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Phase 4 Question Knowledge > Neuro > Flashcards

Neuro Flashcards

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
Q

TIA Mx

A
  • 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
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26
Q

Extradural haemorrhage (EDH)

A
  • blood between skull + dura
  • trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
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27
Q

TBI patho

A

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
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28
Q

EDH Px

A
  • 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)
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29
Q

EDH Ix

A
  • bloods, inc G/S
  • non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
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30
Q

EDH Mx

A
  • 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
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31
Q

Subdural haemorrhage (SDH)

A
  • blood between dura + arachnoid layers
  • acute / subacute / chronic
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32
Q

SDH patho

A
  • 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
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33
Q

SDH Px

A
  • 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
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34
Q

SDH Ix

A
  • non-contrast CT head - hyperdense crescent (sickle) shaped collection
  • bloods etc
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35
Q

SDH Mx

A
  • A-E
  • neuroprotection

Neurosurgery

  • Burr hole / open craniotomy, clot evacuation
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36
Q

Subarachnoid haemorrhage (SAH)

A
  • bleed into subarachnoid space - between arachnoid / pia (where CSF is)
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37
Q

SAH causes

A

Traumatic

  • most common cause of SAH

Spont

  • Berry aneurysm - 85% spont cases - HTN, PKD, EDS, coarc aorta
  • AV malformation
  • pituitary apoplexy
  • mycotic aneurysms
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38
Q

SAH RFs

A
  • 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan’s, EDH, PKD
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39
Q

SAH Px

A
  • 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
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40
Q

SAH Ix

A

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

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41
Q

SAH Mx

A
  • nimodipine

Neurosurgery

  • endovascular coiling
  • neurosurgical clipping
  • intracranial stents / balloons
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42
Q

SAH Cx

A
  • rebleed - 20%
  • cerebral ischaemia
  • hydrocephalus - VP shunt / EVD
  • hyponatraemia (SIADH) - fluid restriction
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43
Q

Intracerebral haemorrhage

A
  • 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
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44
Q

Head injury NICE criteria for CT head, >16yo

A

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
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45
Q

Dementia

A
  • 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
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46
Q

Alzheimer’s dementia

A
  • type of dementia (50-75%)
  • amyloid plaques / tau proteins in brain - reduce transmission, brain cell death
  • widespread cerebral atrophy, esp cortex / hippocampus
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47
Q

Alzheimer’s Px

A
  • Steady decline
  • Memory loss
  • Loss of executive function / dysphasia
  • Aphasia, apraxia, agnosia
  • Changes in planning, reasoning, speech, orientation
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48
Q

Alzheimer’s Mx

A
  • 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
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49
Q

Vascular dementia (VD)

A
  • 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
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50
Q

VD Px

A
  • Stepwise progression - stability + acute decline
  • Cognitive impairment
  • Mood disturbance - psychosis, delusions, hallucinations, paranoia
  • Gait, speech, emotional, memory disturbance
  • Seizures
  • Focal neurology
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51
Q

VD Ix

A
  • screen for depression, psychomotor retardation
  • formal cognitive screen
  • MRI - infarcts, white matter changes
  • Dx with NINDS-AIREN criteria
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52
Q

VD Mx

A
  • cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy
  • address pain, avoid overcrowding, clear communication
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53
Q

Lewy body dementia

A
  • type of dementia (10-15%), >50yo
  • lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
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54
Q

Lewy body Px

A
  • progressive cognitive impairment - may fluctuate, early impairments to attention, executive function
  • sleep disorder
  • visual hallucinations
  • Parkinson’s like sx - bradykinesia, resting tremor, rigidity
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55
Q

Lewy body Mx

A
  • AChE - donepezil, rivastigmine
  • avoid neuroleptics (may develop Parkinsonism)
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56
Q

Frontotemporal dementia (FTD)

A
  • type of dementia (2%), <65yo
  • neuron damage/death at frontal/temporal lobes, atrophy due to tau protein deposition
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57
Q

FTD Px

A

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
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58
Q

FTD Mx

A
  • maybe SSRIs / trazadone for behavioural sx
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59
Q

Dementia general Px

A

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
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60
Q

Dementia DDx

A
  • 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,
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61
Q

Dementia Ix

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

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
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62
Q

Falls causes

A
  • TLOC
  • mechanical fall
  • geriatric fall
  • visual impairment
  • BPPV
  • drugs, polypharmacy
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63
Q

TLOC causes

A
  • syncope
  • seizure
  • hypoglycaemia
  • head injury
  • narcolepsy
  • stroke
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64
Q

Syncope

A
  • 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
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65
Q

