Neurology Flashcards

(292 cards)

1
Q

What are some features of a resting tremor?

A

Worse at rest
Slow tremor (freq 3-5Hz)
Pill rolling of thumb over finger

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2
Q

What are some features of a postural tremor?

A

Worse if arms outstretched

Rapid (8-12Hz)

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3
Q

What are some causes of a postural tremor?

A

Anxiety, hyperthyroidism, alcohol, drugs, benign essential tremor

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4
Q

Describe the MRC muscle power score:

A

0 - no contraction
1 - flicker of contraction
2 - active movement, with gravity eliminated
3 - active movement against gravity
4 - active movement against gravity and resistance
5 - normal power

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5
Q

What nerve roots are tested in biceps reflex?

A

C5/6

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6
Q

What nerve root is tested in triceps reflex?

A

C7

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7
Q

What nerve root is tested in supinator reflex?

A

C6

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8
Q

What nerve roots are tested in knee jerk reflex?

A

L3/4

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9
Q

What nerve roots are tested in ankle jerk reflex?

A

L5, S1

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10
Q

What nerve roots are tested in plantar reflex?

A

L5, S1/2

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11
Q

In which dermatome would you find the clavicles?

A

C3-4

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12
Q

In which dermatome would you find the thumb?

A

C6

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13
Q

In which dermatome would you find the middle finger?

A

C7

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14
Q

In which dermatome would you find the little finger?

A

C8

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15
Q

In which dermatome would you find the nipple?

A

T4

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16
Q

In which dermatome would you find the umbilicus?

A

T10

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17
Q

In which dermatome would you find the big toe?

A

L5

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18
Q

In which dermatome would you find the lateral side of the foot?

A

S1

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19
Q

How would you examine CN I?

A

Test ability of each nostril to distinguish familiar smells

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20
Q

How would you examine CN II?

A

Snellen chart, visual fields, pupil size, shape, symmetry
Direct and consensual light reflex, accommodation, swinging light test
Fundoscopy

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21
Q

How would you examine CN III, IV and VI?

A

Eye movements

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22
Q

How would you examine CN V?

A

Open mouth, move side to side, clench teeth
Check sensation in ophthalmic, maxillary and mandibular divisions
Corneal reflex

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23
Q

How would you examine CN VII?

A

Raise eyebrows, smile, puff out cheeks, shut eyes tight

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24
Q

How would you examine CN VIII?

