Neurology Flashcards
(96 cards)
1
Q
What are the components of the eye exam?
A
Diagnostic facies (acromegaly, Cushing's, hyperthyroid) Frontalis overreactivity Xanthelasma Ptosis/retraction - Middle of pupil to top lid should be 4mm or greater Exophthalmos Cornea - Arcus (hypercholesterolaemia) - Band keratopathy (hypercalcaemia) - Kayser-Fleisher rings Sclera and conjunctiva - Jaundice - Blue (osteogenesis imperfecta) - Pallor - Injection - Telangectasia Acuity Fields Blind spot Fundoscopy - Diabetes - HTN - Optic atrophy, papilloedema, angioid streaks, retinal detachment, central vein or artery thrombosis, retinitis pigmentosa Pupils - Light reflex (direct and consensual), Marcus Gunn (most likely RAPD), accommodation Eye movements - Gaze palsies (supranuclear lesions) - Saccades (horizontal and vertical) - Lid lag (hyperthyroidism) Diplopia worst in which direction? Eye fatiguability (upward 30sec) OR chicken wing Corneal reflex Palpate orbits Auscultate eyes (use bell, stop breathing)
Further: other CN, long tracts, U/A (DM)
2
Q
Lesions in which area are responsible for one-and-a-half syndrome?
A
Dorsal pons (ipsilateral PPRF and MLF)
- Stroke
- MS
- Tumour
3
Q
What are the signs of an upper motor neurone lesion?
A
- Weakness greater in upper limb extensors and lower limb flexors
- Spasticity
- Clonus
- Hyperreflexia and extensor plantar response
4
Q
What are the signs of a lower motor neurone lesion?
A
- Fasciculations
- Wasting
- Hypotonicity
- Weakness
- Decreased or absent reflexes
5
Q
What are the causes of peripheral neuropathy?
A
- Metabolic: DM, uraemia, hypothyroidism, porphyria
- EtOH (+/- thiamine deficiency)
- Drugs and toxins: amiodarone, isoniazid, vincristine, cisplatinum, phenytoin, Vit B6 toxicity, heavy metals
- Amyloidosis
- Guillain-Barre syndrome
- Tumour: lung cancer
- Vitamins: B12 or B1 deficiency, B6 toxicity
- Connective tissue disease: SLE, PAN
- Hereditary
- Idiopathic
6
Q
What is the DDx for predominantly motor neuropathy?
A
DM GBS or CIDP MMN HMSN (Charcot-Marie-Tooth) Acute intermittent porphyria Lead poisoning Diphtheria Dapsone
Always consider MND and NMJ disorders
7
Q
What is the DDx for predominantly sensory neuropathy?
A
DM EtOH Paraproteinaemia B12 deficiency (rarely) Sjogren's syndrome Carcinoma (lung, ovary, breast) Syphilis B6 intoxication Idiopathic
8
Q
What is the DDx for painful peripheral neuropathy?
A
DM EtOH B12 or B1 deficiency Carcinoma Porphyria Arsenic or thallium poisoning Hereditary
9
Q
What are the causes of Horner’s Syndrome?
A
3rd order (distal to superior cervical ganglion)
- Retro-orbital lesions
- Carotid artery lesions: aneurysm, thrombosis or dissection, pericarotid tumour, cluster headache
- Cavernous sinus lesions will affect only pupillomotor sympathetic fibres, which follow the ICA
2nd order (C8-T1 nerve roots to superior cervical ganglion)
- Neck: thyroid malignancy, trauma
- Apical lung tumour (usually SCC)
1st order (hypothalamus to C8-T1 nerve roots)
- Syringomyelia / cervicothoracic cord lesions
- Brain stem lesions: vascular, syringobulbia, tumour
- Hypothalamus/midbrain/pons/medullary lesions
10
Q
What are the components of the speech exam?
