Neurology Flashcards

1
Q

Unconsciousness / coma

A
  • Consciousness – state of wakefulness and awareness of self and surroundings
  • Coma – unarousable unresponsiveness
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2
Q

define vegetative state, minimally conscious syndrome and locked-in syndrome

A

Vegetative state
- widespread cortex damage, brainstem function normal

Minimally conscious syndrome
- some sentient behaviour, eg maybe vague pain perception

Locked-in syndrome
- unresponsiveness due to massive brainstem damage, functioning cortex

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3
Q

Brain-stem death

A

Neurological damage to brainstem resulting in irreversible loss of consciousness and loss of spontaneous ventilation

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4
Q

Pre-conditions for brain-stem death testing

A
  • GCS E1V1M1, mechanically ventilated with apnoea - deep coma
  • Proof that patient condition is due to irreversible, structural brain damage
  • Exclude reversible causes
  • No sedation
  • Normal electrolytes
  • Patient >2mo old
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5
Q

who can perform brain stem death testing?

A
  • 2 doctors of sufficient experience to perform testing - one consultant, the other fully qualified with full GMC registration for minimum 6yrs - neither to be member of transplant team (if considered)
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6
Q

how do you test for brainstem death?

A
  • Fixed pupils which do not respond to sharp changes in intensity of light
  • No corneal reflex
  • Absent oculo-vestibular reflexes - no eye movts in the caloric test - inject >50mls ice-cold water into each ear
  • No response to supraorbital pressure
  • No cough reflex to bronchial stimulation
  • No gag reflex to pharyngeal stimulation
  • No spontaneous ventilation effort with apnoea testing
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7
Q

stroke definiton

A

clinical syndrome consisting of rapid onset neurological deficit which is the result of a vascular lesion and is associated with infarction of central nervous tissue- CVA - sudden interruption in vascular supply of brain -> irreversible brain death

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8
Q

what is an ischaemic stroke? risk factors?

A
  • 85%, thrombo/embolus, also shock, vasculitis

RFs - age, HTN, smoking, high cholesterol, DM, AF, cOCP, FHx, carotid artery stenosis

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9
Q

what is a haemorrhagic stroke? risk factors?

A
  • 15%, intracerebral/subarach
  • RFs - age, HTN, AV malformation, anticoagulation
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10
Q

stroke Px - general

A
  • Sudden onset, asymmetrical
  • Limb weakness
  • Facial weakness
  • Dysphasia
  • Swallowing difficulties
  • Visual field defects (homonymous hemianopia)
  • Sensory loss
  • Ataxia, vertigo (posterior circulation infarct)
  • FAST - face, arms, speech, time
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11
Q

Oxford Stroke Classification

A

Assess for:
- unilateral hemiparesis +/- hemisensory loss of face, arm, leg
- homonymous hemianopia
- higher cognitive dysfunction, eg dysphasia
TACS / PACS / LACS / POCS

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12
Q

Total anterior circulation infarct (TACS)

A
  • ACA and MCA areas affected
  • All 3 of the following:
  • Unilateral weakness (and/or sensory deficit) of the face, arm and leg
  • Homonymous hemianopia
  • Higher cerebral dysfunction (dysphasia, visuospatial disorder)
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13
Q

Partial anterior circulation infarct (PACS)

A
  • Smaller arteries of anterior circulation

2 of:
- Unilateral weakness (and/or sensory deficit) of the face, arm and leg
- Homonymous hemianopia
- Higher cerebral dysfunction (dysphasia, visuospatial disorder)

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14
Q

Lacunar syndrome (LACS)

A
  • Arteries around internal capsule, thalamus, basal ganglia

1 of following
- Unilateral weakness +/- sensory deficit of face, arm, leg
- Pure sensory stroke
- Ataxic hemiparesis

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15
Q

Posterior circulation syndrome (POCS)

A
  • Vertebrobasilar arteries

1 of following
- Cerebellar / brainstem syndromes
- Loss of consciousness
- Isolated homonymous hemianopia

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16
Q

Brainstem infarct

A
  • quadriplegia
  • locked-in syndrome (basilar artery)
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17
Q

Lateral medullary syndrome (PICA) - Wallenberg

A
  • Ipsilateral - ataxia, nystagmus, dysphagia, facial numbness, CN palsy (Horner’s)
  • Contralateral - limb sensory loss
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18
Q

Weber syndrome - branches of PCA that supply midbrain

A
  • Ipsilateral CN3 palsy
  • Contralateral weakness of upper + lower limbs
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19
Q

Anterior inferior cerebellar artery (AICA) - lateral pontine syndrome

A
  • Similar to Wallenberg’s but:
  • Ipsilateral - facial paralysis and deafness
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20
Q

Retinal/ophthalmic artery?

A
  • amaurosis fugax
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21
Q

Stroke by areas: ACA, MCA, PCA

A

ACA
- Contralateral hemiparesis + sensory loss, lower limbs > upper limbs

MCA
- Contralateral hemiparesis + sensory loss, upper > lower
- Contralateral homonymous hemianopia
- Aphasia

PCA
- Contralateral homonymous hemianopia with macular sparing
- Visual agnosia – unable to recognise objects

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22
Q

Stroke Ix

A
  • ROSIER tool
  • NIHSS score
  • non-contrast CT head - low density areas of ischaemia
  • MRI head
  • CT / MR angio
  • BMs - exclude hypo
  • carotid US/CT/MRI
  • ECG - AF
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23
Q

stroke initial Mx?

A
  • Exclude hypoglycaemia
  • Immediate CT brain to exclude haemorrhage
  • Aspirin 300mg daily for two weeks (started after haemorrhage is excluded with a CT)
  • Admission to a specialist stroke centre

thrombolysis - alteplase. if <4.5 hrs since onset of symptoms

thrombectomy - < 6 hours. considered if confirmed blockage of the proximal anterior circulation or proximal posterior circulation

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24
Q

stroke secondary prevention?