A

Repeat whispered number, Weber’s and Rinne’s

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25
How would you examine CN IX and X?
Say ‘ah’, gag reflex, cough
26
How would you examine CN XI?
Shrug shoulders and turn head against resistance
27
How would you examine CN XII?
Tongue movement
28
What are some diseases than can affect the CNs (generally)?
DM, stroke, MS, tumours, sarcoid, vasculitis
29
What might causes a CN I lesion?
Trauma, resp infections, meningitis, frontal lobe tumour
30
What are some causes of bitemporal hemianopia?
Pituitary adenoma (UQ>LQ) Craniopharyngioma (LQ>UQ) ICA aneurysm
31
What are some causes of homonymous hemianopia?
Stroke, abscess, tumour
32
What are some features of optic neuritis?
Pain on moving eye, loss of central vision, RAPD, disc swelling, central scotoma, colour desaturation
33
How would a pt with a CN III lesion present?
Ptosis, large pupil, eye down and out
34
What are some causes of a CN III lesion with pupillary sparing?
Diabetes, HTN, GCA, syphilis
35
What are some causes of a CN III lesion with pupillary involvement?
PICA aneurysm, RICP, tumours
36
How would a pt with a CN IV lesion present?
Diplopia on looking down and in
37
What are some causes of a CN VI lesion?
MS, Wernicke’s encephalopathy, false localising sign in RICP, pontine stroke
38
What is the difference between an UMN and LMN lesion of CN VII?
UMN spares forehead
39
What are some causes of an UMN lesion of CN VII?
Stroke, tumour
40
What are some causes of an LMN lesion of CN VII?
Bell’s, otitis media, cerebellopontine angle tumours e.g. acoustic neuroma, malignant parotid tumour, RH syndrome
41
What are some causes of a CN VIII lesion?
Noise damage, Meniere’s, herpes zoster, acoustic neuroma
42
What can cause dilated pupils?
CN III lesions, mydriatic drugs, cocaine
43
What can cause constricted pupils?
Old age, Horner’s, opiates, miotics
44
Describe the pathway for the pupillary light reflex:
Optic nerve -> superior colliculus -> Edinger-Westphal nuclei bilaterally -> parasympathetic on CNIII causes constriction
45
Describe relative afferent pupillary defect:
Incomplete damage to afferent pathway | Affected pupil will paradoxically dilate when light is moved from normal eye to abnormal eye
46
What is accommodation (eye)?
Pt first looks at distant object then at close object and eye converge and pupils constrict
47
What is Holmes-Adie pupil?
Affected pupil is normally moderately dilated and is poorly reactive to light Slowly reactive to accommodation Damage to PS fibres innervating sphincter pupillae
48
What are some causes of Holmes-Adie pupil?
Usually benign | Lyme disease, syphilis, parvovirus, HSV
49
What is Argyll Robertson pupil?
Pupil is constricted and unreactive to light but reacts to accommodation
50
What is the main cause of Argyll Roberston pupil?
Neurosyphilis
51
What is Hutchinson's pupil?
Pupil on side of lesion first constricts then widely dilates | Due to rapidly rising unilateral intracranial pressure
52
What is internuclear ophthlamoplegia?
Lesion disrupts communication by MLF causing weakness in adduction of ipsilateral eye with nystagmus of contralateral eye when abducting
53
What are some causes of ptosis?
CNIII lesions (compleyte) Sympathetic paralysis (partial) MG Congenital
54
What are some features of UMN lesions?
Spasticity - hypertonia and clasp knife reflex Hyperreflexia Plantars are upgoing (+ve Babinski sign) Extensors affected in arm, flexors in leg
55
What are some features of LMN lesions?
Affected muscles show wasting +/- fasciculation Hypotonia/flaccidity Hyporeflexia or areflexia
56
What sensations are spinothalamic tracts responsible for?
Pain and temperature
57
What sensations are dorsal columns responsible for?
Joint-position and vibration | Two-point discrimination, light touch
58
What is meant by a level in cord lesions?
Sensory, motor and reflex level (e.g. power unaffected above lesion, LMN at lesion, UMN below)
59
What are some features of temporal lobe lesions?
Wernicke’s aphasia Superior homonymous quadrantanopia Amnesia Inexplicable phenomena
60
What are some features of frontal lobe lesions?
Hemiparesis Personality change Broca’s aphasia Executive dysfunction
61
What are some features of parietal lobe lesions?
``` Hemisensory loss Astereognosis Sensory inattention Dysphasia Apraxia Inferior homonymous quadrantanopia ```
62
What are some features of occipital lobe lesions?
Homonymous hemianopia with macula sparing Polyopia (seeing multiple images) Palinopsia (persisting images after stimulus)
63
What are some features of cerebellar lesions?
``` Dysdiadochokinesia Ataxia Nystagmus Intention tremor Slurred speech Hypotonia ```
64
What are some features of cerebellopontine angle lesions?
``` Ipsilateral deafness Nystagmus ↓corneal reflex Ipsilateral cerebellar signs Papilloedema 7th nerve palsy ```
65
In dorsal column lesions, which side will signs appear?
Ipsilateral below lesion
66
In spinothalamic tract lesions, which side will signs appear?
Contralateral below lesion
67
What are some red flag signs associated with headache?
Thunderclap (SAH) Unilateral with eye pain (cluster, glaucoma) Cough initiated/worse in morning (RICP) Persisting with tender scalp and jaw claudication (GCA) Meningism (meningitis, SAH) Immunosuppressed
68
What drugs can cause medication overuse headache?
Analgesics and triptans
69
How are cluster headaches more common in?
Males, smokers
70
What are the presenting features of cluster headaches?