A
Shake hand, check handedness
Expose and inspect
Orientation: name, place, date
DYSPHASIA
Nominal: Name objects on card Describe picture Repetition: "No ifs ands or buts". Comprehension: - 1 step: Point to the ceiling - 2 step: Touch your left ear with your right hand - 3 step: Touch your chin, then your nose, then your ear Reading: "Close your eyes" Writing: Dysgraphia
DYSARTHRIA
Articulation - "Baby hippopotamus" = lips - "Yellow lorry" = tongue - "We see three grey geese" = palate - Pa pa pa = lips - Ta ta ta = tongue - Ka ka ka = palate Fatiguability - Count to 30 = MG Bulbar exam - Palatal movement CN IX, X - Uvular movement CN X (away from lesion) - Cough and swallow CN IX, X - Gag reflex CN IX, X - Tongue fasciculations CN XII - Stick tongue out CN XII
11
Q
What are the main differences between bulbar and pseudobulbar palsy?
A
Pseudobulbar affects CN V, VII, IX, X, XII
Bulbar affects CN IX, X, XII
Gag: increased PBP, decreased BP
Jaw jerk: increased PBP, normal BP
Tongue: spastic PBP, fasciculations and flaccid BP
Speech: spastic PBP, raspy (unilateral) or nasal BP
Emotional lability PBP
12
Q
What are the signs of a non-dominant parietal lobe lesion?
A
Dressing apraxia
Visual and sensory inattention
13
Q
What are the components of a Parkinson’s examination?
A
Observation
- Hypomimia, reduced blink rate
- Drooling
- Resting, pill-rolling tremor
- Hypophonia (low volume, monotonous, tremulous)
Upper limbs
- Pill-rolling tremor (pron-supination), often asymmetrical, 3-5Hz, worsens on distraction/synkinesis, improves on finger-nose.
- Cogwheel rigidity - wrists
- Leadpipe rigidity - elbows
- “Count backwards from 20” => Hypophonia + distraction
- Synkinesis (rigidity worsens with contralateral intentional movements)
- Bradykinesia / hypokinesia (finger fractionation)
- Micrographia
Face
- Glabellar tap (Myerson’s sign)
- Blepharoclonus
- Keiser-Fleisher rings
- Vertical gaze palsy
Gait
- Stooped neck with flexed elbows/knees
- Festinating, shuffling
- Freezing
- Narrow-based
- Small stride length
- Low ground clearance
- Reduced arm swing
- Accentuation of tremor
- Postural instability
- Propulsion / retropulsion
Extra
- Postural hypotension (MSA, L-dopa Rx)
- Seborrhoeic dermatitis (autonomic dysfunction)
PSP
- Impaired vertical gaze (PSP)
- Axial rigidity
MSA
- Pyramidal signs (MSA)
- Cerebellar signs (MSA-c): check reflex, dysmetria, dysdiadochokinesis
CBD
- Limb apraxia
- Cortical sensory loss
- Flexion dystonia of one upper limb
- Myoclonus (CBD)
Basal ganglia stroke/tumour
- Upper and lower limb neuro
14
Q
What is the DDx for Parkinsonism?
A
Parkinson's Disease Parkinson's Plus - PSP, MSA-c or MSA-p, CBD DLB Drugs: - metoclopramide - prochlorperazine - chlorpromazine - sodium valproate - methyldopa Dementia pugilistica Normal pressure hydrocephalus Anoxic brain damage Wilson's disease Toxins: CO, manganese, MPTP
15
Q
What are the treatments for Parkinson’s Disease and what are the potential side effects?
A
Levodopa - motor fluctuations, dyskinesias, hallucinations and postural hypotension
Dopamine agonists - confusion; cabergoline and bromocriptine => cardiac valve fibrosis. Impulse control disorders.
Apomorphine ?dopamine agonist
MAO-Bi (selegiline, rasagiline) - confusion
Anticholinergics (benztropine) for tremor
COMTi - dyskinesia
Amantadine (NMDA-R antagonist) - confusion
16
Q
What are the key clinical features of Charcot-Marie-Tooth Disease?
A
Lower limbs
- Pes cavus
- Clawed toes
- Foot drop
- Absent ankle jerks
- Stork leg deformity
- Palpable common peroneal nerve at fibular head
- Sensory loss (myelinated dorsal columns)
Upper limbs
- Claw hand (MCP extension, IP flexion)
- Distal muscle wasting
- Reduced reflexes
- Length-dependent sensory loss
- Postural tremor
17
Q
What are the key clinical features of Motor Neurone Disease?
