Phase 4 Question Knowledge > Neuro > Flashcards

Neuro Flashcards

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1
Q

Unconsciousness / coma

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

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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2
Q

Brain-stem death

A

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

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3
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
  • 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)
  • Exclude reversible causes
  • No sedation
  • Normal electrolytes
  • Patient >2mo old
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4
Q

Testing for brain 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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5
Q

Stroke

A
  • CVA - sudden interruption in vascular supply of brain -> irreversible brain death
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6
Q

Stroke types

A

Ischaemic

  • 85%, thrombo/embolus, also shock, vasculitis
  • RFs - age, HTN, smoking, high cholesterol, DM, AF, cOCP, FHx, carotid artery stenosis

Haemorrhagic

  • 15%, intracerebral/subarach
  • RFs - age, HTN, AV malformation, anticoagulation
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7
Q

Stroke general Px

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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8
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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9
Q

Total anterior circulation infarct (TACS)

A
  • ACA and MCA
  • All 3 criteria above present
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10
Q

Partial anterior circulation infarct (PACS)

A
  • Smaller arteries of anterior circulation
  • 2 of above criteria present
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11
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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12
Q

Posterior circulation syndrome (POCS)

A
  • Vertebrobasilar arteries

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

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13
Q

Brainstem infarct

A
  • quadriplegia
  • locked-in syndrome (basilar artery)
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14
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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15
Q

Weber syndrome - branches of PCA that supply midbrain

A
  • Ipsilateral CN3 palsy
  • Contralateral weakness of upper + lower limbs
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16
Q

Anterior inferior cerebellar artery (AICA) - lateral pontine syndrome

A
  • Similar to Wallenberg’s (above), but
  • Ipsilateral - facial paralysis and deafness
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17
Q

Retinal/ophthalmic artery

A
  • amaurosis fugax
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18
Q

Stroke by areas

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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19
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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20
Q

Stroke Mx

A

Aspirin 300mg

  • daily for 2wks

Thrombolysis - alteplase (tissue plasminogen activator)

  • <4.5hrs onset of sx, unless CI’d

Thrombectomy

  • <6hrs (with thrombolysis <4.5hrs) if confirmed occlusion of proximal anterior circulation
  • if well 6-24hrs previously + confirmed occlusion of proximal anterior circulation + potential to salvage brain tissue
  • (with thrombolysis <4.5hrs) if well 24hrs previously + confirmed occlusion of proximal posterior circulation + potential to salvage brain tissue

Secondary prevention

  • clopidogrel
  • aspirin + MR dipyridamole
  • statin

Carotid endarterectomy

  • if stenosis >70%
  • consider angioplasty / stenting

Pt with AF

  • warfarin / DOAC
  • start immediately in TIA
  • wait 2wks in acute stroke
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21
Q

Stroke mx of other issues

A

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
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22
Q

TIA

A
  • transient episode of neurological dysfunction caused by focal brain/spinal cord/retinal ischaemia, without acute infarction
  • crescendo TIAs - 2+ TIAs in 1wk - high risk of stroke
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23
Q

TIA Px

A
  • Typically resolve in 1hr
  • Unilateral weakness / sensory loss
  • Aphasia / dysarthria
  • Ataxia, vertigo, loss of balance
  • Visual problems, amaurosis fugax, diplopia, homonymous hemianopia
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24
Q