A
  • Clopidogrel 75mg once daily (alternatively aspirin plus dipyridamole)
  • Atorvastatin 20-80mg (not started immediately – usually delayed at least 48 hours)
  • Blood pressure and diabetes control
  • Addressing modifiable risk factors (e.g., smoking, obesity and exercise)
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25
carotid artery assessment post TIA or stroke ?
Carotid endarterectomy - if stenosis >70% - consider angioplasty / stenting
26
patient found to have AF after TIA/stroke ?
- warfarin / DOAC - start immediately in TIA - wait 2wks in acute stroke
27
Stroke mx of other issues
Fluids - assess hydration - oral hydration, IV saline (no dextrose) Glycaemic control - monitor BMs - aim for 4-11 - IV insulin / glucose infusions for diabetics BP Mx - use anti-HTN if hypertensive emergency - IV labetalol, nicardipine.... Feeding - screen for safe swallow - NG feed - nasal bridle tube / gastrostomy - assess meds for NG feed - nutritional support Disability - Barthel index to measure functional decline
28
TIA?
- transient episode of neurological dysfunction caused by focal brain/spinal cord/retinal ischaemia, without acute infarction
29
crescendo TIA?
2+ TIAs in 1wk - high risk of stroke
30
TIA Px
- Typically resolve in 1hr - Unilateral weakness / sensory loss - Aphasia / dysarthria - Ataxia, vertigo, loss of balance - Visual problems, amaurosis fugax, diplopia, homonymous hemianopia
31
TIA Ix
- CT - MRI, inc diffusion-weighted / blood-sensitive - Carotid imaging - doppler, CT/MR
32
TIA Mx
- aspirin 300mg - don't drive until seen by specialist - clopidogrel / aspirin + dipyridamole - carotid artery endarterectomy if stenosis >70% - atorvastatin 80mg Specialist review - If >1 TIA / suspected cardioembolic source / severe carotid stenosis - ?admission / observation with stroke specialist - Suspected TIA in last 7d – specialist review in 24hrs - Suspected TIA >7d ago – specialist review asap within 7d
33
Extradural haemorrhage (EDH)
- blood between skull + dura - trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
34
EDH Px?
- head trauma - initial LOC / drowsiness - lucid interval, later low GCS - headache, N+V, confusion, seizures, focal neurology, fixed dilated pupil, deep coma
35
what is Cushing's reflex?
response to acutely raised ICP: - bradycardia - irregular respirations - HTN / increase in pulse pressure (systolic up, diastolic down)
36
EDH Ix
- bloods, inc G/S - non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
37
EDH Mx
- A-E Neuroprotection - 30deg tilt head up - oxygenation, loosen collars - intubate + ventilate - normostasis - normotension, normoglycaemia - hyperventilate if coning - IV mannitol - hypertonic saline (3%) - levetiracetam - seizures Neurosurgery - ICP bolt / EVD - burr hole / open craniotomy
38
Subdural haemorrhage (SDH)
- blood between dura + arachnoid layers - acute / subacute / chronic
39
SDH patho?
- rupture of bridging veins between cortex + sinus - infants (NAI), elderly/alcoholics (cerebral atrophy) - bleed forms haematoma - when this breaks down, proteins become soluble - haematoma expands, ICP rises
40
SDH Px
- Head injury - Lucid interval - can be days/wks/months - Personality change, sleepiness, unsteadiness - Fluctuation in consciousness, memory loss, cognitive impairment - Focal neuro deficit - Seizure, headache, coma, vomiting - Fixed dilated pupil
41
SDH Ix
- non-contrast CT head - hyperdense crescent (sickle) shaped collection - bloods etc
42
SDH Mx
- A-E - neuroprotection Neurosurgery - Burr hole / open craniotomy, clot evacuation
43
Subarachnoid haemorrhage (SAH)
bleed into subarachnoid space - between arachnoid / pia (where CSF is)
44
SAH causes?
Traumatic - most common cause of SAH Spont - Berry aneurysm - 85% spont cases - HTN, PKD, EDS, coarc aorta - AV malformation - pituitary apoplexy - mycotic aneurysms
45
SAH RFs
- 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan's, EDH, PKD
46
SAH Px
- sentinel headache - less severe in wks before px - sudden onset occipital headache - thunderclap - N+V - photophobia, neck stiffness, Kernig's, Brudzinski's - coma - seizures - papilloedema, fixed dilated pupil, Cushing's - maybe ST elevation
47
SAH Ix
Non-contrast CT head - bright star-shaped lesions - if CT head <6hrs of sx onset + normal - LP not indicated - CT less reliable >6hrs - so if normal - consider LP LP - >12hrs after sx onset - xanthrochromia - raised opening pressure - raised red cell count CT intracranial angiogram +/- catheter angiogram
48
SAH Mx
- nimodipine Neurosurgery - endovascular coiling - neurosurgical clipping - intracranial stents / balloons
49
SAH Cx
- rebleed - 20% - cerebral ischaemia - hydrocephalus - VP shunt / EVD - hyponatraemia (SIADH) - fluid restriction
50
Intracerebral haemorrhage
- bleed in brain tissue - spontaneous / secondary to ischaemic stroke, tumours, aneurysm rupture Px - similar to SAH - stroke, focal neurology, reduced GCS Ix - non-contrast CT head - hyperdensity in substance of brain
51
Head injury NICE criteria for CT head, >16yo
CT head <1hr if any of: - GCS 12 or lower on initial assessment - GCS <15 2hrs after injury - ?open / depressed skull fracture - Signs of basal skull fracture – haemotympanum, panda eyes, CSF oto/rhinorrhoea, Battle’s sign - Seizure - Focal neurology - 1+ episodes vomiting If none of the above, do CT head <8hrs of injury if there is LOC / amnesia, and any of: - >65yo - Bleeding / clotting disorders - Dangerous MOI - >30 mins retrograde amnesia of events just before injury If no RFs of any category above, CT head <8hrs of injury if anticoagulated: - Warfarin, DOACs, heparin, clopidogrel
52
dementia definition?
- irreversible, progressive decline of higher brain function - cognitive/behavioural sx - memory loss, reasoning/communication problems, change in personality, reduction in ADLs
53
RFx for dementia?
- older, mild cognitive impairment, genetics, Parkinson's, CVA, smoking, DM, sedentary, obesity
54
what is Alzheimer's dementia ?
- type of dementia (50-75%) - amyloid plaques / tau proteins in brain - reduce transmission, brain cell death - widespread cerebral atrophy, esp cortex / hippocampus
55
how does Alzheimer's present?
- Steady decline - Memory loss - Loss of executive function / dysphasia - Aphasia, apraxia, agnosia - Changes in planning, reasoning, speech, orientation
56
Alzheimer's Mx?
- wellbeing activities, cognitive stimulation therapy, group reminiscence therapy, cognitive rehab - 1st - AChE inhibitors - donepezil, galantamine, rivastigmine - 2nd - NMDA receptor antagonist - memantine - antipsychotics - if risk of harming themselves
57
vascular dementia?
- type of dementia (<20%) - Group of syndromes of cognitive impairment caused by ischaemia / haemorrhage secondary to cerebrovascular disease - affects white matter of cerebral hemispheres, grey nuclei, thalamus, striatum Types - stroke-related - multi/single-infarct - subcortical - small vessel disease - mixed dementia - VD + Alzheimer's
58
RFx for vascular dementia?
- previous stroke/TIA, older, AF, DM, lipids, smoking
59
vascular dementia Px?
- Stepwise progression - stability + acute decline - Cognitive impairment - Mood disturbance - psychosis, delusions, hallucinations, paranoia - Gait, speech, emotional, memory disturbance - Seizures - Focal neurolog
60
vascular dementia Ix?
- screen for depression, psychomotor retardation - formal cognitive screen - MRI - infarcts, white matter changes - Dx with NINDS-AIREN criteria
61
vascular dementia Mx?
- cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy - address pain, avoid overcrowding, clear communication
62
what is Lewy body dementia?
- type of dementia (10-15%), >50yo - lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
63
how does Lewy body present?
- progressive cognitive impairment - may fluctuate, early impairments to attention, executive function - sleep disorder - visual hallucinations - Parkinson's like sx - bradykinesia, resting tremor, rigidity
64
how is lewy body Mx?
- AChE - donepezil, rivastigmine - avoid neuroleptics (may develop Parkinsonism)
65
what is Frontotemporal dementia (FTD)?
- type of dementia (2%), <65yo - neuron damage/death at frontal/temporal lobes, atrophy due to tau protein deposition
66
FTD Px?
Behavioural - Change in behaviour / personality, eg disinhibited, impulsive, decline in social skills.. Semantic - Lack of understanding word meanings, difficulty in name retrieval… Non-fluent - Breakdown in speech, apraxia, decline in literac
67
FTD Mx
- maybe SSRIs / trazadone for behavioural sx
68
general management of dementia?
Cognitive impairment - Memory loss, difficulty with reasoning / communication / decisions, dysphasia, coordinating movements, not orientated Behavioural and psychological sx of dementia (BPSD) - Psychosis – delusions / hallucinations, agitation / emotional lability, depression / anxiety, motor disturbance, sleep disturbance Difficulties with ADLs - Household tasks, toileting eating, walking
69
Ddx of dementia
- CJD - HIV-related cognitive impairment / dementia - depression - normal pressure hydrocephalus - mild cognitive impairment - delirium - reversible causes - hypothyroid, Addison's, B12, folate, thiamine, syphilis, tumour, SDH, drugs,
70
dementia Ix
- cognitive screen - bloods: - Exclude reversible causes (eg hypothyroid) – FBC, U/E, LFTs, Ca, glucose, ESR/CRP, TFTs, vit B12, folate - Maybe urine MC+S, CXR, ECG, syphilis serology, HIV testing Imaging - CT / MRI head - consider SPECT scan, PET scan
71
what is a cognitive screen?
NICE recommended - 10-CS - 6CIT - 6-item screener - memory impairment screen - mini-cog - Test your memory Not NICE recommended - AMTS - GPCOG - MMSE
72