Falls RFs

A

Leg weakness, vision problems, balance / gait disturbance, polypharmacy, incontinence, >65yo, depression, arthritis, psychoactive drugs, cognitive impairment

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66
Q

Meds and falls

A

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
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67
Q

Falls Ix

A
  • 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
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68
Q

Falls Mx

A
  • 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
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69
Q

Multiple sclerosis (MS)

A
  • 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
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70
Q

MS types

A

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
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71
Q

MS Px

A
  • 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
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72
Q

MS Dx

A
  • 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
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73
Q

MS Ix

A
  • 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
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74
Q

MS Mx

A

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
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75
Q

MND

A
  • progressive destruction of motor neurons
  • cause unknown, 5% have SOD1 mutation
  • possibly mitochondrial dysfunction - oxidative stress, ROS damages stuff
  • Frontotemporal dementia in 25%
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76
Q

MND types

A

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
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77
Q

MND Px

A
  • 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

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78
Q

MND Ix

A
  • clinical dx
  • NCS - normal
  • EMG - reduced no of APs, increased amplitude
  • MRI - exclude myelopathy etc
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79
Q

MND Mx

A
  • riluzole
  • NIV - resp support
  • baclofen
  • PEG
  • benzos
  • advanced directives
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80
Q

Guillain-Barre syndrome (GBS)

A
  • immune mediated demyelination of PNS
  • triggered by infection - C jejuni
  • ABs against infection attack Schwann cells -> demyelination
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81
Q

GBS Px

A
  • 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
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82
Q

GBS Ix

A
  • LP - raised protein, normal WCC
  • NCS - decreased velocity
  • spirometry - monitor FVC
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83
Q

GBS Mx

A
  • FVC <80% - mechanical ventilation
  • IV Ig 1st line
  • plasma exchange
  • VTE prophylaxis
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84
Q

Myasthenia gravis (MG)

A
  • 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
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85
Q

MG Px

A
  • 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
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86
Q

MG Ix

A
  • ABs - AChR, MuSK, LRP4
  • CK normal
  • CT / MRI thorax - thymus
  • Single fibre electromyography
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87
Q

MG Mx

A
  • AChE inhibitors - pyridostigmine
  • immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate
  • thymectomy
  • rituximab
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88
Q

Myasthenic crisis

A
  • 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
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89
Q

Huntington’s

A
  • 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
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90
Q

Huntington’s Px

A
  • 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
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91
Q

Huntington’s Ix

A
  • genetic testing, genetic counselling
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92
Q

Huntington’s Mx

A
  • MDT, physio, SALT
  • chorea - benzos, tetrabenazine, sulpiride
  • antidepressants
  • advanced directives
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93
Q

Parkinson’s

A
  • Progressive degenerative movt disorder caused by degeneration of dopaminergic neurons in substantia nigra (in basal ganglia)
  • dementia, depression associations
  • from mitochondrial dysfunction / oxidative stress
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94
Q

Parkinson’s Px

A

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
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95
Q

Parkinson’s DDx

A

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
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96
Q

Parkinson’s Ix

A
  • clinical dx
  • CT / MRI head
  • Use UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria
  • SPECT scan
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97
Q

Parkinson’s Mx

A

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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98
Q

Parkinson-plus syndromes

A
  • 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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99
Q

Epilepsy

A

neurological condition characterised by recurrent seizures

Associated conditions

  • cerebral palsy
  • tuberous sclerosis
  • mitochondrial diseases
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100
Q

Seizure

A

excessive, abnormal electrical activity in the brain

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101
Q

Causes of seizures

A
  • epilepsy
  • febrile convulsions
  • alcohol withdrawal
  • non-epileptic
  • idiopathic
  • stroke, haemorrhage
  • head injury
  • Alzheimer’s, dementia
  • tumour
  • metabolic disturbance - electrolytes
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102
Q

Non-epileptic seizure px

A
  • 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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103
Q

Types of seizures

A

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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104
Q

Epilepsy Px

A

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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105
Q

Epilepsy px by lobe

A

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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106
Q

Epilepsy DDx

A
  • Vasovagal syncope
  • Non-epileptic attacks
  • Cardiac syncope
  • Hypoglycaemia
  • Hemiplegic migraine
  • TIA
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107
Q

Epilepsy Ix

A
  • 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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108
Q

Epilepsy Mx overview

A
  • 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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109
Q

Generalised tonic clonic Mx

A

M - sodium valproate
F - lamotrigine / levetiracetam

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110
Q

Focal seizure Mx

A

1st - lamotrigine / levetiracetam
2nd - carbamazepine

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111
Q

Absence seizure Mx

A

1st - ethosuximide
2nd - M - sodium valproate
- F - lamotrigine / levetiracetam

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112
Q

Myoclonic seizures Mx

A

M - sodium valproate
F - levetiracetam

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113
Q

Tonic/ atonic seizures Mx

A

M - sodium valproate
F - lamotrigine

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114
Q

Pregnancy in epilepsy

A
  • 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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115
Q