TIA Ix

A
  • CT
  • MRI, inc diffusion-weighted / blood-sensitive
  • Carotid imaging - doppler, CT/MRI
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25
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
26
Extradural haemorrhage (EDH)
- blood between skull + dura - trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
27
TBI patho
Primary brain injury - at time of trauma - compression, tension, shearing of brain tissue, cell membrane disruption, vascular disruption, inflammation Secondary brain injury - hypoxia, hypotension, raised ICP -> further injury Monro-Kellie doctrine - brain, blood, CSF in cranium - intial LOC, then CSF lost to compensate for increased bleed, then no more left, brain compressed - further LOC - herniation - fixed dilated pupil due to compression of psym fibres of oculomotor nerve
28
EDH Px
- head trauma - initial LOC / drowsiness - lucid interval, later low GCS - headache, N+V, confusion, seizures, focal neurology, fixed dilated pupil, deep coma Cushing's reflex - bradycardia - irregular respirations - HTN / increase in pulse pressure (systolic up, diastolic down)
29
EDH Ix
- bloods, inc G/S - non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
30
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
31
Subdural haemorrhage (SDH)
- blood between dura + arachnoid layers - acute / subacute / chronic
32
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
33
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
34
SDH Ix
- non-contrast CT head - hyperdense crescent (sickle) shaped collection - bloods etc
35
SDH Mx
- A-E - neuroprotection Neurosurgery - Burr hole / open craniotomy, clot evacuation
36
Subarachnoid haemorrhage (SAH)
- bleed into subarachnoid space - between arachnoid / pia (where CSF is)
37
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
38
SAH RFs
- 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan's, EDH, PKD
39
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
40
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
41
SAH Mx
- nimodipine Neurosurgery - endovascular coiling - neurosurgical clipping - intracranial stents / balloons
42
SAH Cx
- rebleed - 20% - cerebral ischaemia - hydrocephalus - VP shunt / EVD - hyponatraemia (SIADH) - fluid restriction
43
Intracerebral haemorrhage
- bleed in brain tissue - spontaneous / secondary to ischaemic stroke, tumours, aneurysm rupture Px - similar as above - stroke, focal neurology, reduced GCS Ix - non-contrast CT head - hyperdensity in substance of brain
44
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
45
Dementia
- irreversible, progressive decline of higher brain function - cognitive/behavioural sx - memory loss, reasoning/communication problems, change in personality, reduction in ADLs RFs - older, mild cognitive impairment, genetics, Parkinson's, CVA, smoking, DM, sedentary, obesity
46
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
47
Alzheimer's Px
- Steady decline - Memory loss - Loss of executive function / dysphasia - Aphasia, apraxia, agnosia - Changes in planning, reasoning, speech, orientation
48
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
49
Vascular dementia (VD)
- 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 RFs - previous stroke/TIA, older, AF, DM, lipids, smoking,.... Types - stroke-related - multi/single-infarct - subcortical - small vessel disease - mixed dementia - VD + Alzheimer's
50
VD Px
- Stepwise progression - stability + acute decline - Cognitive impairment - Mood disturbance - psychosis, delusions, hallucinations, paranoia - Gait, speech, emotional, memory disturbance - Seizures - Focal neurology
51
VD Ix
- screen for depression, psychomotor retardation - formal cognitive screen - MRI - infarcts, white matter changes - Dx with NINDS-AIREN criteria
52
VD Mx
- cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy - address pain, avoid overcrowding, clear communication
53
Lewy body dementia
- type of dementia (10-15%), >50yo - lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
54
Lewy body Px
- progressive cognitive impairment - may fluctuate, early impairments to attention, executive function - sleep disorder - visual hallucinations - Parkinson's like sx - bradykinesia, resting tremor, rigidity
55
Lewy body Mx
- AChE - donepezil, rivastigmine - avoid neuroleptics (may develop Parkinsonism)
56
Frontotemporal dementia (FTD)
- type of dementia (2%), <65yo - neuron damage/death at frontal/temporal lobes, atrophy due to tau protein deposition
57
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 literacy
58
FTD Mx
- maybe SSRIs / trazadone for behavioural sx
59
Dementia general Px
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
60
Dementia DDx
- 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,
61
Dementia Ix
Cognitive screen NICE recommended - 10-CS - 6CIT - 6-item screener - memory impairment screen - mini-cog - Test your memory Not NICE recommended - AMTS - GPCOG - MMSE 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
62
Falls causes
- TLOC - mechanical fall - geriatric fall - visual impairment - BPPV - drugs, polypharmacy
63
TLOC causes
- syncope - seizure - hypoglycaemia - head injury - narcolepsy - stroke
64
Syncope
- transient LOC due to cerebral hypoperfusion - low BP after fall in CO / SVR Reflex - increase in vagal tone - vasovagal - pain/emotions - carotid sinus hypersensitivity - situational syncope - micturition, defecation, cough 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 Cardiac - structural - MI, valvular heart disease, cardiomyopathy - Arrhythmias - tachy/brady