causes of falls?
- TLOC - mechanical fall - geriatric fall - visual impairment - BPPV - drugs, polypharmacy
73
TLOC causes
- syncope - seizure - hypoglycaemia - head injury - narcolepsy - stroke
74
define syncope?
- transient LOC due to cerebral hypoperfusion - low BP after fall in CO / SVR
75
define orthostatic / postural hypotension
- fall in BP >20/10 on standing - blood pools in legs with gravity, reduced venous return, reduced preload/EDV/SV/CO (normally compensated for by sympathetic increase in HR/SVR - Primary autonomic failure - older, Parkinson's, Lewy Body, MSA - secondary - DM, adrenal insufficiency, hypothyroid - drugs - anti-HTN, diuretics, alcohol - hypovolaemia - D+V, diuresis, haemorrhage
76
cardiac syncope?
- structural - MI, valvular heart disease, cardiomyopathy - Arrhythmias - tachy/brady
77
reflex syncope?
- increase in vagal tone - vasovagal - pain/emotions - carotid sinus hypersensitivity - situational syncope - micturition, defecation, cough
78
RFx for falls?
Leg weakness, vision problems, balance / gait disturbance, polypharmacy, incontinence, >65yo, depression, arthritis, psychoactive drugs, cognitive impairment
79
medication that can contribute to falls
Meds that cause postural hypotension - Nitrates, diuretics, anticholinergics, antidepressants, BBs, levodopa, ACEi Meds that cause falls by other mechanisms - Benzos, antipsychotics, opiates, anti-epileptics, codeine, digoxin, sedatives
80
how to investigate falls?
- Hx - before, during, after, any LOC, injuries, anticoags, collateral hx, seizure sx, what were they doing at the time, ?self-mobilised, DHx, SHx... - A-E - Full cardio, resp, GI, neuro exams - Check c-spine, pelvis, hips (?SLR), - Obs, BMs, urine dip, ECG - FBC, U/E, LFTs, bone profile, Mg, CRP, CK if long lie, coag, haematinics, vit D, TFTs - XR of injured limbs - CT head - ECHO - Lying standing BP - Turn 180 tests / timed up and go test - Tilt table test - measure HR / BP as table with pt on tilts - for postural hypotension
81
managing falls?
- tx cause - adjust meds - IV fluids - Cardiology referral if needed - Maybe neuro obs - Vasovagal / situational - reassure - Inpatient – 1-1 nursing, non-slip socks, low rise beds / mattresses on floor - Walking aids - OT/PT review - Package of care if going home
82
management of postural hypotension?
- Tx cause, review meds, ensure hydrated - Fludrocortisone - Midodrine – alpha agonist – for true autonomic dysfunction
83
Multiple sclerosis (MS)
- chronic condition of demyelination in the CNS - plaques of demyelination in brain + spinal cord - autoimmune inflammatory response - new myelin is less efficient - genetics, EBV, low vit D, smoking, obesity
84
what types of MS are there/
- clinically isolated - relapsing remitting - secondary progressive - primary progressive
85
clinically isolated syndrome in context of MS?
- First episode of demyelination - May never have another episode, or go on to develop MS
86
relapsing remitting MS?
- 85% - Acute attacks (1-2mo), periods of remission
87
secondary progressive MS?
- Deterioration after relapsing-remitting – neuro signs between relapses - 65% with relapsing-remitting go on to develop secondary progressive within 15yrs of dx
88
primary progressive MS?
- 10% pts - Progressive deterioration from onset - More common in older pts
89
MS Px?
- sx can progress over >24hrs, last days-wks, then improve Visual - optic neuritis - unilateral reduced vision, central scotoma, pain with eye movt, impaired colour vision, RAPD - optic atrophy - Uhthoff's - vision worse with warm body temp - internuclear ophthalmoplegia - impaired adduction ipsilaterally, nystagmus contralaterally, conjugate lateral gaze disorder Sensory - paraesthesia, numbness, trigeminal neuralgia, Lhermitte's syndrome Motor - spastic weakness - legs Cerebellar - ataxia - sensory / cerebellar - tremor Others - urinary incontinence - sexual dysfunction - intellectual dysfunction - transverse myelitis
90
MS Dx
- 2+ relapses of different parts of CNS - disseminated in space and time - Or objective clinical evidence of one lesion + reasonable hx evidence of previous relapse
91
MS Ix?
- exclude ddx - FBC, CRP/ESR, U/E, LFTs, glucose, HIV, auto-ABs, Ca, vit B12 - MRI - high-signal T2 lesions, periventricular plaques, Dawson fingers - LP - oligoclonal bands - electrophysiology - visual evoked potential studies delayed
92
management of MS
Acute relapses - high dose oral / IV steroids - methyl/prednisolone Disease-modifying therapies - natalizumab, ocrelizumab, fingolimod... Symptomatic tx - fatigue - amantadine, mindfulness, CBT - spasticity - baclofen/gabapentin, diazepam, physio - bladder dysfunction - USS, ISC, anticholinergics (solifenacin) - oscillopsia - gabapentin/memantin
93
MND
- progressive destruction of motor neurons - cause unknown, 5% have SOD1 mutation - possibly mitochondrial dysfunction - oxidative stress, ROS damages stuff - Frontotemporal dementia in 25%
94
types of MND?
- amyotrophic lateral sclerosis (ALS) - progressive bulbar palsy (PBP) - progressive muscular atrophy (PMA) - Primary lateral sclerosis (PLS)
95
Amyotrophic lateral sclerosis (ALS)
- <80% - most common - Loss of motor neurons in motor cortex and anterior horn of cord – UMN + LMN
96
Progressive bulbar palsy (PBP)
- 10-20% - CN9-12 – LMN - Affects muscles of talking + swallowing, tongue also
97
Progressive muscular atrophy (PMA)
- <10% - Anterior horn lesion – LMN - Affects distal muscle groups before proximal
98
Primary lateral sclerosis (PLS)
- Loss of bets cells in motor cortex – UMN - Marked spastic leg weakness, pseudobulbar palsy, no cognitive decline
99
MND Px
- Insidious, progressive weakness – often first in upper limbs - Fatigue when exercising - Clumsiness, dropping things - Dysarthria – slurred speech - dysphagia, fluid regurg, choking LMN signs - Muscle wasting - Reduced tone – flaccid paralysis - Fasciculations - Reduced reflexes UMN signs - Increased tone – spastic paralysis - Brisk reflexes - Upgoing plantar reflex
100
LMN signs?
- Muscle wasting - Reduced tone – flaccid paralysis - Fasciculations - Reduced reflexes
101
UMN signs?
- Increased tone – spastic paralysis - Brisk reflexes - Upgoing plantar reflex
102
MND Ix?
- clinical dx - NCS - normal - EMG - reduced no of APs, increased amplitude - MRI - exclude myelopathy etc
103
MND Mx?
- riluzole - NIV - resp support - baclofen - PEG - benzos - advanced directives
104
Guillain-Barre syndrome (GBS) - definition
- immune mediated demyelination of PNS - triggered by infection - C jejuni - ABs against infection attack Schwann cells -> demyelination
105
GBS Px
- hx of GE - sx peak in 2-4wks, recovery years - back/leg pain - progressive, symmetrical weakness of limbs - ascending, reflexes reduced, mild paraesthesia, neuropathic pain, hypotonia - CNs - diplopia, bl facial nerve palsy, oropharyngeal weakness - resp muscle weakness - autonomic - diarrhoea, urinary retention
106
GBS Ix?
- LP - raised protein, normal WCC - NCS - decreased velocity - spirometry - monitor FVC
107
GBS Mx?
- FVC <80% - mechanical ventilation - IV Ig 1st line - plasma exchange - VTE prophylaxis
108
Myasthenia gravis (MG)
- autoimmune disorder with ABs against AChR in NMJ - ABs block receptors, prevent ACh stimulation, - thymoma link - autoimmune link - thyroid, pernicious anaemia, RA, SLE, thymic hyperplasia
109
MG Px
- muscle weakness - worse with activity, improves with rest - extraocular - diplopia - proximal muscle weakness - face, neck, limb girdle - ptosis - dysphagia - slurred speech, jaw fatigue
110
what tests can you do to elicit fatiguability
Tests to elicit fatigability - Count to 50, voice quietens - Prolonged upward gazing exacerbates diplopia - Repeated blinking -> ptosis - Repeated abduction of one arm 20x -> unilateral weakness on that side
111
MG Ix
- ABs - AChR, MuSK, LRP4 - CK normal - CT / MRI thorax - thymus - Single fibre electromyography
112
MG Mx
- AChE inhibitors - pyridostigmine - immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate - thymectomy - rituximab
113
myasthenia crisis?
- life-threatening cx of MG - acute worsening of sx - often triggered by illness - eg resp infection - monitor FVC - resp failure - NIV / mechanical ventilation Mx - IV Ig - plasmapheresis
114
what is Huntington's?
- Inherited neurodegenerative disorder which causes chorea - jerky, semi-purposeful movements - autosomal dominant - CAG repeats, may see anticipation - lack of GABA + ACh, decreased inhibition of dopamine release -> excessive movts
115
how does Huntington's present?
- Sx develop >35yo - Starts with mild psychotic / behavioural sx - cognitive, psych, mood problems - Chorea - random, irregular, abnormal body movts - Personality changes - irritable, apathy, depression, intellectual impairment - Dystonia - Rigidity - Speech / swallowing difficulties - Saccadic eye movts
116
Huntington's Ix
- genetic testing, genetic counselling
117
Huntington's Mx
- MDT, physio, SALT - chorea - benzos, tetrabenazine, sulpiride - antidepressants - advanced directives
118
Parkinson's - definition
- Progressive degenerative movt disorder caused by degeneration of dopaminergic neurons in substantia nigra (in basal ganglia) - dementia, depression associations - from mitochondrial dysfunction / oxidative stress
119
key Parkinson's triad?
Tremor - worse at rest, 4-6Hz - worse stressed/tired - improves on voluntary movt - pill-rolling Bradykinesia - hypokinesia/poverty of movt - short, shuffling steps, reduced arm swinging - difficulty initiating movt - micrographia / monotonous hypophonic speech Rigidity - increased tone in limbs/trunk - lead pipe - throughout ROM - cogwheel - superimposed tremor
120
other potential Parkinson's sx?