A
Observation
- Walking aids, AFO
Head
- Bulbar palsy or pseudobulbar palsy (facial muscles, pharyngeal elevation, tongue fasciculations/wasting/flaccidity, speech)
- Ocular muscles NEVER involved in MND
Limbs
- Fasciculations and wasting, often asymmetrical and distal
- Claw hand
- Hypertonia
- Weakness
- Hyperreflexia
- Hoffman’s sign (middle DIP flexion => thumb flexion)
- No sensory defects
Other
- Increased work of breathing
- Assess upright vs supine FVC
18
Q
What are the key clinical features of a cerebellar syndrome?
A
Upper limbs
- Dysdiadochokinesis
- Dysmetria
- Intention tremor
- Rebound phenomenon
- Cerebellar hypotonia
- Normal or pendular reflexes
Face
- Coarse horizontal nystagmus
- Hypo- or hypermetric saccades
- Slurred, staccato speech
Lower limbs
- Wide-based gait
- Rhomberg’s negative
19
Q
What are the key clinical features of myotonic dystrophy?
A
Face
- Myopathic facies: lifeless, lean, expressionless. Mouth open.
- Frontal balding
- Ptosis
- Difficulty opening eyes after tight closure
- Cataracts
- Wasting of temporalis, maseter, SCMs
Upper limbs
- Wasting of small muscles of the hands
- Claw hand
- Distal weakness
- Myotonia on hand grip, or hypothenar percussion
Lower limbs
- Foot drop (high-stepping gait)
- Reduced deep tendon reflexes
Heart
- Cardiomyopathy
- MVP
- AF
- Conduction defects ?PPM
Other
- DM
- Hypogonadism: gynaecomastia, testicular atrophy
- Respiratory infections (hypogamma)
- Somnolence
20
Q
What is the DDx for internuclear ophthalmoplegia?
A
MS
Brainstem lesions (infarct, haemorrhage, tumour, aneurysm)
Wernicke’s encephalopathy
SLE
Miller Fisher Syndrome
Drug overdose (tricyclic antidepressants, phenytoin, carbamazepine, opiates, barbiturates)
21
Q
What are the causes of bilateral ptosis with normal pupils?
A
Senile ptosis Thyrotoxic myopathy MG Myotonic dystrophy FSH dystrophy Ocular myopathy Oculopharyngeal dystrophy Duchenne and Becker muscular dystrophy Chronic progressive external ophthalmoplegia Tabes dorsalis (syphilis)
22
Q
What are the causes of a third nerve palsy?
A
Central:
- Vascular (brain stem stroke)
- Tumour
- Demyelination
- Trauma
Peripheral:
- pComm aneurysm
- Tumour causing raised ICP
- Nasopharyngeal carcinoma
- Orbital lesions
- Basal meningitis
- Infarction (DM, arteritis)
- Trauma
- Cavernous sinus lesions
23
Q
What are the causes of mononeuritis multiplex?
A
DM PAN / GPA / EGPA SLE / RA / Sjogren's Sarcoidosis / Amyloidosis Lymphoma / Carcinoma Lyme disease Leprosy
24
Q
Which drugs are associated with tremor?
A
Beta agonists
Ciclosporin and tacrolimus
Anticonvulsants
Lithium
25
Which disorders are associated with trinucleotide repeats?
```
Huntington's Disease
Myotonic Dystrophy
Fragile X
Friedrich's ataxia
Spinocerebellar ataxia
```
26
What is the DDx for motor neuropathy?
```
CIDP / GBS
Lead
Diphtheria
Porphyria
Dapsone
```
27
What is the DDx for sensory neuropathy?
```
DM
Hypothyroidism
EtOH
Uraemia
Sarcoidosis
Amyloidosis
Paraneoplastic
Vasculitis
B12 deficiency
Infections: HIV, leprosy, Lyme disease
Drugs: Isoniazid, metronidazole, chloroquine, hydralazine, flecainide, pyridoxine, colchicine
```
28
What is the DDx for sensorimotor neuropathy?