Rapid-onset of excruciating pain around one eye that may become watery and bloodshot with lid swelling and miosis
71
How long do cluster headaches last for (duration of headache and clusters)?
15-180min | Occur once or twice a day, and often nocturnal Clusters last 4-12w
72
What is the acute treatment for cluster headaches?
100% O2 for 15 mins | Sumatriptan SC at onset
73
What are some preventive measures or treatments for cluster headaches?
Avoid triggers such as alcohol | Corticosteroids, verapamil, lithium
74
What are the features of trigeminal neuralgia?
Intense, stabbing pain lasting seconds in trigeminal | distribution
75
What can trigger trigeminal neuralgia?
Washing area, shaving, eating, talking
76
What are some secondary causes of trigeminal neuralgia?
Compression of trigeminal root by aneurysmal intracranial vessels Tumour Chronic meningeal inflamm, MS, zoster
77
What is the management for trigeminal neuralgia?
Carbamazepine | Lamotrigine, phenytoin or gabapentin
78
What are the features of migraine?
Visual/other aura lasting 15-30 min followed within 1h by unilateral, throbbing headache (4-72h) During headache: N+V, photo/phonophobia
79
How can the aura present in migraine?
Visual – jumbling of lines, dots, zigzags, scotomata, hemianopia Paraesthesia, dysarthria, ataxia, hemiparesis, dysphasia
80
What are some partial triggers for migraine?
CHOCOLATE | Chocolate, Hangovers, Orgasms, Cheese, Oral contraceptives, Lie-ins, Alcohol, Travel, Exercise
81
How should a migraine attack be managed?
Oral triptan with NSAID/paracetamol
82
What is the prophylactic treatment for migraine?
Propranolol, topiramate
83
What causes vasovagal syncope?
Occurs due to reflex bradycardia ± peripheral vasodilation provoked by emotion, pain or standing too long
84
What are some pre-syncopal symptoms in vasovagal syncope?
Nausea, pallor, sweating, narrowing of vf
85
What causes carotid sinus syncope?
Hypersensitive baroreceptors cause excessive reflex | bradycardia on minimal stimulation (e.g. head-turning, shaving)
86
What are some symptoms of spinal cord compression?
Bilateral leg weakness, a sensory level ± preceding back pain Normal findings above level, LMN at level, UMN below
87
What are some causes of spinal cord compression?
Secondary malignancy, cervical disc prolapse, | haematoma (warfarin), myeloma, abscess
88
How should spinal cord compression be managed?
Urgent dex, followed by chemo/RT if cancer
89
What are the features of conus medullaris lesions?
Mixed UMN/LMN signs | Leg weakness, early urinary retention and constipation, back pain, sacral sensory disturbance and erectile dysfunction
90
What are the features of cauda equina lesions?
Back pain, radicular pain down legs, areflexic paralysis of legs, sensory loss in root distribution, ↓sphincter tone
91
What can cause unilateral foot drop?
DM, common peroneal nerve palsy, stroke, prolapsed disc, MS
92
What is an ataxic gait?
Wide based, falls, cannot walk heel to toe
93
What can cause an ataxic gait?
Cerebellar lesions (MS, tumours, alcohol, phenytoin) Proprioceptive sensory loss (sensory neuropathy, ↓B12)
94
Describe an intention tremor and its cause:
Irregular, large amplitude, worse at end of purposeful acts | Cerebellar damage
95
Describe a re-emergent tremor and its cause:
Postural tremor developing after delay of 10s e.g. PD
96
What is chorea?
Non-rhythmic, jerky, purposeless movements flitting from one place to another e.g. raising shoulders, flexing/extending fingers
97
What can cause chorea?
Huntington’s, Sydenham’s chorea
98
What is athetosis?
Slow, confluent, purposeless movements
99
What is myoclonus?
Sudden involuntary focal or general jerks
100
How does tardive dyskinesia present and what is commonly the cause?
Vacuous chewing and grimacing movements
101
What are some examples of acute dystonias?
Torticollis (head pulled back) Trismus (oromandibular spasm) Oculogyric crisis (eyes drawn up)
102
What is the Circle of Willis?
Anastomotic ring at base of brain fed by internal carotids and basilar artery
103
What parts of the brain are supplied by the anterior cerebral artery?
Supplies frontal and medial part of cerebrum
104
What parts of the brain are supplied by the middle cerebral artery?
Supplies lateral part of each hemisphere
105
What parts of the brain are supplied by the posterior cerebral artery?
Supplies occipital lobe
106
What are the features of lateral medullary syndrome?
Ipsilateral facial pain, temp loss, Horner’s, ataxia + nystagmus Contralateral limb/torso pain and temp loss
107
What vessels can be affected to give rise to lateral medullary syndrome?
PICA or vertebra
108
Which vessel is affected to give rise to lateral pontine syndrome?
AICA
109
What are the features of Weber's syndrome?
Ipsilateral CNIII palsy and contralateral hemiplegia
110
Which vessel is affected to give rise to Weber's syndrome?
PCA branches to midbrain
111
What is subclavian steal syndrome?
Subclavian artery stenosis proximal to origin of vertebral artery may cause blood to be stolen by retrograde flow down this vertebral artery into the arm causing brainstem ischemia typically after use of arm
112
What are the features of a subarachnoid haemorrhage?
Sudden onset excruciating headache, typically occipital, ‘thunderclap’ Vomiting, collapse, seizures, coma often follow Meningism: neck stiffness, Kernig’s sign
113
What are the main causes of a subarachnoid haemorrhage?
``` Berry aneurysm rupture (80%) AV malformation (15%) ```
114
Where are berry aneurysms often located?
Junctions of pos com with ICA, ant com with ACA or bifurcation of MCA