65
Falls RFs
Leg weakness, vision problems, balance / gait disturbance, polypharmacy, incontinence, >65yo, depression, arthritis, psychoactive drugs, cognitive impairment
66
Meds and 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
67
Falls Ix
- 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 - Maybe short synacthen test for adrenal insufficiency
68
Falls Mx
- 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 Postural hypotension - Tx cause, review meds, ensure hydrated - Fludrocortisone - Midodrine – alpha agonist – for true autonomic dysfunction - Falls clinic follow up
69
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
70
MS types
Clinically isolated syndrome - First episode of demyelination - May never have another episode, or go on to develop MS Relapsing-remitting - 85% - Acute attacks (1-2mo), periods of remission Secondary progressive - Deterioration after relapsing-remitting – neuro signs between relapses - 65% with relapsing-remitting go on to develop secondary progressive within 15yrs of dx - See gait / bladder disorders Primary progressive - 10% pts - Progressive deterioration from onset - More common in older pts
71
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
72
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
73
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
74
MS Mx
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/memantine
75
MND
- progressive destruction of motor neurons - cause unknown, 5% have SOD1 mutation - possibly mitochondrial dysfunction - oxidative stress, ROS damages stuff - Frontotemporal dementia in 25%
76
MND types
Amyotrophic lateral sclerosis (ALS) - <80% - most common - Loss of motor neurons in motor cortex and anterior horn of cord – UMN + LMN Progressive bulbar palsy (PBP) - 10-20% - CN9-12 – LMN - Affects muscles of talking + swallowing, tongue also Progressive muscular atrophy (PMA) - <10% - Anterior horn lesion – LMN - Affects distal muscle groups before proximal Primary lateral sclerosis (PLS) - Loss of bets cells in motor cortex – UMN - Marked spastic leg weakness, pseudobulbar palsy, no cognitive decline
77
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 more in notes on px by location
78
MND Ix
- clinical dx - NCS - normal - EMG - reduced no of APs, increased amplitude - MRI - exclude myelopathy etc
79
MND Mx
- riluzole - NIV - resp support - baclofen - PEG - benzos - advanced directives
80
Guillain-Barre syndrome (GBS)
- immune mediated demyelination of PNS - triggered by infection - C jejuni - ABs against infection attack Schwann cells -> demyelination
81
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
82
GBS Ix
- LP - raised protein, normal WCC - NCS - decreased velocity - spirometry - monitor FVC
83
GBS Mx
- FVC <80% - mechanical ventilation - IV Ig 1st line - plasma exchange - VTE prophylaxis
84
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
85
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 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
86
MG Ix
- ABs - AChR, MuSK, LRP4 - CK normal - CT / MRI thorax - thymus - Single fibre electromyography
87
MG Mx
- AChE inhibitors - pyridostigmine - immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate - thymectomy - rituximab
88
Myasthenic 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
89
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
90
Huntington's Px
- 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
91
Huntington's Ix
- genetic testing, genetic counselling
92
Huntington's Mx
- MDT, physio, SALT - chorea - benzos, tetrabenazine, sulpiride - antidepressants - advanced directives
93
Parkinson's
- Progressive degenerative movt disorder caused by degeneration of dopaminergic neurons in substantia nigra (in basal ganglia) - dementia, depression associations - from mitochondrial dysfunction / oxidative stress
94
Parkinson's Px
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 Other 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
95
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
96
Parkinson's Ix
- clinical dx - CT / MRI head - Use UK Parkinson's Disease Society Brain Bank Clinical Diagnostic Criteria - SPECT scan
97
Parkinson's Mx
First line - if motor sx affecting QoL - levodopa - if not - dopamine agonist / levodopa / MAO-B 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.... Dopamine agonist - bromocriptine, cabergoline, pergolide - mimic dopamine action - S/Es - pulm/cardiac fibrosis, impulse control disorders MAO-B inhibitors - selegiline, rasagiline - inhibit MAO-B - break down dopamine, serotonin, adrenaline - can use with levodopa to reduce end of dose sx worsening COMT inhibitors - entacapone - inhibits enzyme that breaks down levodopa - take with levodopa to slow its breakdown Neuropsych sx - anti-depressants, anti-psychotics Anti-muscarinics - block cholinergic receptors - drug-induced Parkinsonism - procyclidine
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Parkinson-plus syndromes
- 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
neurological condition characterised by recurrent seizures Associated conditions - cerebral palsy - tuberous sclerosis - mitochondrial diseases
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Seizure