```
DM
Hypothyroidism
EtOH
Uraemia
Sarcoidosis
Vasculitis
Paraneoplastic
CIDP / GBS
HMSN (Charcot-Marie-Tooth)
Drugs: Vincristine, vincblastine, paclitaxel, cisplatin, amiodarone, nitrofurantoin, gold
```
29
What is the DDx for a small fibre neuropathy?
```
DM
Hypothyroidism
EtOH
Amyloidosis
Isoniazid, metronidazole, cisplatin, disulfiram
Heavy metals : gold, arsenic, thallium
Sjogren's syndrome
Primary biliary cirrhosis
HIV
```
30
What are the causes of demyelinating polyneuropathies?
```
GBS
CIDP
Multiple myeloma
MGUS
HMSN type 1 and 3
HNPP
Refsum's
POEMS
MMN
HIV
Diphtheria
Tacrolimus
Perhexiline
Chloroquine
```
31
What are the typical features of Friedrich's ataxia?
Bilateral pes cavus
Kyphoscoliosis
High-arched palate (also Marfan's, Turner's, tuberous sclerosis)
Cerebellar signs: Broad-based gait, dysdiadochokinesis, dysmetria, intention tremor, rebound phenomenon, horizontal nystagmus, broken pursuit or saccadic hypo- or hypermetria.
Distal wasting and weakness in a pyramidal distribution
Reduced posterior column sensation (and positive Rhomberg's), depressed lower limb reflexes, upgoing plantars.
Hypertrophic cardiomyopathy - jerky pulse, double apical pulse, ES murmur
Diabetes - retinopathy
Optic atrophy
Sensorineural deafness
32
Which disorders can cause Lhermitte's sign?
MS
Cervical myelopathy
Cervical cord tumour
Subacute combined degneration of the cord (B12 deficiency)
33
What is the DDx for CSF oligoclonal bands?
```
MS
NMO
SAH
GBS
SLE
Behcet's disease
CNS lymphoma
Meningoencephalitis
Neurosarcoidosis
Neurosyphilis
```
34
Which patients with myasthenia gravis should have thymectomies?
Generalised disease AND
Less than 3-5yrs since disease onset AND
Age under 60-65 AND
Symptoms not fully relieved by anticholinesterase drugs (eg. pyridostigmine)
35
What is the treatment ladder in myasthenia gravis?
Pyridostigmine +/- thymectomy
Prednisone + azathioprine
Mycophenolate
Rituximab
Alternatives: MTX, tacrolimus, cyclosporin
36
Which medications should be avoided in myasthenia gravis?
```
Muscle relaxants
Penicillamine
Amonoglycosides
Fluoroquonilones
Macrolides
```
37
What are the upper limb myotomes?
```
Shoulder
- AB C5, 6
- AD C6, 7, 8
Elbow
- Flex C5, 6
- Ext C7, 8
Wrist
- Flex C6, 7
- Ext C7, 8
Finger
- Flex C7, 8
- Ext C7, 8
- AB C8, T1
- AD C8, T1
```
38
What are the lower limb myotomes?
```
Hip
- Flex L2, 3
- Ext L5, S1, 2
- AD L2, 3, 4
- AB L4, 5, S1
Knee
- Flex L5, S1
- Ext L3, 4
Ankle
- Plant S1, 2
- Dorsi L4, 5
- Ev L5, S1
- Inv L5, S1
```
39
What are the different patterns on nerve conduction studies?
Axonal neuropathy
- Reduced CMAP amplitude
Demyelinating neuropathy
- Slowed conduction velocity
- Prolonged terminal latency
- Conduction block if severe
Compressive neuropathy
- Prolonged terminal latency
- Conduction block if severe
40
What are the different patterns on electromyography?