- hypomimia - reduced facial movts - flexed posture - saliva drooling - psych - depression, dementia, psychosis, sleep disturbance, cognitive impairment, memory problems - anosmia - REM sleep behaviour disorder - fatigue - postural hypotension
121
Parkinson's DDx
Causes of Parkinsonism - Parkinson's disease, drug induced (antipsychotics, metoclopramide, domperidone), PSP, MSA, Wilson's, CO poisoning Drug-induced parkinsonism - rapid onset motor sx, bilateral - rigidity/resting tremor uncommon Benign essential tremor - symmetrical, 6-12Hz, improves at rest, worse with voluntary movts, improves with alcohol
122
Parkinson's Ix
- clinical dx - CT / MRI head - Use UK Parkinson's Disease Society Brain Bank Clinical Diagnostic Criteria - SPECT scan
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Parkinson's Mx?
First line - if motor sx affecting QoL - levodopa - if not - dopamine agonist / levodopa / MAO-B Neuropsych sx - anti-depressants, anti-psychotics Anti-muscarinics - block cholinergic receptors - drug-induced Parkinsonism - procyclidine
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what is levodopa?
- synthetic dopamine, less effective over time - add decarboxylase inhibitor - prevent peripheral metabolism - co-beneldopa (Madopa) - co-careldopa (Sinemet) - S/Es - dyskinesia (amantadine to tx), on-off effect, end-of-dose wearing off, postural hypotension, arrhythmias, N+V, psychosis
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what is a dopamine agonist?
- bromocriptine, cabergoline, pergolide - mimic dopamine action - S/Es - pulm/cardiac fibrosis, impulse control disorders
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MAO-B inhibitors
- selegiline, rasagiline - inhibit MAO-B - break down dopamine, serotonin, adrenaline - can use with levodopa to reduce end of dose sx worsening
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COMT inhibitors
- entacapone - inhibits enzyme that breaks down levodopa - take with levodopa to slow its breakdown
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Parkinson's plus syndrome?
- group of neurodegenerative diseases with classical sx of Parkinson's + additional features Multiple system atrophy (MSA) - neurons of brain degenerate, inc basal ganglia - Parkinsonism, autonomic dysfunction, cerebellar dysfunction Progressive supranuclear palsy (PSP) - postural instability, falls, impairment of vertical gaze - parkinsonism, cognitive impairment Corticobasal degeneration (CBD) Dementia with Lewy bodies (DLB) Frontotemporal dementia (Pick's disease)
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epilepsy definiton?
neurological condition characterised by recurrent seizures
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definition of a seizure?
Disordered electrical activity in the brain leading to clinical manifestationsexcessive, abnormal electrical activity in the brain
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causes of seizure
- epilepsy - febrile convulsions - alcohol withdrawal - non-epileptic - idiopathic - stroke, haemorrhage - head injury - Alzheimer's, dementia - tumour - metabolic disturbance - electrolytes
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Non-epileptic seizure px
- Situational - Longer - Closed eyes / mouth during tonic clonic - No incontinence / tongue biting - May have psychological cause - Don't occur during sleep - Preserved consciousness - Purposeful movements - Pelvic thrusting - crying afterwards
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types of seizure?
Focal (partial) seizures - one hemisphere, specific area - simple - no LOC - complex - impaired awareness - secondary generalised - evolve into bl, convulsive seizure Generalised seizure - widespread, both hemispheres, LOC - Tonic-clonic - muscle tensing + muscle jerking - Absence - blank/unresponsive for 10-20s - Myoclonic - rapid, brief muscle jerks - Atonic - sudden loss of muscle tone
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types of seizure in paeds?
- infantile spasms / West syndrome - tx with ACTH / vigabatrin - Lennox-Gastaut syndrome - Benign rolandic epilepsy - Juvenile myoclonic epilepsy
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epilepsy presentation
Tonic clonic - Tonic clonic - LOC - Tongue biting - Incontinence - Groaning Focal - Motor – Jacksonian march - Sensory – déjà vu, jamais vu, strange smells / tastes / sight / sound Absence - Stop activity, vacant, 20-30s, carries on Myoclonic - Sudden isolated jerk of muscle Tonic seizure - Sudden increased tone, intense stiffness, no jerking - Characteristic cry / grunt Atonic - Loss of muscle tone, fall
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what is an aura in epilepsy?
- Pt aware - Strange gut feeling, déjà vu, strange smells, flashing lights - Implies focal seizure, but not necessarily in temporal lobe
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what is post ical period in epilepsy?
- Confused, tired, irritable, low - Todd’s paralysis – temporary weakness after seizure - May have dysphasia – from focal seizure in temporal lobe
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how does epilepsy present: temporal lobe?
Temporal lobe (memory, emotion, speech understanding) - Rising epigastric sensation - Aura – déjà vu, jamais vu, auditory hallucinations, funny smells, fear - Anxiety, out-of-body experience, automatisms – eg lip smacking, chewing, fiddling
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how does epilepsy present: frontal lobe?
Frontal lobe (motor and thought processing) - Motor features – posturing, peddling movts of leg - Jacksonian march (seizure ‘marches’ up/down motor homunculus starting in face/thumb) - Post-ictal Todd’s paralysis – paralysis of limbs involved in seizure
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how does epilepsy present: parietal lobe?
Parietal lobe (interprets sensations) - Paraesthesia
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how does epilepsy present: occipital lobe?
Occipital lobe (vision) - Visual phenomena – spots, lines, flashes
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DDx for blackouts?
Vasovagal syncope. Cardiac syncope. Non-epileptic attacks. Intermittent hydrocephalus (rare). Migraine (if no loss of awareness).
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Ix for epilepsy?
clinical dx = >2 unprovoked seizures >24hrs apart Bloods - FBC, U/E, bone profile, Mg, LFTs, glucose ECG ?infective cause - blood/urine cultures, LP, CXR CT / MRI head EEG - support dx
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epilepsy general Mx guide
- most neurologists start AEDs after 2nd seizure - start after 1st seizure if - neuro deficit, structural abnormality, EEG unequivocal of epilepsy, family find further seizure unacceptable - prescribe by brand (not generic)
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how long do you need to be seizure free before driving/
>12mo
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Generalised tonic clonic Mx
M - sodium valproate F - lamotrigine / levetiracetam
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Focal seizure Mx
1st - lamotrigine / levetiracetam 2nd - carbamazepine
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Absence seizure Mx
1st - ethosuximide 2nd - M - sodium valproate - F - lamotrigine / levetiracetam
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Myoclonic seizures Mx
M - sodium valproate F - levetiracetam
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Tonic/ atonic seizures Mx
M - sodium valproate F - lamotrigine
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epilepsy in pregnancy? KEY POINTS
- folic acid 5mg OD - carbamazepine is least teratogenic - Breastfeeding is safe for mothers on antiepileptics Phenytoin = cleft palate, give vit K in last month of pregnancy also Lamotrigine = congenital malformation rate low Sodium valproate - significant risk of neurodevelopmental delay
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contraception and epilepsy?
on levetiracetam? can use all contraceptive types on lamotrigine? UKMEC 3: cocp. UKMEC 1; POP, implant, depo, IUD, IUS on phenytoin, carbamazepine, barbiturates, primidone, topiramate, oxycarbazepine? UKMEC 3: cocp. UKMEC 2: implant. UKMEC1: depo, IUD, IUS
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epilepsy complications
- aspiration pneumonia - sudden unexpected death in epilepsy (SUDEP)
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Status epilepticus
- seizure > 5mins - or multiple seizures without regaining consciousness in between
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Status epilepticus Ix
- A-E - Check pupils - BMs - VBG, seizure bloods - ?Toxins - Check for focal neurology, meningism, temp, tongue biting, incontinence etc - ?CT head - ?LP - EEG – and to check seizures controlled after mx
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Status epilepticus Mx
- Secure airway - NPA, adjuncts etc - Recovery position - 15L O2 - Get IV access (or IO) At 5 mins – benzodiazepine - Buccal midazolam 10mg - Rectal diazepam 10mg - IV lorazepam 4mg At 10 mins – benzodiazepine At 15 mins – IV phenytoin / levetiracetam, call ITU At 20 mins – RSI
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headache types: primary versus secondary
Primary - no underlying cause - tension, migraine, cluster Secondary - underlying cause - eg GCA, glaucoma, haemorrhage, meds
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Headache red flags