Contraception and epilepsy

A

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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116
Q

Epilepsy cx

A
  • aspiration pneumonia
  • sudden unexpected death in epilepsy (SUDEP)
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117
Q

Status epilepticus

A
  • seizure > 5mins
  • or multiple seizures without regaining consciousness in between
118
Q

Status epilepticus Ix

A
  • 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
119
Q

Status epilepticus Mx

A
  • 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

120
Q

Headache

A

Primary

  • no underlying cause
  • tension, migraine, cluster

Secondary

  • underlying cause
  • eg GCA, glaucoma, haemorrhage, meds….
121
Q

Headache red flags

A
  • 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
122
Q

Tension headache

A
  • common primary headache - most common cause of primary headache in adults
  • infrequent/frequent episodic, chronic

Causes

  • eg stress, depression, alcohol, skipping meals, dehydration
123
Q

Tension headache Px

A
  • Episodic, 30 mins - 7d
  • Like tight band around head
  • No aura, N+V, not aggravated by routine physical activity
  • May have photophobia / phonophobia
124
Q

Tension headache Mx

A
  • paracetamol, NSAIDs, aspirin
  • limit analgesia to <6d/mo
  • acupuncture
  • physio, exercise, CBT, relaxation
  • trial of amitriptyline
125
Q

Migraine

A
  • 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
126
Q

Migraine patho

A
  • 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
127
Q

Migraine Px

A

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
128
Q

Migraine dx criteria (5,4,3,2,1)

A

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

129
Q

Migraine Mx

A

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
130
Q

Cluster headache

A
  • most disabling type of primary headache
  • potential triggers - alcohol, strong smells, exercise
131
Q

Cluster headache Px

A
  • 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
132
Q

Cluster headache Ix

A
  • MRI with gadolinium contrast
133
Q

Cluster headache Mx

A
  • neurology referral

Acute

  • 15L O2
  • S/C triptan (or intranasal)

Prophylaxis

  • verapamil
  • occipital nerve block, prednisolone, lithium
134
Q

Medication overuse headache Px

A
  • Present for >15d per month
  • Developed/worsened whilst taking regular symptomatic medication
  • Pts using opioids and triptans at most risk
  • May be psychiatric comorbidity
135
Q

Medication overuse headache Mx

A
  • withdraw simple analgesics + triptans abruptly (may worsen headache initially)
  • gradually withdraw opioids

Withdrawal sx

  • Vomiting, hypotension, tachycardia, restlessness, sleep disturbance, anxiety
136
Q

Post-LP headache

A
  • 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
137
Q

Sinusitis

A
  • 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)
138
Q

Cervical spondylosis

A
  • Caused by degenerative changes in c-spine
  • Neck pain, worse on movt, often also headaches
139
Q

Hormonal headache

A
  • 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
140
Q

Brain tumour

A
  • primary / secondary (10x more common)
141
Q

Brain tumour patho

A
  • 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
142
Q

Brain tumour Px

A

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
143
Q

Brain tumours Ix

A
  • CT with contrast
  • MRI brain
  • Biopsy - during surgery
  • LP CI’d (raised ICP)
144
Q

Brain tumours Mx

A
  • surgery
  • chemo / radio
  • dexamethasone
  • levetiracetam
  • palliative care
145
Q

Meningitis

A
  • inflammation of meninges
  • viral most common, presume bacterial until otherwise, also fungal, non-infective
  • meningococcal septicaemia - bacterial infection spreads to bloodstream - non-blanching rash
146
Q

Meningitis causes

A

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
147
Q

Meningitis Px

A
  • fever
  • neck stiffness
  • vomiting
  • headache
  • photophobia
  • altered consciousness
  • seizures
  • non-blanching rash
  • Kernig’s
  • Brudzinski’s
148
Q

Meningitis LP interpretation

A
  • 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
149
Q

Meningitis Ix

A
  • LP
  • bloods - cultures, meningococcal PCR, FBC, U/E, glucose, lactate, coag, VBG
  • maybe throat swab
  • CT not normally indicated
150
Q

Meningitis Mx

A

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
151
Q

Meningitis Cx

A
  • 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
152
Q

Encephalitis

A
  • infection / inflammation of brain parenchyma
  • mostly viral
  • typically temporal / inferior frontal lobes
153
Q