Short duration, low amplitude, early recruitment = myopathic
Long duration, high amplitude, reduced recruitment = neuropathic
Fibrillation potentials = active denervation/inflammation
Reinnervation = long-duration, high-amplitude polyphasic MUAPs
Acute denervation
- Prolonged insertional activity
- Fibrillations and positive sharp waves on spontaneous activity
- Reduced recruitment
Active (inflammatory) myopathy
- Prolonged insertional activity
- Fibrillations and positive waves on spontaneous activity
- Early recruitment
Chronic neuropathies
- Fasciculations
- Neuropathic potentials: large amplitude, long-duration, polyphasic potentials
- Reduced recruitment
Motor neurone disease
- Fasciculations
- Fibrillation potentials
- Neuropathic potentials: large amplitude, long-duration, polyphasic MUAPs, reduced recruitment
Chronic myopathies
- Myopathic potentials: small-amplitude, short-duration, polyphasic potentials
- Rapid recruitment
Myotonic dystrophy
- Myotonic discharges
41
What is the differential for a proximal myopathy?
EtOH
Drugs: Statins, colchicine, antimalarial drugs, penicillamine
Endocrine: Steroids / Cushing's, hypo/hyperthyroidism, diabetes, hyperparathyroidism, osteomalacia
Electrolytes: Hypokalaemia, hypophosphataemia, hypocalcaemia, hyper/hyponatraemia
Viral: influenza, parainfluenza, coxsackie, echovirus, adenovirus, HIV, CMV, EBV
Haemodialysis
Polymyositis/dermatomyositis
Inclusion body myositis (prox lower limbs)
Duchenne/Becker muscular dystrophy
Vasculitis
Overlap autoimmune syndromes: SLE/RA/Sjogren/scleroderma
RA, Sjogren
42
What are the differentials for cerebellar dysfunction with decreased/absent lower limb reflexes?
- Friedreich's ataxia - weakness, dorsal column loss, optic atrophy, hearing loss, kyphoscoliosis, CCF, DM
- Ataxia telangiectasia: oculocutaneous telangiectasias, diabetes, pulmonary disease
- Roussy-Levy variant of CMT
- Vit E deficiency
- Abetalipoproteinaemia
- Refsum disease: icthyosis, sensorimotor neuropathy
43
What are the differentials for length-dependent motor neuropathies?
```
CMT
CIDP
GBS
Thiamine deficiency
Vasculitis
Sarcoidosis
Paraneoplastic
HIV
Tick paralysis
Lyme disease
Arsenic poisoning
Porphyria
```
44
What are the differentials for hereditary spastic paraparesis?
- MND (primary lateral sclerosis): bulbar and upper limb involvement
- Spinal cord tumours (meningioma, neuroma, mets)
- Spinal cord infarction, bleeding, AVM
- Syringomyelia (usually sensory also)
- Demyelination: Progressive MS, NMO
- Transverse myelitis: MS, NMO, viral, autoimmune
- Adrenoleukodystrophy (adrenal insufficiency + myelopathy)
- Deficiencies: B12, copper
- Autoimmune: SLE, Sjogren syndrome, Antiphospholipid syndrome
- Infections: HIV, neurosyphilis, neuroborelliosis
- Hereditary: Spinocerebellar ataxia, Friedreich ataxia, apolipoproteinaemia
- Cerebral palsy
45
What are the components of the higher centres examination?
Dominant parietal
- Gerstman's syndrome
Non-dominant parietal
- Dressing apraxia
General parietal
- Sensory and visual inattention
- Agraphasthesia, astereognosis
- Constructional apraxia
Temporal
- Short term memory: 3 words
- Long term memory: when were the Sydney Olympics?
- Recall 3 words
Frontal
- Grasp, pout, palmar-mental reflexes
- Interpret a rolling stone gathers no moss
- Anosmia
- Shuffling gait with freezing
- Ipsilateral optic atropy + contralateral papilloedema
46
What is the DDx for MND?
Cervical myeloradiculopathy (has sensory disturbance)
MMN
HMSN
Friedreich's ataxia (dorsal column and cerebellar)
Subacute degeneration of the spinal cord (dorsal column + peripheral neuropathy )
Syphilis (dorsal column)
Cord compression, demyelination (sensory changes)
Other anterior horn cell disorders: poliomyelitis, spinal muscular atrophy
47
What is the DDx for chorea?