- Fever, photophobia, neck stiffness - meningitis, encephalitis, brain abscess - Focal neurology - New cognitive disturbance - Impaired GCS - Visual disturbance - eg GCA, glaucoma, tumour - Red eye - glaucoma - Sudden-onset headache - 'thunderclap' - Worse on coughing, straining - Postural, worse standing / lying / bending over - Vomiting - Hx of trauma - Hx of malignancy - Pregnancy - pre-eclampsia - Immunocompromised - HIV, immunosuppressants - <20yo and hx of malignancy - Seizures
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What is a tension headache and what can cause them?
- common primary headache - most common cause of primary headache in adults - infrequent/frequent episodic, chronic Causes - eg stress, depression, alcohol, skipping meals, dehydration
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Tension headache Px?
- Episodic, 30 mins - 7d - Like tight band around head - No aura, N+V, not aggravated by routine physical activity - May have photophobia / phonophobia
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Tension headache Px?
- Episodic, 30 mins - 7d - Like tight band around head - No aura, N+V, not aggravated by routine physical activity - May have photophobia / phonophobia
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Tension headache management?
- paracetamol, NSAIDs, aspirin - limit analgesia to <6d/mo - acupuncture - physio, exercise, CBT, relaxation - trial of amitriptyline
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what is a migraine
- Complex neuro condition causing episodes of headaches + associated sx - most common type in paeds/teenagers Types - migraine +/- aura - silent migraine - aura, no headache - hemiplegic migraine
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Name some possible migraine triggers
- likely combination of structural, functional, chemical, vascular, inflammatory factors Triggers - CHOCOLATE - Chocolate - Hangovers - Orgasms - Cheese, caffeine - Oral contraceptives - Lie-ins - Alcohol - Tumult – loud noise (also bright lights) - Exercise
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Migraine Px ( 5 stages of migraine: prodrome, headache, aura)
Prodrome - Can begin several days before - Yawning, fatigue, mood change Headache - 4-72hrs - Unilateral - Throbbing / pounding - Aggravated by routine physical activity - Photophobia - Phonophobia - N+V Aura - <60mins - Vision – lines, loss of visual fields (eg scotoma) - Sensation – tingling, numbness - Language - dysphasia Resolution stage - Headache may fade away / relieved abruptly by vomiting / sleeping Postdromal / recovery phase
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Migraine dx criteria (5,4,3,2,1)
A - at least 5 attacks fulfilling criteria B-D B - last 4-72hrs C - at least 2 of: - Unilateral - Pulsating - Moderate / severe pain - Aggravated by routine activity D - during headache, at least 1 of: - N +/- V - Photophobia, phonophobia E - no other cause
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Hemiplegic migrane
- Unilateral limb weakness - Ataxia - Impaired consciousness - Mimic stroke / TIA
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Hemiplegic migrane
- Unilateral limb weakness - Ataxia - Impaired consciousness - Mimic stroke / TIA
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Migraine Mx - acute
Acute - NSAIDs, paracetamol - triptans - sumatriptan - stimulate serotonin receptors - antiemetics - prochlorperazine / metoclopramide - no opiates
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Migraine Mx - prevention
avoid triggers - propranolol - topiramate - amitriptyline - specialist - candesartan, valproate, MAbs - menstrual migraines - prophylactic triptans, mefenamic acid, aspirin
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Migraine Mx - prevention
avoid triggers - propranolol - topiramate - amitriptyline - specialist - candesartan, valproate, MAbs - menstrual migraines - prophylactic triptans, mefenamic acid, aspirin
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Migraine Mx - in pregnancy
Pregnancy - paracetamol - NSAIDs 1st/2nd trim - no aspirin/opioids
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Migraine Mx - in pregnancy
Pregnancy - paracetamol - NSAIDs 1st/2nd trim - no aspirin/opioids
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Cluster headache - definition + triggers?
- most disabling type of primary headache - potential triggers - alcohol, strong smells, exercise
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Cluster headache Px?
- Occur in clusters which last several weeks, occur once a year - Intense sharp, stabbing pain / headache around one eye - Occurs 1-2 times/day, episode lasts 15mins-2hrs - Restless during attack - Redness, lacrimation, lid swelling, swelling - Nasal stuffiness - Miosis / ptosis in minority
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Cluster headache Ix?
- MRI with gadolinium contrast
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Cluster headache Mx
- neurology referral Acute - 15L O2 - S/C triptan (or intranasal) Prophylaxis - verapamil - occipital nerve block, prednisolone, lithium
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Medication overuse headache Px
- Present for >15d per month - Developed/worsened whilst taking regular symptomatic medication - Pts using opioids and triptans at most risk - May be psychiatric comorbidity
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Medication overuse headache Mx
- withdraw simple analgesics + triptans abruptly (may worsen headache initially) - gradually withdraw opioids Withdrawal sx - Vomiting, hypotension, tachycardia, restlessness, sleep disturbance, anxiety
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Post-LP headache - what is it and how does it present?
- due to leak of CSF after dural puncture Px - 24-48hrs after LP, can be <1wk later - Can last several days - Worsens when upright, improves with recumbent position
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Post-LP headache - Mx
- supportive - analgesia, rest - if pain >72hrs, tx to prevent SDH - blood patch / epidural saline / IV caffeine
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sinusitis?
- Inflammation of paranasal sinuses in face - Pain / pressure after viral URTI - May have swelling / tenderness of affected areas - Most resolve in 2-3wks - >10d - steroid nasal spray, abx (phenoxymethylpenicillin
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Cervical spondylosis
- Caused by degenerative changes in c-spine - Neck pain, worse on movt, often also headaches
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Hormonal headache
- low oestrogen - similar to migraines - unilateral, pulsatile headache, nausea - eg 2d before and first 3d of menstrual period / early pregnancy / perimenopause - triptans / NSAIDs (mefenamic acid) to tx
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brain tumour
- primary / secondary (10x more common)
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brain tumour pathophysiology
- can be benign/malignant, high/low grade Primary - adults - majority supratentorial, children infratentorial Secondary - NSCLC - most common - SCLC - breast - melanoma - renal cell - GI - SOL, CSF lost to compensate, eventually no more CSF, ICP rises acutely -> worsening in sx, eventually coning, death
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Brain tumour Px
Progressive focal neurology - location dependent - eg personality change - frontal lobe - sensory loss, motor change, speech changes, visual field defects, DANISH for cerebellar Raised ICP - Papilloedema - Headache, worse lying down / waking / at night / coughing - Vomiting - CN3/6 palsies - Unilateral ptosis - Drowsiness Seizures - focal seizures more common
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Brain tumours Ix
- CT with contrast - MRI brain - Biopsy - during surgery - LP CI'd (raised ICP)
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Brain tumours Mx
- surgery - chemo / radio - dexamethasone - levetiracetam - palliative care
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meningitis
- inflammation of meninges - viral most common, presume bacterial until otherwise, also fungal, non-infective
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meningococcal septicaemia
bacterial infection spreads to bloodstream - non-blanching rash
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bacterial causes of meningitis
0-3mo - GBS, E coli, listeria 3mo-6yo - N meningitidis, S pneumoniae, H influenzae 6-60yo - N meningiditis, S pneumoniae >60yo - S pneumoniae, N meningiditis, Listeria Immuno - listeria
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viral causes of meningitis
- coxsackie, HSV, VZV, CMV, HIV, measles, mumps
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non infective causes of meningitis
- malignancy, chemicals, meds, sarcoidosis, SLE
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Meningitis Px
- fever - neck stiffness - vomiting - headache - photophobia - altered consciousness - seizures - non-blanching rash - Kernig's - Brudzinski's
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kernig's sign?
Position the patients supine with their hips flexed to 90°. This test is positive if there is pain on passive extension of the knee
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brudzinski's sign?
Position the patients supine and passively flex their neck. This test is positive if this manoeuvre causes reflex flexion of the hip and knee
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Meningitis LP interpretation: bacterial
- cloudy - high protein - low glucose - high WCC (neutrophils) - culture growth