115
What are some risk factors for subarachnoid haemorrhage?
Previous aneurysmal SAH, smoking, alcohol, HTN, bleeding disorders, FH
116
What conditions are associated with berry aneurysms?
PKD, CoA and Ehlers-Danlos
117
What investigations should be performed if subarachnoid haemorrhage is suspected?
Urgent CT head LP (>12h after onset to see xanthochromia) CT angio to identify multiple aneurysms
118
What is the management for subarachnoid haemorrhage?
Maintain cerebral perfusion through hydration Nimodipine reduces vasospasm Surgery: endovascular coiling v surgical clipping
119
What are some complications of subarachnoid haemorrhage?
Rebleeding (20%), cerebral ischemia, hydrocephalus, | hyponatremia
120
What are some features of dural venous sinus thrombosis (general)?
Symptom onset is gradual (days or weeks) | Headache, vomiting, seizures, poor vision, papilloedema, focal CNS signs
121
What are some causes of intracranial venous thrombosis?
Pregnancy, COCP, head injury, dehydration, tumours, recent LP
122
What is the management for intracranial venous thrombosis?
Anticoag with LMWH then warfarin | Endovascular thrombolysis/thrombectomy
123
What is the cause of a subdural haematoma?
Bleeding from bridging veins between cortex and venous sinuses resulting in accumulating haematoma between dura and arachnoid
124
Untreated, what are the consequences of a subdural haematoma?
↑ICP, tentorial herniation and coning
125
What are some risk factors for a subdural haematoma?
Trauma (can be minor and long ago), falls Low ICP Elderly Anticoag
126
What are some presenting features of a subdural haematoma?
Fluctuating level of consciousness, sleepiness, headache, personality change, unsteadiness, seizures
127
Describe what the imaging will show in a subdural haematoma:
Crescent/sickle shaped collection of blood If acute = hyperdense (bright) If chronic = hypodense (dark)
128
What is the management for a subdural haematoma?
Reverse clotting abnormalities | Evacuation by craniotomy or burr hole washout
129
What is the cause of an extradural haematoma?
Traumatic skull fracture (temporal/parietal), causing laceration of middle meningeal artery and vein Blood accumulates between bone and dura
130
What are some presenting features of a extradural haematoma?
Lucid interval (hours to days), ↓GCS, RICP, increasingly severe headache, vomiting, confusion, seizures
131
Describe what the imaging will show in a extradural haematoma:
Biconvex/lens shaped haematoma
132
What is the management for a extradural haematoma?
Clot evacuation ± ligation of bleeding vessel
133
What is epilepsy?
Recurrent tendency to spontaneous, intermittent, abnormal electrical activity in part of brain, manifesting as seizures
134
What are some symptoms that may be experienced in the post-ictal state?
Headache, confusion, myalgia, temporary weakness, dysphasia
135
How is epilepsy diagnosed?
History, establish type of seizure, rule out provoking causes EEG, MRI
136
What advice in regards to driving should be given to those diagnosed with epilepsy?
Patient must contact DVLA and avoid driving until seizure-free for >1y
137
What are the two main classifications of seizures?
Focal and generalised
138
What are the main types of generalised seizures?
Absence Tonic-clonic Myoclonic Atonic
139
What is the difference between generalised and focal seizures?
Focal: originating within networks linked to one hemisphere Generalised: simultaneous onset of widespread electrical discharge with no localising feature
140
Lip smacking and deja vu as part of seizures are localising features to which lobe of the brain?
Temporal
141
What is Jackonsian March and which brain lobe is this localised to?
Spreading motor seizures starting with face or thumb | Frontal lobe
142
What are the first line medications for focal seziures?
Carbamazepine or lamotrigine
143
What are the first line medications for tonic-clonic seziures?
Valproate or lamotrigine
144
What are the first line medications for absence seziures?
Valproate or ethosuximide
145
What is the first line medication for myoclonic seziures?
Valproate
146
When and how should anti-epileptics be stopped?
If pt is seizure free for >2y | Dose must be slowly decreased over 3-6m
147
What are some side effects of carbamazepine?
Leukopenia, diplopia, blurred vision, impaired balance, drowsiness, rash, SIADH
148
What type of seizures may be exacerbated by carbamazepine?
Absence and myoclonic seizures
149
What are the side effects of lamotrigine?
Rash (0.1% SJS), blurred vision, photosensitivity
150
What are some side effects of sodium valproate?
Teratogen, nausea, liver failure, pancreatitis, hair loss, oedema, ataxia
151
What are some side effects of phenytoin?
↓intellect, depression, acne, gingival hyperplasia, polyneuropathy
152
What is the extrapyramidal triad of Parkinonism?
Tremor Hypertonia Bradykinesia
153
What are some causes of Parkinsonism?
``` Parkinson’s disease Progressive supranuclear palsy Multi-system atrophy Cortico-basal degeneration Lewy body dementia Drugs (metoclopramide, neuroleptics) ```
154
What is the pathophysiology of Parkinson's disease?
Loss of dopaminergic neurons in substantia nigra, associated with Lewy bodies in basal ganglia, brainstem and cortex
155
What are some features of Parkinson's disease?
Triad Autonomic dysfunction (postural hypotension, constipation, urinary frequency) Sleep disturbance Depression, dementia and psychosis
156
Are Parkinson's disease signs typically asymmetrical or symmetrical?
