystrophy
- Bilateral Horner's
- Chronic Progressive External Ophthalmoplegia
261
Which cranial nerve is contralateral
- CN 4 (trochlear)
262
Features of Brown-Sequard Syndrome
- Ipsilateral motor loss below (corticospinal)
- Ipsilateral vibration/proprioception loss below
- Contralateral pinprick/temperature loss below
263
Mode of inheritance in Huntington's
- Autosomal dominant (if have mutation, you have 100% chance of getting the disease)
264
Management of Huntington's
- Mainly supportive (MDT)
- Tetrabenazine helps with chorea
265
Thrombolysis absolute contraindications
- BP >185/110
- Seizure at time of onset
- Warfarin and INR >1.7
- DOAC in last 48hrs
- Ischaemic stroke in last 3 months
- Previous haemorrhagic stroke
- Severe liver disease inc varices
- LP in last 10 days
266
Features of complicated form of Hereditary Spastic Paraparesis
- Neuropathy
- Retinopathy
- Cognitive issues