excessive, abnormal electrical activity in the brain
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Causes of seizures
- 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 - don't occur when alone
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Types of seizures
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 Paeds - infantile spasms / West syndrome - tx with ACTH / vigabatrin - Lennox-Gastaut syndrome - Benign rolandic epilepsy - Juvenile myoclonic epilepsy
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Epilepsy Px
Focal - Motor – Jacksonian march - Sensory – déjà vu, jamais vu, strange smells / tastes / sight / sound Aura - Pt aware - Strange gut feeling, déjà vu, strange smells, flashing lights - Implies focal seizure, but not necessarily in temporal lobe Tonic clonic - Tonic clonic - LOC - Tongue biting - Incontinence - Groaning Post-ictal - Confused, tired, irritable, low - Todd’s paralysis – temporary weakness after seizure - May have dysphasia – from focal seizure in temporal lobe 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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Epilepsy px by 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 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 Parietal lobe (interprets sensations) - Paraesthesia Occipital lobe (vision) - Visual phenomena – spots, lines, flashes
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Epilepsy DDx
- Vasovagal syncope - Non-epileptic attacks - Cardiac syncope - Hypoglycaemia - Hemiplegic migraine - TIA
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Epilepsy Ix
- 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 Mx overview
- 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) - seizure free for >12mo before driving
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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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Pregnancy in epilepsy
- folic acid 5mg OD - carbamazepine least teratogenic - Phenytoin - cleft palate, give vit K in last month of pregnancy also - Lamotrigine - congenital malformation rate low - Breastfeeding - safe for mothers on antiepileptics - No sodium valproate - significant risk of neurodevelopmental delay
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Contraception and epilepsy
If on phenytoin, carbamazepine, barbiturates, primidone, topiramate, oxcarbazepine: - UKMEC 3: the COCP and POP - UKMEC 2: implant - UKMEC 1: Depo-Provera, IUD, IUS If on lamotrigine - UKMEC 3: the COCP - UKMEC 1: POP, implant, Depo-Provera, IUD, IUS If cOCP chosen, should contain minimum 30mcg ethinylestradiol If on levetiracetam - can use all contraceptive types
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Epilepsy cx
- 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
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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Tension headache
- 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 Mx
- paracetamol, NSAIDs, aspirin - limit analgesia to <6d/mo - acupuncture - physio, exercise, CBT, relaxation - trial of amitriptyline
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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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Migraine patho
- 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
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 Hemiplegic - Unilateral limb weakness - Ataxia - Impaired consciousness - Mimic stroke / TIA
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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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Migraine Mx
Acute - NSAIDs, paracetamol - triptans - sumatriptan - stimulate serotonin receptors - antiemetics - prochlorperazine / metoclopramide - no opiates Prevention - avoid triggers - propranolol - topiramate - amitriptyline - specialist - candesartan, valproate, MAbs - menstrual migraines - prophylactic triptans, mefenamic acid, aspirin Pregnancy - paracetamol - NSAIDs 1st/2nd trim - no aspirin/opioids - safe to prescribe HRT
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Cluster headache
- 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
- 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 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 patho
- can be benign/malignant, high/low grade Primary - adults - majority supratentorial, children infratentorial - various types (see notes) 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 - meningococcal septicaemia - bacterial infection spreads to bloodstream - non-blanching rash
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Meningitis causes
Bacterial 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 Viral - coxsackie, HSV, VZV, CMV, HIV, measles, mumps Non-infective - 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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Meningitis LP interpretation
- send for - bacterial culture, viral PCR, cell count, protein, glucose, lactate, ?TB Ix - Bacterial - cloudy, high protein, low glucose, high WCC (neutrophils), culture growth - Viral - clear, mildly high protein, normal glucose, high WCC (lymphocytes), negative culture - TB - slightly cloudy, low glucose, high protein, PCR to dx - delay LP if - sepsis/rash, B/C compromise, bleeding risk, raised ICP