```
Huntington's disease
Sydenham's chorea
Chorea gravidorum
Senility
Drugs (L-dopa, phenothiazines, OCP, phenytoin)
Stroke
Hyperthyroidism
Idiopathic hypoparathyroidism
SLE
PCV or other causes of hyperviscosity
Wilson's disease
Vasculitis
Viral encephalitis
```
48
What are the common causes of cerebellar syndrome?
```
Toxic and metabolic
- EtOH
- Hypothyroidism
- B12 or Vit E deficiency
- Hypocalcaemia, hypomagnesaemia
Drugs:
- Lithium
- Phenytoin, carbamazepine
- ChemoRx
Malignancy
- Cerebellar mets
- Paraneoplastic. Gynae (anti-Yo), bronchogenic carcinoma
Posterior fossa masses
- Haemangioblastoma (VHL)
- Vestibular schwannoma
Cerebellar stroke (clot or bleed)
Vertebrobasilar insufficiency
Brainstem lesions
Posterior fossa masses
Congenital
- Friedreich's ataxia
- Ataxia telangectasia
```
49
What are the causes of Argyll-Robertson pupils?
```
Neurosyphilis
Autonomic neuropathy
MS
Sarcoidosis
Pinealoma
Tumours of the posterior 3rd ventricle
Syringobulbia
Brainstem encephalitis
```
50
Which are the risk factors for vitamin B6 deficiency and associated neuropathy?
```
Isoniazid
Penicillamine
Hydralazine
Haemodialysis
Malnutrition
```
51
What are the causes of peripheral neuropathy and autonomic neuropathy?
```
DM
B12 deficiency
EtOH
Chemotherapy
HIV
Amyloidosis
Porphyria
Heavy metal toxicity
```
52
What are the causes of unilateral and bilateral cerebellar syndromes?
Unilateral:
- Multiple sclerosis
- Stroke
- Tumour (VHL)
Bilateral:
- EtOH
- Hypothyroidism
- Paraneoplastic (bronchogenic carcinoma)
- Friedrich’s ataxia
- Ataxia telangiectasia
53
What does pupillary sparing with a 3rd nerve palsy suggest?
Vascular cause, such as DM or arteritis
- Pupillomotor fibres are around the periphery of the CNIII
Compression of CNIII usually affects pupil
54
What are the causes of a CN III palsy?
```
DM
HTN
Multiple sclerosis
PCom aneurysm
Syphilis
Vasculitis
Parasellar neoplasms
Base of skull carcinoma
Basal meningitis
Encephalitis
Ophthalmologic migraine
```
55
How may CN III nuclear lesions present?
Bilateral CN III palsy
| Unilateral CN III palsy with bilateral partial ptosis and contralateral superior rectus weakness
56
What are the causes of a unilateral facial nerve (CN VII) palsy?
```
Bell’s palsy
Parotid tumour
Herpes Zoster (Ramsay-Hunt)
Otitis media
Cerebellopontine angle tumour (CN V and CN VIII)
Old polio
Skull fracture
Pontine nuclear lesion (commonly involves CN VI also)
```
57
What are the causes of a cerebellopontine angle lesion?
```
Acoustic neuroma (vestibular schwannoma)
Pontine glioma
Meningioma
Cholesteatoma
Haemangioblastoma
Medulloblastoma or astrocytoma of cerebellum
Basilar artery aneurysm
Nasopharyngeal carcinoma
Meningeal involvement from syphillis or TB
```
58
Which genetic conditions predispose to cerebellopointine angle tumours?
NF2 (schwannomas)
| Familial adenomatous polyposis (medulloblastomas)
59
What are the causes of foot drop?
```
MND
Lumbosacral plexus lesion
Poliomyelitis
Sciatic nerve palsy
Peroneal nerve palsy (mononeuritis, plaster cast of leg, rapid weight loss, crossed legs, leprosy) - inversion preserved
Peripheral neuropathy
- CMT (HMSN)
- Lead/arsenic poisoning
Myotonic dystrophy, IBM, MG
```
60
What are the findings in tabes dorsalis?