Asymmetrical
157
Why should starting levodopa sometimes be delayed?
Efficacy reduces over time, requiring larger doses with greater SEs
158
What are the consequences of sudden withdrawal of Parkinson's disease meds?
Acute akinesia and neuroleptic malignant syndrome
159
What is co-prescribed with levodopa?
Dopa-decarboxylase inhibitor as co-careldopa
160
What are some side effects of levodopa?
Dyskinesia, painful dystonia, psychosis, visual hallucinations, N+V, postural hypotension, arrhythmias
161
What is an example of a dopamine agonists used in treatment of PD?
Ropinirole
162
What are some side effects of dopamine agonists?
Drowsiness, nausea, hallucinations, compulsive behaviour
163
What other drugs may be used in the management of Parkinson's disease (not levodopa)?
Apomorphine Anticholinergics (younger pts) MAO-B inhibitors (rasagiline) COMT inhibitors
164
What are some features of progressive supranuclear palsy?
Early postural instability, vertical gaze palsy, rigidity of trunk > limbs, symmetrical onset, speech and swallowing problems
165
What are some features of multi-system atrophy?
Early autonomic features e.g. incontinence, postural BP drop | Cerebellar + pyramidal signs, rigidity > tremor
166
What are some features of cortico-basal degeneration?
Akinetic rigidity involving one limb, cortical sensory loss (e.g. astereognosis), apraxia
167
How is multiple sclerosis defined?
Inflammatory plaques of demyelination in CNS disseminated in space and time - occurring at multiple sites, with >30d between attacks
168
What is the median age of onset of MS?
30y
169
What is Uhthoff's phenomenon (think MS)?
Symptoms may worsen with heat
170
What is Lhermitte's sign?
Electric sensation through back and limbs on flexing neck
171
What are some common presenting features of MS?
``` Unilateral optic neuritis Pins and needles Spastic weakness Urinary retention, incontinence Fatigue Speech and swallowing problems ```
172
How is MS diagnosed using the McDonald criteria?
2 or more attacks - must last >1h with >30d between attacks | 2 or more lesions
173
How does MS progress?
Most relapsing-remitting course with initial recovery between relapse. With time, remission becomes incomplete, so disability accumulates 10% display steadily progressive disability in absence of relapses (primary progressive MS)
174
What are some poor prognostic signs in MS?
Older male, motor signs at onset, many early relapses, many MRI lesions, axonal loss
175
What lifestyle changes can be made to help with the management of MS?
Regular exercise, stop smoking, avoid stress
176
What are some disease modifying drugs used in MS?
Beta-interferon, dimethyl fumarate
177
What drug can be used in treating relapses in MS?
IV methylpred
178
What are some features associated with SOLs?
RICP Seizures Evolving focal neurology Subtle personality changes
179
What are the causes of SOLs?
Tumour, aneurysm, abscess, chronic subdural, cysts
180
Give some examples of primary brain tumours?
Astrocytoma, glioblastoma, oligodendroma, ependymoma, meningioma, medulloblastoma, lymphoma
181
What treatments can form part of the management plan for brain tumours?
``` Surgery if accessible RT/chemo Ventriculo-peritoneal shunt Seizure prophylaxis Dex or IV mannitol ```
182
What is the triad of features seen in normal pressure hydrocephalus?
Urinary incontinence, dementia, gait abnormality (similar to PD)
183
What is the treatment for normal pressure hydrocephalus?
Ventriculoperitoneal shunting
184
What are some presenting features of idiopathic intracranial hypertension?
Narrowed visual fields, blurred vision, 6th nerve palsy, enlarged blind spot if papilloedema
185
What is the typical patient presenting with idiopathic intracranial hypertension?
Obese females in 3rd decade
186
What are the management options for idiopathic intracranial hypertension?
Weight loss Acetazolamide or topiramate, loop diuretics, pred LP Optic sheath fenestration or lumbar-peritoneal shunt if drugs fail
187
Where is the lesion in Wernicke's aphasia?
Superior temporal gyrus
188
What are the features of Wernicke's aphasia?
Sentences that make no sense, word substitution | and neologisms but speech remains fluent
189
Where is the lesion in Broca's aphasia?
Inferior frontal gyrus
190
What are the features of Broca's aphasia?
Speech is non-fluent, laboured, and halting
191
What area connects Wernicke's and Broca's area?
Arcuate fasiculus
192
What are the features of Bell's palsy?
Abrupt onset with complete unilateral facial weakness at 24-72h Ipsilateral numbness around ear, ageusia, hypersensitivity to sounds Unable to wrinkle forehead
193
What are some infective causes of FN palsy?
Ramsay-Hunt, Lyme disease, meningitis, TB, HIV
194
What are some neurological causes of FN palsy?
Stroke, tumour, MS | Acoustic neuroma, meningioma
195
What are systemic causes of FN palsy?
DM, sarcoid, Guillain-Barre
196
What is the management for Bell's palsy?
Pred within 72h Protect eye – dark glasses, artificial tears, tape to close eyes at night
197
What is Ramsay-Hunt syndrome?
Latent VZV reactivating in geniculate ganglion of 7th CN
198
What are some features of Ramsay-Hunt syndrome?
Painful vesicular rash on auditory canal, pinna, tongue, palate Ipsilateral facial palsy, loss of taste, vertigo, tinnitus, dry mouth and eyes
199
How should Ramsay-Hunt syndrome be treated?
Antivirals and pred
200
What can cause brachial plexus injury?
Trauma, RT, prolonged wearing of heavy rucksack, cervical rib, thoracic outlet compression
201