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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
Community - meningococcal: - IM benzylpenicillin Broad spec abx - <3mo – IV cefotaxime + amoxicillin - 3mo-50yo – IV cefotaxime / ceftriaxone - >50yo – cefotaxime / ceftriaxone + amoxicillin Focused abx tx Meningococcal meningitis - IV benzylpenicillin / cefotaxime / ceftriaxone Pneumococcal meningitis - IV cefotaxime / ceftriaxone - add vancomycin if risk of penicillin-resistant pneumococcal infection – recent foreign travel / prolonged abx exposure H influenzae meningitis - IV cefotaxime / ceftriaxone Listeria meningitis - IV amoxicillin + gentamicin - If previous penicillin allergy, consider chloramphenicol - IV dexamethasone - avoid <3mo, shock/meningococcal - IV fluids Viral - self-limiting - acyclovir if ?secondary to HSV Mx of contacts - close contact <7d sx onset - oral ciprofloxacin single dose, rifampicin 2nd line - offer meningococcal vaccination/booster
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Meningitis Cx
- 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 UMN lesion - forehead spared - strokes, tumours - bilateral - MND, pseudobulbar palsies 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
- 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 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 DDx
- sleep apnoea - restless legs in bed - hypothyroid
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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 (various...)
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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
- CT head - MRI - LP - drain CSF, measure opening pressure (do not use in obstructive causes)
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Hydrocephalus 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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Tuberous sclerosis
- autosomal dominant condition causing features in multiple systems - development of hamartomas (benign neoplastic growths) - TSC1/2 mutations - proteins control size/growth of cells
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Tuberous sclerosis Px
- Classical px – child with epilepsy + skin features of tuberous sclerosis Skin signs - Ash leaf spots – depigmented areas - Shagreen patches – thickened, dimpled, pigmented patches of skin - Angiofibromas – small skin-coloured / pigmented papules – on nose / cheeks - Subungual fibromata – fibromas in nail bed – circular, painless lumps, displace nail - Café-au-lait spots – light brown (coffee + milk) flat lesions on skin - Poliosis – patch of white hair on head, eyebrows, eyelashes, beard Neuro signs - Epilepsy - LD, developmental delay Other sx - Rhabdomyoma in heart - Gliomas (tumour of brain + spinal cord) - Polycystic kidneys - Lymphangioleiomyomatosis – abnormal growth in smooth muscle, often affecting lungs (lung cysts) - Retinal hamartomas
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Tuberous sclerosis Mx
- supprotive - mTOR inhibitors - tx cx
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Aphasia
Wernicke’s (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 Broca’s (expressive) - Lesion of inferior frontal gyrus, superior division of left MCA - Speech non-fluent, laboured, halting, repetition impaired - Comprehension normal Conduction aphasia - From stroke affecting arcuate fasciculus – connection between Wernicke’s and Broca’s - Speech fluent, repetition poor - Comprehension normal Global aphasia - Large legion affecting all three areas above - Severe expressive and receptive aphasia
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Restless legs syndrome (RLS)
- spontaneous, continuous lower limb movts, associated with paraesthesia Causes - FHx, IDA, uraemia, DM, pregnancy
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RLS 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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RLS Ix
- Clinical dx - Ferritin - r/o iron deficiency anaemia
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RLS 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, also under pain specialist
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Vestibular schwannoma
- acoustic neuroma - 90% cerebellopontine angle tumours - Bl in NF2 Px - CN8 – vertigo, unilateral sensorineural hearing loss, unilateral tinnitus - CN5 – absent corneal reflex - CN7 – facial palsy Ix - Urgent ENT referral - MRI of cerebellopontine angle - Audiometry Mx - Surgery - Radiotherapy - Watch and wait
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Pituitary apoplexy
- sudden enlargement of pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage/infarction - eg HTN, pregnancy, trauma, anticoags Px - sudden onset thunderclap headache - vomiting, neck stiffness - Bitemporal superior quadrantic visual field defect - Extraocular nerve palsies - Features of pituitary insufficiency – eg hypotension / hyponatraemia secondary to hypoadrenalism Ix - MRI Mx - steroid replacement (loss of ACTH) - careful 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 Px
- cape-like distribution - neck, shoulders, arms - loss of temp sensation - light touch, proprioception, vibration preserved - spastic weakness - lower limbs - neuropathic pain - upgoing plantars - bladder/bowel dysfunction - Horner's syndrome (rare) - scoliosis years later