```
Dorsal column signs
Positive Romberg's
Ataxic gait
Argyll-Robertson pupils
Rarely: optic atrophy, spinothalamic signs
```
61
What is the pathway involved in pupillary light reflex?
Optic nerve
Superior colliculus (pretectal midbrain)
Decussation of Meynert
Edinger-Westphal nucleus (midbrain)
62
What is a reverse Argyll-Robertson pupil and what causes it?
Reacts but does not accomodate
| - Caused by Parkinsonism causes by encephalitis lethargica
63
What are some causes of anisocoria?
```
CN III palsy
Horner's syndrome
Iritis
Monocular blindness or amblyopia
Stroke
Severe head trauma
```
64
What are the characteristics of an Adie tonic pupil?
- Idiopathic parasympathetic lesion (ciliary ganglion or postganglionic short ciliary nerves within the orbit)
- Acute: pupillary dilatation with partial paresis or accommodation and light reaction
- Chronic: slow pupillary dilatation following release of accommodative constriction
- Associated with depressed lower limb reflexes and segmental anhydrosis
65
Which myopathies affect the proximal upper limbs and distal lower limbs?
FSH muscular dystrophy (facial)
| Emery-Dreifuss (X-linked, colour blind, face spared)
66
What key feature distinguishes Hereditary spastic paraparesis from other causes of spastic paraparesis?
Spasticity > weakness
67
What are the causes of botemporal hemianopia?
PItuitary tumour (superior fields affected first)
Craniopharyngioma (inferior fields affected first)
Suprasellar meningioma
AComm aneurysms (compress superior chiasm)
Metastases
Glioma
68
What are the causes of spinal cord hemisection?
```
Syringomyelia
Haematomyelia
Cord tumour
Degenerative spinal disease
Multiple myeloma
Bullet or stab wounds
```
69
What are the causes of bulbar palsy?
```
MND
GBS
Neurosyphilis
Neurosarcoidosis
Poliomyelitis
Syringobulbia
Nasopharyngeal tumour, particularly post-radiotherapy
```
70
What are the causes of carpal tunnel syndrome?
```
OCP
Pregnancy
Hypothyroidism
RA
Acromegaly
Hyperparathyroidism
Amyloidosis (eg multiple myeloma, dialysis - beta2microglobulin)
Sarcoidosis
```
71
What are the neurological findings in cauda equina syndrome?
Flaccid asymmetrical paralysis
Diminshed knee/ankle jerks
Saddle distribution sensory loss up to L1
Plantars downgoing
72
What are some causes of central scotoma?
```
MS
Optic nerve compression by tumour, aneurysm
Glaucoma
Macular degeneration
Toxins: methanol, tobacco, lead, arsenic
Retinal artery thrombosis: retinal artery thrombosis, GCA, syphilis, iodiopathic
Paget's disease
Vit B deficiency
Retinitis pigmentosa
Freidrich's ataxia
Leiber's optic atrophy
Valsalva retinopathy
Bungee jumping
```
73
What is the significance of anti-Yo antibodies in cerebellar syndromes?
Suggests paraneoplastic syndrome secondary to gynae, usuall ovarian, cancer
74
How are cerebellar lesions localised?
Tandem gait disturbance - anterior lobe
Truncal ataxia - posterior lobe
Limb ataxia - lateral lobes
75
What is the management of Huntington's disease?
- Antidepressants
- Valproate and olanzapine for psychotic features
- Consider mantadine, riluzole, tetrabenazine
76
What maternal factors predispose to neural tube defects?
Sodium valproate use in pregnancy
| Maternal DM
77
What extra-muscular features are associated with FSH dystrophy?
High frequency hearing loss
Retinal telangiectasias
OSA
78
Which conditions can present with absent deep tendon reflexes and upgoing plantars?
MND
Peripheral neuropathy with stroke
B12 deficiency and subacute degeneration of the cord
Tabes dorsalis (syphilis)
79
Which conditions present with spinocerbellar ataxia?