What investigations may be performed if polyneuropathy is suspected?
FBC, glucose, U+Es, LFTs, TSH, B12, electrophoresis, ANA, ANCA, CXR, urinalysis
202
What are the features of sensory neuropathies?
Numbness, pins + needles, affects glove and stocking | Difficulty handling small objects
203
What are the features of motor neuropathies?
Often progressive, weak/clumsy hands, difficulty walking, difficulty breathing, wasting in distal muscles of hand and feet, hyporeflexia
204
What are some causes of polyneuropathy?
DM, renal failure, Guillain-Barre, sarcoid, B12 + folate def., Charcot-Marie-Tooth
205
What drugs can causes polyneuropathy?
Vincristine, cisplatin, isoniazid, nitrofurantoin, phenytoin, metronidazole, amiodarone
206
What are some causes of autonomic neuropathy?
DM, amyloidosis, GB, Sjogren’s, HIV, SLE, Lambert-Eaton
207
What is the median age of onset of motor neuron disease?
60y
208
What is the pathophysiology of motor neuron disease?
Selective loss of neurons in motor cortex, CN nuclei and anterior horn cells UMN and LMN can be affected but no sensory loss and doesn’t affect eye movements
209
What are the 4 clinical patterns seen in motor neuron disease?
Amyotrophic lateral sclerosis (80%): combined UMN and LMN signs Progressive bulbar palsy (10-20%): only affects CN IX-XII Progressive muscular atrophy (<10%): LMN signs only Primary lateral sclerosis (rare): UMN signs
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How can motor neuron disease present?
Stumbling spastic gait, foot drop, weak grip and shoulder abduction, or aspiration pneumonia
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What is the only drug that improves survival in motor neuron disease and what's its MoA?
Riluzole: glutamate and NMDA antagonist
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What is bulbar palsy?
Diseases of nuclei of CN IX-XII in medulla | LMN lesion
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What are the features of bulbar palsy?
Flaccid fasciculating tongue, absent jaw jerk and quiet speech
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What are the causes of bulbar palsy?
MND, Guillain-Barre, MG, syringobulbia, brainstem tumours
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What is corticobulbar palsy?
UMN lesion of muscles of swallowing and talking due to bilateral lesions above the mid-pons e.g. corticobulbar tracts
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What are the features of corticobulbar palsy?
Slow tongue movements, slow deliberate speech, jaw jerk hypereflexia, unprovoked weeping
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What are the causes of corticobulbar palsy?
MS, MND, stroke, central pontine myelinolysis
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What is the pathophysiology of cervical spondylosis?
Degen of annulus fibrosus combined with osteophyte formation leads to narrowing of spinal canal and intervertebral foramina As neck flexes and extends, cord is dragged over bony spurs
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How can cervical spondylosis present?
Neck stiffness, crepitus on moving neck, stabbing/dull arm pain, forearm/wrist pain
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How should cervical spondylosis be managed?
Urgent MRI, analgesia, cervical collars | Neurosurgery if no improvement in 4-6w
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What is the pathophysiology of myasthenia gravis?
Autoimmune disease mediated by antibodies to nicotinic ACh receptor on postsynaptic side of NMJ
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What are some presenting features of myasthenia gravis?
Ptosis, diplopia, weakness at end of day, myasthenic snarl on smiling, ‘peek sign’
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What can exacerbate myasthenia gravis?
Pregnancy, hypokalaemia, infection, exercise
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What investigations should be performed if myasthenia gravis is suspected?
Antibodies: ↑anti-AChR antibodies in 90% | EMG, CT (exclude thymoma)
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What is the management for myasthenia gravis?
Anti-cholinesterase e.g. pyridostigmine Treat relapses with pred Thymectomy if required
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What is a myasthenic crisis?
Life-threatening weakness of resp. muscles during a relapse
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How should myasthenic crisis be managed?
Monitor FVC, vent support | Plasmapheresis or IV Ig and treat trigger
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What is the pathophysiology of Lambert-Eaton syndrome?
Antibodies are to voltage gated Ca2+ channels on pre-synaptic membrane
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What are some features of Lambert-Eaton syndrome?
Gait difficulty, autonomic (dry mouth, constipation), hyporeflexia and weakness that improves after exercise
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What is the management for Lambert-Eaton syndrome?
Pyridostigmine, 3,4-diaminopyridine, IV Ig | Regular CXR/CT
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What are the features of syringomyelia?
Absent pain and temp sensation (cape-like distribution) with preserved DC senses Wasting of hands, Horner’s
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What are the features of temporal arteritis?
Headache, temporal artery and scalp tenderness, jaw claudication, sudden unilateral blindness, morning stiffness