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Syringomyelia Ix
- full spine MRI with contrast - brain MRI (exclude chiari)
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Syringomyelia Mx
- tx cause - shunt if persistent
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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 Homonymous hemianopia - Incongruous – optic tract lesion - Congruous – optic radiation / occipital cortex - Macula sparing – occipital cortex Homonymous quadrantanopias - Superior – lesion of inferior optic radiations, temporal lobe, Meyer’s loop - Inferior – superior optic radiations, parietal lobe, Baum’s 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
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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
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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
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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
225
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
226
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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Long thoracic nerve palsy
Causes - Axillary surgery, stab wound, carrying backpack for long time Motor - Serratus anterior – scapula winging, impaired abduction of arm beyond 90 degrees
228
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)
229
Inferior gluteal nerve palsy (L5-S2)
Causes - Posterior hip dislocation Motor - Paralysis of glut maximus – impaired thigh extension – difficulty standing up, climbing stairs
230
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)
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Lateral femoral cutaneous palsy (L2-3)
Causes - Compression at level of inguinal ligament – increased abdo pressure, external compression, tumours Sensory - Pain, paraesthesia – lateral surface of anterior thigh
232
Obturator nerve palsy (L2-3)
Causes - Pelvic ring fracture, obturator hernia Motor - Paralysis of hip adductors Sensory - Medial thigh
233
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
234
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
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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
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Sural nerve palsy (L4-S3)
Causes - Achilles tendon rupture, entrapment Sensory - Posterolateral lower leg, lateral border of foot, small area under heel
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Olfactory (I)
- Palsy – change in smell
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Optic (II)
- Palsy – change in sight – see visual field defects
239
Oculomotor (III)
- All eye muscles except LR and SO. Pupil constriction, accommodation, eyelid opening - Palsy – ptosis, down + out eye, fixed dilated pupil
240
Trochlear (IV)
- Superior oblique - Palsy – defective downward gaze – vertical diplopia – noticed when looking down – head tilt to compensate
241
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
242
Abducens (VI)
- Lateral rectus - Palsy – defective abduction – horizontal diplopia
243
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
244
Vestibulocochlear (VIII)
- Hearing, balance - Palsy – hearing loss, vertigo, nystagmus. Acoustic neuromas – Schwann cell tumours of cochlear nerve
245
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)
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Vagus (X)
- Phonation, swallowing, innervates viscera - Palsy – uvula deviates away from site of lesion, loss of gag reflex (efferent)
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Accessory (XI)
- Head + shoulder movt - Palsy – weakness turning head to contralateral side, weakness shrugging shoulder
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Hypoglossal (XII)
- Tongue movt - Palsy – tongue deviates towards side of lesion
249
ALS
- Affects corticospinal tracts (UMN) and LMNs - upper and lower motor neuron signs
250
Poliomyelitis
Affects anterior horns - LMN signs
251
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)
252
Friedrich's ataxia
- Same as above - Other features of cerebellar disease as well as ataxia - eg intention tremor
253
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
254
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
255
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
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Neurosyphilis
Dorsal columns - loss of proprioception, vibration (o Eg from B12 deficiency, MS, tumour compression, neck hyperextension, posterior spinal artery infarct o DCML – loss of fine touch, vibration, proprioception Wide steppage gait – sensory ataxia. Positive Romberg’s)
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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 Lateral corticospinal tracts – bilateral spastic paresis DCML – bilateral loss of proprioception + vibration Spinocerebellar tracts – bilateral limb ataxia
258
Cerebral venous sinus thrombosis (CVST)
- thrombosis in brain's venous sinuses - prevents blood draining - 50% isolated sagittal sinus - also cavernous sinus thrombosis / lateral sinus
259
CVST Px
- sudden headache - N+V - reduced GCS
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CVST Ix
- MRI venography, CT alternative - D dimer elevated maybe
261
CVST Mx
- anticoagulation - LMWH, then warfarin
262