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Meningitis LP interpretation: viral
- clear - mildly high protein - normal glucose - high WCC (lymphocytes) - negative culture
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Meningitis LP interpretation: TB
- slightly cloudy - low glucose - high protein - PCR to dx
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meningitis Ix?
- LP - bloods - cultures, meningococcal PCR, FBC, U/E, glucose, lactate, coag, VBG - maybe throat swab - CT not normally indicated
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meningitis Mx in community
- IM benzylpenicillin
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meningitis Mx?
- <3mo – IV cefotaxime + amoxicillin - 3mo-50yo – IV cefotaxime / ceftriaxone - >50yo – cefotaxime / ceftriaxone + amoxicillin NB Vancomycin added if risk of penicillin resistant pneumococcal infection e.g. recent foreign travel or prolonged antibiotic exposure Steroids used in bacterial meningitis to reduce the frequency and severity of hearing loss and neurological damage - dexamethasone QDS for 4 days if >3m
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Mx of meningitis contacts?
Mx of contacts - close contact <7d sx onset - oral ciprofloxacin single dose, rifampicin 2nd line - offer meningococcal vaccination/booster
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complications of meningitis?
- Sensorineural hearing loss - most common - Seizures - Focal neuro deficit - Cognitive impairment - Sepsis, cerebral abscess - Brain herniation, hydrocephalus - With meningococcal meningitis - Waterhouse-Friderichsen syndrome - adrenal insufficiency secondary to adrenal haemorrhage
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encephalitis?
- infection / inflammation of brain parenchyma - mostly viral - typically temporal / inferior frontal lobes
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Encephalitis causes
Viral - HSV1 (cold sores), HSV2 (genital herpes), VZV, CMV, EBV, HIV, mumps, measles - HSV encephalitis - tends to affect temporal lobes Non-viral - bacterial, TB, malaria, polio, mumps, rubella, measles
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Encephalitis Px
- Fever - Headache - Psych sx, unusual behaviour - Altered cognition, consciousness - Seizures, often focal - Vomiting - Focal neurology, eg aphasia
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Encephalitis Ix
- LP - high lymphocytes/protein, send for PCR, CI if low GCS, unstable, seizing - CT (contrast enhanced) / MRI - petechial haemorrhages in medial temporal/inferior frontal lobes - EEG - lateralised periodic discharges at 2Hz - HIV testing, swabs elsewhere
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Encephalitis Mx
- IV acyclovir
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facial nerve palsy ?
- dysfunction of facial nerve -> unilateral facial weakness - each side of forehead has UMN innervation from both sides of brain
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facial nerve palsy: UMN lesion
- forehead spared - strokes, tumours - bilateral - MND, pseudobulbar palsies
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facial nerve palsy: LMN lesion
- forehead not spared (paralysed) - Bell's, Ramsay Hunt, OM, OE, HIV....
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Bell's palsy?
- acute, unilateral idiopathic facial nerve paralysis - cause unknown, maybe HSV involvement
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Bell's palsy Px
- Unilateral facial muscle weakness, forehead affected (LMN facial palsy) - Rapid onset <72hrs - Post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis (sounds seem louder), eye not closing, drooling
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Bell's palsy dx
- dx made when no other cause for paralysis found
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Bell's palsy Mx
- oral prednisolone within 72hrs - eye care - drops, tape closed at night - refer if atypical sx / worsening / no improvement
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Ramsay Hunt syndrome: Px and Mx
- reactivation of VZV in geniculate ganglion of CN7 Px - auricular pain - facial nerve palsy - vesicular rash around ear - vertigo, tinnitus Mx - oral acyclovir - corticosteroids
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brain abscess?
- pus-filled swelling in brain Causes - from middle ear / sinuses - trauma - surgery to scalp - penetrating head injuries - emboli from IE
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brain abscess px?
- headache - dull, persistent - fever - focal neurology - site dependent - raised ICP - nausea, papilloedema, seizures
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brain abscess ix ?
CT head
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brain abscess Mx
- surgery - craniotomy, debride - IV abx - ceftriaxone + metronidazole - dexamethasone
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Charcot-Marie-Tooth disease
- hereditary peripheral neuropathy - predominantly motor - majority autosomal dominant Px - Hx of frequently sprained ankles - Foot drop - High-arched feet (pes cavus) - Hammer toes - Distal muscle weakness / atrophy – inverted champagne bottle legs - Hyporeflexia - Stork leg deformity - Peripheral sensory loss Mx - OT/PT, podiatry
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DDx of peripheral neuropathy
- alcohol - B12 deficiency - cancer (myeloma), CKD - diabetes, drugs (amiodarone, isoniazid, nitrofurantoin, metronidazole) - vasculitis
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essential tremor
- autosomal dominant condition - tremor usually affecting upper limbs/hands
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essential tremor Px
- postural tremor (worse on movt) 6-12Hz - symmetrical - improved with alcohol/rest - worse when tired, stressed, caffeine - absent during sleep - titubation - head tremor
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essential tremor Mx
- propranalol - primidone
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Idiopathic intracranial HTN (IIH)
- build up of pressure around brain RFs - female, obese, pregnancy, drugs
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IIH Px
- headache, worse in morning/coughing/straining - nausea - blurred vision - papilloedema - enlarged blind spot - sixth nerve palsy
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IIH Ix
- assess neurology, eyes, vision - CT / MRI - LP
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IIH Mx
- wt loss - acetazolamide / topiramate - act as diuretics - repeated LP - surgery - eg shunt / optic nerve sheath decompression
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narcolepsy
- long term brain condition causing pt to fall asleep suddenly at inappropriate times - low levels orexin (hypocretin)
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narcolepsy Px
- Onset in teenage years - Hypersomnolence - sleepy during day, difficult to stay awake - Sleep attacks - fall asleep suddenly / w/o warning - Cataplexy - sudden loss of muscle tone triggered by emotion - Sleep paralysis - Vivid hallucinations on going to sleep / waking up
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narcolepsy Ix
- multiple sleep latency EEG - CSF orexin levels - low
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narcolepsy Mx
- Daytime stimulants - modafinil - Night time - sodium oxybate
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trigeminal neuralgia
- pain syndrome - severe unilateral pain in face - affects any branch of trigeminal nerve - ophthalmic, maxillary, mandibular - idiopathic / nerve compression - possible triggers - light touch, cold weather, spicy food, caffeine, citrus
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Trigeminal neuralgia Px
- intense facial pain, seconds-hrs - electricity-like - attacks worsen over time
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Trigeminal neuralgia red flags for serious underlying cause
- Sensory changes - Deafness, ear problems - Skin / oral lesions - Pain only in ophthalmic division, or bilaterally - Optic neuritis - MS FHx - <40yo
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Trigeminal neuralgia Mx
- carbamazepine - neurology referral if atypical features, eg <50yo - surgery to decompress/intentionally damage nerve
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Neurofibromatosis
- genetic condition that causes nerve tumours (neuromas) to develop throughout nervous system - tumours benign, but cause neuro/structural problems - NF1>NF2
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NF1
- gene on c17 - codes for neurofibromin - autosomal dominant Dx - need 2/7 - CRABBING C – café-au-lait spots (>6), >5mm in children or >15mm in adults R – relative with NF1 A – axillary / inguinal freckles BB – bony dysplasia (eg Bowing of long bone or sphenoid wing dysplasia) I – Iris hamartomas (Lisch nodules) (>2) – yellow brown spots on iris N – neurofibromas (>2) or 1 plexiform neurofibroma G – glioma of optic nerve Ix - clinical dx - genetic testing - XR - bone lesions - CT / MRI Mx - no tx - mx sx / tx cx
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NF2
- gene on c22 - codes for merlin - lack of leads to Schwannomas - autosomal dominant Px - acoustic neuromas - hearing loss, tinnitus, balance issues - schwannoma Mx - surgery to resect
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hydrocephalus
- excessive volume of CSF in ventricular system in brain - production/absorption imbalance Obstructive - tumours, haemorrhage, aqueduct stenosis Non-obstructive - choroid plexus tumour, reabsorption failure, normal pressure hydrocephalus