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What investigations should be performed if temporal arteritis is suspected?
CRP, plasma viscosity ↑↑, ↑platelets, ↑ALP, ↓Hb Temporal artery biopsy within 14d of starting steroids
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What is the management for temporal arteritis?
Pred immediately | IV methylpred if evolving visual loss or history of amaurosis fugax
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What is the triad in Horner's syndrome?
Miosis, partial ptosis and anhidrosis
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What conditions can cause Horner's syndrome?
``` Demyelination Vascular disease Syringomyelia Pancoast’s tumour Carotid dissection ```
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What is Guillain-Barre syndrome?
Acute inflammatory demyelinating polyneuropathy | Few weeks after an infection, symmetrical ascending muscle weakness starts
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What are some triggers for Guillain-Barre syndrome?
Campylobacter, CMV, mycoplasma, zoster, HIV, EBV
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What are the diagnostic features of Guillain-Barre?
Progressive weakness >1 limb and areflexia | Progression up to 4w
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What is the management for Guillain-Barre?
IV Ig or early plasmapheresis | Spirometry, vent support
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What condition presents similarly to Guillain-Barre but progresses over a time period greater than 4 weeks?
Chronic inflammatory demyelinating polyradiculopathy (CIDP)
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What neurological signs are seen in subacute degeneration of the cord?
Combination of DC loss causing sensory and LMN signs and corticospinal tract loss causing motor and UMN signs
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What is the classic reflex triad seen in subacute degeneration of the cord?
Extensor plantars, absent knee jerks and absent ankle jerks
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What is the classical triad seen in Wernicke's encephalopathy?
Confusion, ataxia and ophthalmoplegia
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How is Wernicke's encephalopathy managed?
Thiamine (Pabrinex) IV/IM then oral supplements
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What is the consequence of Wernicke's encephalopathy?
Korsakoff’s syndrome (amnesia and confabulation)
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What is the cause of Creutzfeldt-Jakob disease?
Prion, a misfolded form of a normal protein that can transform other proteins into prion proteins
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What are some signs of Creutzfeldt-Jakob disease?
Progressive dementia, focal CNS signs, myoclonus, depression, complex visual disturbances, hallucinations
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How can variant CJD be distinguished from sporadic CJD?
``` Earlier age of presentation in vCJD (29y v 60y) Longer survival and later dementia Psych features are early signs Painful sensory symptoms commoner Normal EEG ```
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What are the features of complete cord transection syndrome?
Spinal shock and autonomic dysfunction with higher lesions Priapism Complete loss of sensation and paralysis below lesion
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What are some causes of complete cord transection syndrome?
Trauma, infarction, transverse myelitis, abscess, tumour
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What are the features of Brown-Sequard syndrome?
Ipsilateral loss of motor function, loss of proprioception, vibration, 2- point discrimination Contralateral loss of pain and temp sensation
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What are some causes of Brown-Sequard syndrome?
Penetrating trauma, fractured vertebrae, tumour, abscess, MS
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What are the features of anterior cord syndrome?
Flaccid paralysis below level, loss of pain/temp, autonomic dysfunction (based on level) Preserved dorsal columns
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What are some causes of anterior cord syndrome?
Flexion injury | Anterior spinal artery injury - atherosclerosis or iatrogenic secondary to cross-clamping of aorta intraop
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What are the features of central cord syndrome?
Motor > sensory, UL > LL, distal > proximal, bladder dysfunction and urinary retention
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What are some causes of central cord syndrome?
Trauma (hyperextension in elderly, hyperflexion in younger), cervical spinal stenosis, degenerative spinal disease, syringomyelia
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What are the features of posterior cord syndrome?
Loss of DCs but maintained motor function and pain/temp sensation
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What are some causes of posterior cord syndrome?
Spondylosis, spinal stenosis, infection, Vit B12 def, occlusion of paired posterior spinal arteries
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What is the general management for cord injuries?
``` Consider intubation (C5 or above), ICU, early immobilisation of C-spine, consider surgery ```