CVST specific syndromes
Sagittal sinus thrombosis - Seizures, hemiplegia - Parasagittal biparetial / bifrontal haemorrhagic infarctions sometimes seen - Empty delta sign on venography Cavernous sinus thrombosis - Other causes – local infection (sinusitis), neoplasia, trauma - Periorbital oedema - Ophthalmoplegia – 6th nerve damage, then 3/4th - Trigeminal nerve involvement – hyperaesthesia of upper face, eye pain - Central retinal vein thrombosis Lateral sinus thrombosis - 6/7th CN palsies
263
Degenerative cervical myelopathy (DCM)
- stenosis of c-spine leading to spinal cord compression, neurology - age-related osteoarthritis
264
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
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DCM Ix
MRI c spine
266
DCM Mx
- refer for spinal services assessment - decompressive surgery
267
Ataxia telangiectasia
- Autosomal recessive disorder – defect in ATM gene – codes for DNA repair enzymes - An inherited combined immunodeficiency disorder Px - Early childhood - Cerebellar ataxia - Telangiectasia (spider angiomas) - IgA deficiency – recurrent chest infections - 10% risk of malignancy, lymphoma, leukaemia, non-lymphoid tumours
268
Friedreich's ataxia
- Autosomal recessive, trinucleotide repeat disorder (GAA repeat in X25 gene on c9) - Does not demonstrate anticipation Px - 10-15yo - Gait ataxia - Kyphoscoliosis - Neuro sx - absent ankle jerks / extensor plantars, cerebellar ataxia, optic atrophy, spinocerebellar tract degeneration - HOCM - DM - High-arched palate
269
Foot drop causes
- 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
270
Internuclear ophthalmoplegia
- a cause of horizontal dysconjugate eye movt - due to lesion in medial longitudinal fasciculus (MLF) Causes - MS - vascular disease Px - Impaired adduction of eye on same side as lesion - Horizontal nystagmus of abducting eye on contralateral eye
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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
272
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
273
Dystrophinopathies
- x-linked recessive - due to mutation in dystrophin gene - dystrophin connects muscle membrane to actin, part of cytoskeleton
274
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
275
Becker muscular dystrophy
- Non-frameshift insertion – both binding sites preserved – milder form Px - After 10yo - Intellectual impairment much less common
276
Cataplexy
- Sudden / transient loss of muscular tone caused by strong emotion – eg laughter, being frightened. 2/3 pts who have narcolepsy have cataplexy Px - Buckling knees - Collapse
277
Thoracic outlet syndrome (TOS)
- Compression of brachial plexus, subclavian artery, or vein at site of thoracic outlet (space between clavicle and first rib) - neurogenic (90%) / vascular - pts usually thin young women, long neck, drooping shoulder - after neck trauma, may have cervical rib
278
TOS Px
Neuro - painless muscle wasting of hand muscles - weak grasp - numbness/tingling - autonomic nerves - cold hands, blanching, swelling Vascular - vein - diffuse arm swelling, distended veins - artery - claudication, ulceration/gangrene if severe
279
TOS Ix
- CXR, c-spine XR - CT / MRI - venography / angiography - anterior scalene block - confirm neurogenic TOS
280
TOS Mx
- Conservative mx - education, rehab, physio, taping - Surgical decompression - Botox?
281
Multiple system atrophy (MSA)
- Parkinsonism - Autonomic disturbance - erectile dysfunction, postural hypotension, atonic bladder - Cerebellar signs
282
Cavernous sinus
- On body of sphenoid bone - runs from superior orbital fissure to petrous temporal bone - Medial - is pituitary fossa, sphenoid sinus - Lateral - temporal lobe Lateral wall components - from top to bottom - Oculomotor nerve - Trochlear nerve - Ophthalmic nerve - Maxillary nerve Contents of sinus - from medial to lateral - Internal carotid artery (and sympathetic plexus) - Abducens nerve Blood supply - Ophthalmic vein, superficial cortical veins, basilar plexus of veins posteriorly. - Drains into the internal jugular vein via: the superior and inferior petrosal sinuses
283
Paroxysmal hemicrania
- Attacks of severe, unilateral headache - usually orbital, supraorbital, temporal region - Autonomic features also - Last 30 mins, multiple times a day - Tx with indomethacin
284
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
285
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
286
Neuropathy
pathological process affecting nerve(s)
287
Mononeuropathy
- single nerve affected - eg CTS
288
Mononeuritis multiplex
Several individual nerves affected Systemic causes - WARDS PLC - Wegener's granulomatosis - AIDs/amyloid - RA - DM - Sarcoidosis - PAN (polyarteritis nodosa) - Leprosy - Carcinoma
289
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
290
Spontaneous intracranial hypotension
- CSF leak causes headaches - Leak typically from thoracic nerve root sleeves - RFs - Marfan's - Headache worse upright - Ix - MRI with gadolinium - Mx - conservative, epidural blood patch
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Von Hippel-Lindau syndrome
- Autosomal dominant condition – predisposes to neoplasia - Abnormality in VHL gene on c3 Features - Cerebellar haemangiomas – cause SAHs - Retinal haemangiomas - Renal cysts, and elsewhere - Phaeochromocytoma - RCC
Phase 4 Question Knowledge
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