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hydrocephalus Px
- raised ICP sx - headache - worse in morning, lying down, Valsalva - N+V - papilloedema - coma - infants increased head circumference
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hydrocephalus Ix and Mx
Ix - CT head - MRI - LP - drain CSF, measure opening pressure (do not use in obstructive causes) Mx - external ventricular drain (EVD) - ventriculoperitoneal shunt (VP shunt) - surgical tx for obstructive pathology
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Normal pressure hydrocephalus
- reversible cause of dementia in elderly - reduced CSF absorption at arachnoid villi / head injury / SAH / meningitis
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Normal pressure hydrocephalus Px
- urinary incontinence - dementia, bradyphrenia (slowed thinking) - gait abnormality - sx develop over a few months
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Normal pressure hydrocephalus Ix
- CT head - hydrocephalus, ventriculomegaly, no sulcal enlargement
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Normal pressure hydrocephalus Mx
- VP shunt
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wernicke's aphasia?
- receptive - Lesion of superior temporal gyrus, typically supplied by inferior division of left MCA - Forms speech before sending it to Broca’s - Sentences make no sense, word substitution, neologisms, but speech fluent (word salad) - Comprehension impaired
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Broca's aphasia?
- expressive - Lesion of inferior frontal gyrus, superior division of left MCA - Speech non-fluent, laboured, halting, repetition impaired - Comprehension normal
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conduction aphasia
- From stroke affecting arcuate fasciculus – connection between Wernicke’s and Broca’s - Speech fluent, repetition poor - Comprehension norma
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restless legs syndrome: definition and causes?
- spontaneous, continuous lower limb movts, associated with paraesthesia Causes - FHx, IDA, uraemia, DM, pregnancy
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restless legs Px
- Akathisia - uncontrollable urge to move legs - worse at night initially, then progresses to during day, sx worse at rest - Crawling / throbbing sensations - Noted in sleep by partner - periodic limb movts of sleeps (PLMS
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restless legs Ix
- Clinical dx - Ferritin - r/o iron deficiency anaemia
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restless legs Mx
- Walking, stretching, massaging - Iron - Dopamine agonist 1st line - pramipexole, ropinirole - Benzos - Gabapentin
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Reye's syndrome
- Severe progressive encephalopathy affecting children - Associated with fatty infiltration of liver, kidneys, pancreas - Known association with aspirin, ?viral cause also Px - Preceding viral illness - Confusion, seizures, cerebral oedema, coma - Fatty infiltration of liver, kidneys, pancreas - Hypoglycaemia Mx - Supportive
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Lambert-Eaton myasthenic syndrome
- paraneoplastic syndrome of reduced ACh release at NMJ - SCLC, breast, ovarian, also autoimmune - AB against presynaptic voltage-gated Ca channels in PNS
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Lambert-Eaton myasthenic syndrome Px
- limb girdle weakness (lower limbs first) - hyporeflexia - exercise improves sx - autonomic - dry mouth, impotence, difficulty peeing - no ptosis (MG)
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Lambert-Eaton myasthenic syndrome Ix
- EMG - incremental response to repetitive electrical stimulation
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Lambert-Eaton myasthenic syndrome Mx
- tx cancer - immunosuppression - prednisolone / azathioprine - amifampridine / 3,4-diaminopyridine - pyridostigmine - IVIg, plasma exchange
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neuropathic pain
- pain which arises following damage/disruption to nervous system Px - burning, tingling, pins/needles, electric shocks, loss of sensation to touch Ix - DN4 questionnaire Mx - amitriptyline / duloxetine / gabapentin / pregabalin - tramadol for exacerbations - topical capsaicin - pain clinic - physiotherapy
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Complex regional pain syndrome
- Areas are affected by abnormal nerve functioning -> neuropathic pain, abnormal sensations - Usually one limb, often triggered by injury to area - Painful, hypersensitive (even to clothing), swell, change colour/temp, flush, sweat - Tx as neuropathic - pain specialist
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what is a vestibular schwannoma?
- acoustic neuroma - 90% cerebellopontine angle tumours - Bl in NF2
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how might vestibular schwannoma present?
- CN8 – vertigo, unilateral sensorineural hearing loss, unilateral tinnitus - CN5 – absent corneal reflex - CN7 – facial palsy
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how would you investigate and manage vestibular schwannoma
Ix - Urgent ENT referral - MRI of cerebellopontine angle - Audiometry Mx - Surgery - Radiotherapy - Watch and wait
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what is pituitary apoplexy?
- sudden enlargement of pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage/infarction - eg HTN, pregnancy, trauma, anticoags Ix - MRI Mx - steroid replacement (loss of ACTH) - monitor fluid balance - surgery
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Syringomyelia
collection of CSF in spinal cord - syringobulbia - fluid-filled cavity in medulla Causes - chiari malformation - trauma - tumours - idiopathic
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syringomyelia Ix and Mx
- full spine MRI with contrast - brain MRI (exclude chiari) - tx cause - shunt if persistent
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what is autonomic dysreflexia?
- clinical syndrome with spinal cord injury at/above T6 - Afferent signals (most commonly from faecal impaction / urinary retention) cause sympathetic spinal reflex via thoracolumbar outflow - The usual centrally-mediated parasympathetic response prevented by cord lesion - Results in unbalanced physiological response – extreme HTN, flushing, sweating above level of cord lesion, agitation, haemorrhagic stroke Mx - Remove / control stimulus - Tx HTN +/- bradycardia
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visual field defects?
- Left homonymous hemianopia means visual field defect to left, ie lesion of right optic tract - Homonymous quadrantanopias – PITS - Congruous defect – complete / symmetrical visual field loss. Incongruous – incomplete / asymmetric - Incongruous defects – optic tract lesion, congruous defects – optic radiation lesion / occipital cortex
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homonymous hemianopia
- Incongruous – optic tract lesion - Congruous – optic radiation / occipital cortex - Macula sparing – occipital cortex
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Homonymous quadrantanopias
- Superior – lesion of inferior optic radiations, temporal lobe, Meyer’s loop - Inferior – superior optic radiations, parietal lobe, Baum’s
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bitemporal hemianopia
- Lesion of optic chiasm - Upper quadrant > lower – inferior chiasmal compression – pituitary tumour - Lower > upper – superior compression - craniopharyngioma
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Arnold-Chiari malformation
- Downward displacement / herniation of cerebellar tonsils through foramen magnum – congenital / acquired through trauma Px - Hydrocephalus from obstruction of CSF outflow - Headache - Syringomyelia
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Erb's palsy?
- Damage to C5,6 - From excessive lateral flexion of neck – trauma / birth injury Px - Sensory loss in C5,6 dermatomes - Weak elbow flexion, wrist extension, arm abduction + external rotation -> flexed wrist, extended forearm, internally rotated + adducted arm (waiter’s tip posture) - Asymmetric moro reflex in infants
278
Klumpke's palsy
- Damage to lower trunk of brachial plexus – T1 (C8-T1) - Due to hyperabduction of arm – trauma / birth injury, or compression – Pancoast tumour / rib Px - Sensory loss in C8, T1 dermatomes - Weakness of intrinsic hand muscles -> total claw hand - Absent grasp reflex in infants
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Axillary nerve palsy (C5-6)
Causes - Anterior shoulder dislocation - Fracture surgical neck of humerus Motor - Deltoid – impaired abduction, atrophy - Teres minor – impaired external rotation Sensory - Lateral shoulder
280
Musculocutaneous nerve palsy (C5-7)
Causes - Trauma, Erb’s Motor - Impaired elbow flexion – brachialis / coracobrachialis - Impaired forearm supination – biceps brachii Sensory - Lateral forearm, elbow to base of thumb
281
Median nerve palsy (C5-T1)
Causes - Proximal – supracondylar humerus fracture - Distal – carpal tunnel syndrome, wrist lac Motor - Supplies LOAF – lateral 2 lumbricals, opponens pollicis, abductor pollicis brevis, flexor pollicis brevis - Median claw, ape hand - Can’t make OK sign - Thenar muscle atrophy - Proximal damage – unable to pronate, weak wrist flexion, wrist ulnar deviation Sensory - Palmar thumb, index, middle fingers, lateral ring finger
282
Radial nerve palsy (C5-T1)
