Neurology Flashcards
What are the components of the eye exam?
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)
Lesions in which area are responsible for one-and-a-half syndrome?
Dorsal pons (ipsilateral PPRF and MLF)
- Stroke
- MS
- Tumour
What are the signs of an upper motor neurone lesion?
- Weakness greater in upper limb extensors and lower limb flexors
- Spasticity
- Clonus
- Hyperreflexia and extensor plantar response
What are the signs of a lower motor neurone lesion?
- Fasciculations
- Wasting
- Hypotonicity
- Weakness
- Decreased or absent reflexes
What are the causes of peripheral neuropathy?
- 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
What is the DDx for predominantly motor neuropathy?
DM GBS or CIDP MMN HMSN (Charcot-Marie-Tooth) Acute intermittent porphyria Lead poisoning Diphtheria Dapsone
Always consider MND and NMJ disorders
What is the DDx for predominantly sensory neuropathy?
DM EtOH Paraproteinaemia B12 deficiency (rarely) Sjogren's syndrome Carcinoma (lung, ovary, breast) Syphilis B6 intoxication Idiopathic
What is the DDx for painful peripheral neuropathy?
DM EtOH B12 or B1 deficiency Carcinoma Porphyria Arsenic or thallium poisoning Hereditary
What are the causes of Horner’s Syndrome?
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
What are the components of the speech exam?
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
What are the main differences between bulbar and pseudobulbar palsy?
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
What are the signs of a non-dominant parietal lobe lesion?
Dressing apraxia
Visual and sensory inattention
What are the components of a Parkinson’s examination?
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
What is the DDx for Parkinsonism?
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
What are the treatments for Parkinson’s Disease and what are the potential side effects?
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
What are the key clinical features of Charcot-Marie-Tooth Disease?
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
What are the key clinical features of Motor Neurone Disease?
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
What are the key clinical features of a cerebellar syndrome?
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
What are the key clinical features of myotonic dystrophy?
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
What is the DDx for internuclear ophthalmoplegia?
MS
Brainstem lesions (infarct, haemorrhage, tumour, aneurysm)
Wernicke’s encephalopathy
SLE
Miller Fisher Syndrome
Drug overdose (tricyclic antidepressants, phenytoin, carbamazepine, opiates, barbiturates)
What are the causes of bilateral ptosis with normal pupils?
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)
What are the causes of a third nerve palsy?
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
What are the causes of mononeuritis multiplex?
DM PAN / GPA / EGPA SLE / RA / Sjogren's Sarcoidosis / Amyloidosis Lymphoma / Carcinoma Lyme disease Leprosy
Which drugs are associated with tremor?
Beta agonists
Ciclosporin and tacrolimus
Anticonvulsants
Lithium
Which disorders are associated with trinucleotide repeats?
Huntington's Disease Myotonic Dystrophy Fragile X Friedrich's ataxia Spinocerebellar ataxia
What is the DDx for motor neuropathy?
CIDP / GBS Lead Diphtheria Porphyria Dapsone
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
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
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
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
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
Which disorders can cause Lhermitte’s sign?
MS
Cervical myelopathy
Cervical cord tumour
Subacute combined degneration of the cord (B12 deficiency)
What is the DDx for CSF oligoclonal bands?
MS NMO SAH GBS SLE Behcet's disease CNS lymphoma Meningoencephalitis Neurosarcoidosis Neurosyphilis
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)
What is the treatment ladder in myasthenia gravis?
Pyridostigmine +/- thymectomy
Prednisone + azathioprine
Mycophenolate
Rituximab
Alternatives: MTX, tacrolimus, cyclosporin
Which medications should be avoided in myasthenia gravis?
Muscle relaxants Penicillamine Amonoglycosides Fluoroquonilones Macrolides
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
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