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How long must patients stop driving for after their first unprovoked seizure?
6m | 12m if structural abnormality or epileptiform activity on EEG
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After withdrawing anti-epileptics, how long must patients wait before driving?
6m after last dose
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How long must patients stop driving for after an explained syncopal episode?
4w
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How long must patients stop driving for after an unexplained syncopal episode?
6m
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How long must patients stop driving for after an multiple syncopal episodes?
12m
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Describe the result of electromyography in myasthenia gravis:
Diminished response to repetitive stimulation
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Describe the result of electromyography in Lambert-Eaton syndrome:
Incremental response to repetitive stimulation
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Describe the result of electromyography with regard to action potentials in neuropathy:
↑AP duration, ↑AP amplitude
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Describe the result of electromyography with regard to action potentials in myopathy:
↓AP duration, ↓AP amplitude
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What are the causes of RICP?
Tumours, head injury, haemorrhage, infection, hydrocephalus, cerebral oedema
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What are some features of RICP?
Headache (worse on coughing), vomiting, altered GCS, ↓visual acuity, papilloedema Cushing’s response (↓HR, ↑BP), Cheyne-Stokes respiration
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What investigations should be performed if RICP is suspected?
FBC, U+Es, LFTs, glucose, serum osmolality, clotting, blood culture, CT head
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What is the management for RICP?
Elevate head of bed Mannitol, dex Restrict fluid <1.5L/d Consider craniotomy or burr hole
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What is uncal herniation?
Lateral supratentorial mass pushes inferomedial temporal lobe (uncus) against midbrain
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What is tonsillar herniation?
Cerebellar tonsils through foramen magnum
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What is subfalcian herniation?
Cingulate gyrus (medial frontal lobe) is forced under falx cerebri
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What are some early features in meningitis?
Headache, fever, leg pain, cold peripheries, abnormal skin colour
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What are some features seen later in meningitis?
Neck stiffness, photophobia, Kernig’s sign, ↓GCS, seizures, non-blanching rash, shock
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What are some investigations that should be performed if meningitis is suspected?
U+Es, FBC, LFT, glucose, coagulation, swabs, CXR | CSF MC+S, protein, lactate, glucose, PCR
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What is the management for meningitis?
Ceftriaxone + amoxicillin if >60y or immunocompromised
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What are some features of encephalitis?
Bizarre behaviour, confusion, ↓GCS, fever, headache, focal neuro signs, seizures, infectious prodrome
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What are some causes of encephalitis?
Viral: HSV1/2, VZV, measles, mumps | Non-viral: any bacterial meningitis, TB
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What are some investigations that should be performed if encephalitis is suspected?
Blood culture, viral PCR, contrast-enhanced CT, LP, CSF PCR, EEG
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What is the management for encephalitis?
Start acyclovir within 30min of pt arriving for 14d
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What is status epilepticus?
Seizures lasting >30min or repeated seizures without intervening consciousness
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What is the management for status epilepticus?
Lorazepam slow IV bolus, if no response repeat after 10-20min Can also give rectal diazepam or buccal midazolam Then phenytoin infusion
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What are the features of cauda equina?
Lower back pain, urinary incontinence/retention, reduced sensation in perianal area, decreased anal tone
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What investigation is needed if cauda equina is suspected?
Urgent MRI
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What is the management for cauda equina?
Surgical decompression, often laminectomy
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What is the criteria for doing a head CT within 1h in regard to head injury?
GCS <13 on initial assessment, or <15 after 2h Focal neuro deficit Suspected open or depressed skull fracture or basal skull fracture Post-traumatic seizure Vomiting more than once
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What is the criteria for doing a head CT within 8h in regard to head injury?
Any LoC or amnesia AND 65+y, coagulopathy, high impact injury, retrograde amnesia of >30min
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What drugs can exacerbate myasthenia gravis?
Gent, opiates, tetracycline, beta blockers, quinine