```
Roussy-Levy disease
Refsum's disease
Bassen-Kornzweig syndrome
Olivopontocerebellar degeneration
Machado-Joseph disease - ophthalmoparesis, spasticity, dystonia, Parkinsonism
Dentatorubral pallidoluysian atrophy
```
80
What are the features of POEMS syndrome?
```
Polyneuropathy
Organomegaly
Endocrinopathy
M protein
Skin changes
```
```
Lambda = POEMS
Kappa = MGUS
```
81
Which structures are involved in vertical gaze?
Rostral interstitial nucleus of the MLF (pontine)
Midbrain
Posterior commisure
82
What are the causes of upbeat and downbeat nystagmus respectively?
Upbeat: cerebellar anterior vermis
Downbeat: brainstem lesions, meningoencephalitis, hypomagnesaemia
83
How does Madras motor neurone diseaes present?
- Asymmetrical limb weakness and wasting
- Bulbar and facial involvement
- Sensorineural deafness
- Early onset < 30 yo
84
What is the DDx for a demyelinating CNS disease?
MS
NMO
Leukodystrophies
Tuberous sclerosis (patchy demyelination)
85
What are the causes of myotonia?
```
Myotonic dystrophy
Congenital myotonia (associated diffuse hypertrophy)
Paramyotonia congenita
Polymyositis
Myotubular myopathy
Acid maltase deficiency
Colchicine, clofibrate
```
86
Which diseases cause prominent distal weakness?
Myotonic dystrophy type 1
Inclusion body myositis
Welander's distal myopathy
87
What are the clinical findings in neurofibromatosis type 1?
```
Neurofibromas
Cafe-au-lait spots
Axillary or inguinal freckling
Kyphoscoliosis
Lisch nodules in the iris
HTN (phaeo or renal artery stenosis)
Osseous lesions such as sphenoid dysplasia, long bone pseudoarthrosis
```
88
What levels of radial nerve injury causes triceps and brachioradialis weakness respectively?
- Triceps: above the junction between the upper and middle thirds of the humerus
- Brachioradialis: middle third of the humerus
89
What are the causes of bilateral facial nerve palsy?
GBS
Sarcoidosis
Melkersson-Rosenthal syndrome (recurrent facial oedema, plication of tongue)
MG may mimic
90
Where is the lesion if ipsilateral facial nerve and abducens nerve palsies are present?
Brainstem nucleus (pons)
91
Which cranial nerves are usually affected in syringobulbia?
V, VII, IX, X
92
What are the clinical findings in ataxia telangiectasia?
Cerebellar ataxia
Eye movement abnormalities
Oculocutaneous telangectasias
Immune deficiency
Associations:
- Insulin resistance
- Pulmonary disease
- Increased rate of malignancies
93
What are the causes of transverse myelitis?
- MS, NMO, ADEM
- HSV-2, VZV, CMV
- Syphilis, mycoplasma, spinal cord abscess, aspergillosis
- SLE, Sjogren's, MCTD, SSc, sarcoidosis, Behcet's
- Anterior spinal artery or posterior spinal artery occlusion
- Intramedullary mets, meningioma, neurofibroma, paraneoplastic
94
Which connective tissue diseases predispose to intracranial aneurysms?
Polycystic kidney disease
Ehlers-Danlos disease (type IV)
Pseudoxanthoma elasticum
Fibromuscular dysplasia
95
What are the causes of retinal artery occlusion?
```
Giant cell arteritis
Syphilis
Collagen vascular diseases
Intranasal cocaine and arteriolar spasm
Sickle cell disease
Increased orbital pressure (eg retrobulbar haemorrhage, Graves ophthalmopathy)
```
96
What is the DDx for optic neuropathy?
Optic neuritis:
- MS
- NMO
- ADEM
- Behcet's
- SLE
- Sarcoidosis
- TB
- Syphilis
- Bartonella
- Viral
Compression:
- Tumours. Meningioma, pituitary masses, glioma
- Mets
- Thyroid ophthalmopathy
- Aneurysms
Toxic:
- B12 deficiency
- Methanol intoxication
- Tobacco