Causes - Axilla – crutch, Saturday night palsy - Midshaft fracture of humerus - Wrist fracture, tight bracelets Motor - Impaired elbow extension - Wrist drop – impaired extension Sensory - Dorsal thumb, index, middle, and lateral ring finger
283
Ulnar nerve palsy (C7-T1)
Causes - Proximal – fracture of medial epicondyle of humerus - Distal – ulnar tunnel syndrome, cycling, hook of hamate fracture Motor - Motor to medial 2 lumbricals, adductor pollicis, interossei, hypothenar, flexor carpi ulnaris - Ulnar claw – hyperextension of MCPJ, flexion at distal and proximal IPJ of 4/5th digits - Wasting + paralysis of intrinsic hand muscles (except lateral two lumbricals) - Wasting + paralysis of hypothenar muscles - Damage at elbow – as above + radial deviation of wrist Sensory - Medial half of ring finger, little finger, dorsal and palmar
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Superior gluteal nerve palsy (L4-S1)
Causes - IM injection Motor - Paralysis of glut medius / minimus – impaired hip abduction. Positive Trendelenburg (lateral pelvic tilt towards opposite side)
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Inferior gluteal nerve palsy (L5-S2)
Causes - Posterior hip dislocation Motor - Paralysis of glut maximus – impaired thigh extension – difficulty standing up, climbing stairs
286
Femoral nerve palsy (L2-4)
Causes - Trauma, prolonged pressure Motor - Impaired hip flexion, knee extension Sensory - Anteromedial thigh (anterior cutaneous branches), medial lower leg/edge of foot (saphenous nerve lesion)
287
Obturator nerve palsy (L2-3)
Causes - Pelvic ring fracture, obturator hernia Motor - Paralysis of hip adductors Sensory - Medial thigh
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Sciatic nerve palsy (L4-S3)
Causes - Iatrogenic, IM injection - Trauma, hip dislocation, total hip arthroplasty Motor - Impaired knee flexion (hamstrings), motor deficits of tibial nerve and common peroneal Sensory - Lower leg + foot
289
Tibial nerve palsy (L4-S3)
Causes - Tibial fracture, tarsal tunnel syndrome Motor - Impaired foot inversion / plantarflexion – can’t walk on toes, cannot invert foot (TIPPED) Sensory - Sole of foot
290
Common peroneal nerve palsy (L4-S2)
Causes - Fracture of fibular head Motor - Deep peroneal nerve – paralysis of dorsiflexors – foot drop, high-stepping gait - Superficial nerve – impaired pronation / eversion of foot (TIPPED) Sensory - Deep – flip-flop zone between first and second toes - Superficial – lateral lower leg, dorsum of foot
291
Olfactory (I)
Palsy – change in smell
292
Optic (II)
Palsy – change in sight
293
Oculomotor (III)
- All eye muscles except LR and SO. Pupil constriction, accommodation, eyelid opening - Palsy – ptosis, down + out eye, fixed dilated pupi
294
Trochlear (IV)
- Superior oblique - Palsy – defective downward gaze – vertical diplopia – noticed when looking down – head tilt to compensate
295
Trigeminal (V)
- Facial sensation, mastication - Palsy – trigeminal neuralgia, loss of corneal reflex (afferent), loss of facial sensation, paralysis of mastication muscles, deviation to jaw to weak side
296
Abducens (VI)
- Lateral rectus - Palsy – defective abduction – horizontal diplopia
297
Facial (VII)
- Facial movt, taste anterior 2/3 tongue, lacrimation, salivation - Palsy – flaccid paralysis of upper + lower face, loss of corneal reflex (efferent), loss of taste, hyperacusis
298
Vestibulocochlear (VIII)
- Hearing, balance - Palsy – hearing loss, vertigo, nystagmus. Acoustic neuromas – Schwann cell tumours of cochlear nerve
299
Glossopharyngeal (IX)
- Taste posterior 1/3 tongue. Salivation, swallowing, mediates input from carotid body + sinus - Palsy – hypersensitive carotid sinus reflex, loss of gag reflex (afferent)
300
Vagus (X)
- Phonation, swallowing, innervates viscera - Palsy – uvula deviates away from site of lesion, loss of gag reflex (efferent)
301
Accessory (XI)
- Head + shoulder movt - Palsy – weakness turning head to contralateral side, weakness shrugging shoulder
302
Hypoglossal (XII)
- Tongue movt - Palsy – tongue deviates towards side of lesion
303
poliomyelitis
Affects anterior horns - LMN signs
304
brown-sequard syndrome
- Hemi-section of spinal cord Lateral corticospinal tract – ipsilateral spastic paresis below lesion - Decussates at medulla DCML – ipsilateral loss of proprioception and vibration, fine touch - Decussates at medulla Lateral spinothalamic – contralateral loss pain and temp sensation - Decussates almost immediately in spinal cord (1-2 levels below lesion)
305
friedrich's ataxia
- Other features of cerebellar disease as well as ataxia - eg intention tremor
306
Anterior cord syndrome
- Eg from anterior spinal artery occlusion, flexion/extension - Lateral corticospinal tracts – bilateral spastic paresis - Lateral spinothalamic tracts – bilateral loss of pain + temp sensation
307
Central cord syndrome
- Eg from hyperextension - Small lesions - spinothalamic. Large lesions - spinothalamic, DCML, corticospinal - Weakness and numbness - more so in upper limbs than lower limbs - cape-like distribution
308
Posterior cord syndrome
- Eg from B12 deficiency, MS, tumour compression, neck hyperextension, posterior spinal artery infarct - DCML – loss of fine touch, vibration, proprioception - Wide steppage gait – sensory ataxia. Positive Romberg’s
309
Subacute combined generation of spinal cord
- B12 deficiency, and E - Dorsal columns and lateral corticospinal tracts affected - Joint position + vibration lost first, then distal paraesthesia - UMN signs in legs – upgoing plantars, brisk knee, absent ankle jerk - If untreated – stiffness + weakness persist
310
Cerebral venous sinus thrombosis (CVST)
- thrombosis in brain's venous sinuses - prevents blood draining - 50% isolated sagittal sinus - also cavernous sinus thrombosis / lateral sinus
311
CVST Px
- sudden headache - N+V - reduced GCS
312
CVST Ix and Mx
- MRI venography, CT alternative - D dimer elevated maybe Mx - anticoagulation - LMWH, then warfarin
313
Degenerative cervical myelopathy (DCM)
- stenosis of c-spine leading to spinal cord compression, neurology - age-related osteoarthritis
314
DCM Px
- subtle early sx, progressive - pain - neck, upper/lower limbs - loss of motor function - dexterity, impaired gait - loss of sensory function - numbness - autonomic - urinary/faecal incontinence, impotence - Hoffman's sign positive
315
DCM Ix and Mx
MRI c spine - refer for spinal services assessment - decompressive surgery
316
causes of foot drop?
- Common peroneal nerve lesion - eg fracture of fibular head, compression - L5 radiculopathy - would also show weakness of hip abduction - Sciatic nerve lesion - Superficial / deep peroneal nerve lesion - Central nerve lesions, eg stroke - other features would be present
317
myotonic dystrophy
- inherited myopathy, sx develop ~20-30yo, affects skeletal, cardiac, smooth muscle - autosomal dominant trinucleotide repeat disorder, DM1/2 types - distal/proximal weakness more prominent Px - Myotonic facies – long, haggard appearance - Frontal balding - Bilateral ptosis - Cataracts - Dysarthria - Myotonia (tonic spasm) - Weakness of arms + legs, distal initially - Mild mental impairment - DM - Testicular atrophy - Cardiac – HB, cardiomyopathy - Dysphagia
318
Creutzfeldt-Jakob disease (CJD)
- rapidly progressive neuro condition - prion proteins - induce formation of amyloid folds - sporadic / new variant Px - dementia - rapid onset - myoclonus Ix - CSF - normal - EEG – biphasic, high amplitude sharp waves (only in sporadic CJD) - MRI – hyperintense signals in basal ganglia and thalamus
319
Duchenne muscular dystrophy (DMD)
- Frameshift mutation – one/both binding sites lost – more severe form Px - Progressive proximal muscle weakness from 5yo - Calf pseudohypertrophy - Gower’s sign – child uses arms to stand from squatting - 30% - LD
320
Becker muscular dystrophy
- Non-frameshift insertion – both binding sites preserved – milder form Px - After 10yo - Intellectual impairment much less common
321
Cataplexy
- Sudden / transient loss of muscular tone caused by strong emotion – eg laughter, being frightened. 2/3 pts who have narcolepsy have cataplexy
322
Thoracic outlet syndrome (TOS)
- Compression of brachial plexus, subclavian artery, or vein at site of thoracic outlet (space between clavicle and first rib)
323
reflexes
- S1-2 - ankle - L3-4 - knee - C5-6 - biceps - C7-8 - triceps - S1,2 tie shoe, L3,4 kick the door, C5,6 pick up sticks, C7,8 shut gate
324
radiculopathy?
- compression of nerve root / LMN - eg pinched nerve - degenerative disc disease, OA, facet joint degeneration, spondylosis - radicular pain, weakness in limbs, numbness / paraesthesia - MRI, maybe EMG - neuropathic pain meds, tx cause, surgery
325
neuropathy?
pathological process affecting nerve(s)
326
mononeuropathy
- single nerve affected - eg CTS
327
mononeuritis multiplex
Several individual nerves affected Systemic causes - WARDS PLC - Wegener's granulomatosis - AIDs/amyloid - RA - DM - Sarcoidosis - PAN (polyarteritis nodosa) - Leprosy - Carcinoma
328
polyneuropathy
- many nerves affected - diffuse, symmetrical - Can be motor, sensory, sensorimotor, autonomic - Demyelination / axonal degeneration - Often distal – glove + stocking Mostly motor - GBS - Porphyria - Lead poisoning - Hereditary sensorimotor neuropathies (HSMN) – Charcot-Marie-Tooth - Chronic inflammatory demyelinating polyneuropathy (CIDP) - Diphtheria Mostly sensory - Diabetes - Uraemia - Leprosy - Alcoholism - B12 deficiency - subacute combined degeneration of spinal cord - Amyloidosis Px - Sensory deficit - Weakness, muscle cramps, fasciculations, atrophy - Poor balance, ataxia Mx - Tx cause - Neuropathic pain meds if needed