Encephalitis causes

A

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
154
Q

Encephalitis Px

A
  • Fever
  • Headache
  • Psych sx, unusual behaviour
  • Altered cognition, consciousness
  • Seizures, often focal
  • Vomiting
  • Focal neurology, eg aphasia
155
Q

Encephalitis Ix

A
  • 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
156
Q

Encephalitis Mx

A
  • IV acyclovir
157
Q

Facial nerve palsy

A
  • 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….
158
Q

Bell’s palsy

A
  • acute, unilateral idiopathic facial nerve paralysis
  • cause unknown, maybe HSV involvement
159
Q

Bell’s palsy Px

A
  • 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
160
Q

Bell’s palsy dx

A
  • dx made when no other cause for paralysis found
161
Q

Bell’s palsy Mx

A
  • oral prednisolone within 72hrs
  • eye care - drops, tape closed at night
  • refer if atypical sx / worsening / no improvement
162
Q

Ramsay Hunt syndrome

A
  • reactivation of VZV in geniculate ganglion of CN7

Px

  • auricular pain
  • facial nerve palsy
  • vesicular rash around ear
  • vertigo, tinnitus

Mx

  • oral acyclovir
  • corticosteroids
163
Q

Brain abscess

A
  • pus-filled swelling in brain

Causes

  • from middle ear / sinuses
  • trauma
  • surgery to scalp
  • penetrating head injuries
  • emboli from IE
164
Q

Brain abscess Px

A
  • headache - dull, persistent
  • fever
  • focal neurology - site dependent
  • raised ICP - nausea, papilloedema, seizures
165
Q

Brain abscess Ix

A
  • CT head
166
Q

Brain abscess mx

A
  • surgery - craniotomy, debride
  • IV abx - ceftriaxone + metronidazole
  • dexamethasone
167
Q

Charcot-Marie-Tooth disease

A
  • 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
168
Q

DDx peripheral neuropathy

A
  • alcohol
  • B12 deficiency
  • cancer (myeloma), CKD
  • diabetes, drugs (amiodarone, isoniazid, nitrofurantoin, metronidazole)
  • vasculitis
169
Q

Essential tremor

A
  • autosomal dominant condition - tremor usually affecting upper limbs/hands
170
Q

Essential tremor Px

A
  • postural tremor (worse on movt) 6-12Hz
  • symmetrical
  • improved with alcohol/rest
  • worse when tired, stressed, caffeine
  • absent during sleep
  • titubation - head tremor
171
Q

Essential tremor Mx

A
  • propranalol
  • primidone
172
Q

Idiopathic intracranial HTN (IIH)

A
  • build up of pressure around brain

RFs

  • female, obese, pregnancy, drugs…
173
Q

IIH Px

A
  • headache, worse in morning/coughing/straining
  • nausea
  • blurred vision
  • papilloedema
  • enlarged blind spot
  • sixth nerve palsy
174
Q

IIH Ix

A
  • assess neurology, eyes, vision
  • CT / MRI
  • LP
175
Q

IIH Mx

A
  • wt loss
  • acetazolamide / topiramate - act as diuretics
  • repeated LP
  • surgery - eg shunt / optic nerve sheath decompression
176
Q

Narcolepsy

A
  • long term brain condition causing pt to fall asleep suddenly at inappropriate times
  • low levels orexin (hypocretin)
177
Q

Narcolepsy Px

A
  • 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
178
Q

Narcolepsy DDx

A
  • sleep apnoea
  • restless legs in bed
  • hypothyroid
179
Q

Narcolepsy Ix

A
  • multiple sleep latency EEG
  • CSF orexin levels - low
180
Q

Narcolepsy Mx

A
  • Daytime stimulants - modafinil
  • Night time - sodium oxybate
181
Q

Trigeminal neuralgia

A
  • 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
182
Q

Trigeminal neuralgia Px

A
  • intense facial pain, seconds-hrs
  • electricity-like
  • attacks worsen over time
183
Q

Trigeminal neuralgia red flags for serious underlying cause

A
  • Sensory changes
  • Deafness, ear problems
  • Skin / oral lesions
  • Pain only in ophthalmic division, or bilaterally
  • Optic neuritis
  • MS FHx
  • <40yo
184
Q

Trigeminal neuralgia Mx

A
  • carbamazepine
  • neurology referral if atypical features, eg <50yo
  • surgery to decompress/intentionally damage nerve
185
Q

Neurofibromatosis

A
  • genetic condition that causes nerve tumours (neuromas) to develop throughout nervous system
  • tumours benign, but cause neuro/structural problems
  • NF1>NF2
186
Q

NF1

A
  • 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…)
187
Q

NF2

A
  • 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
188
Q

Hydrocephalus

A
  • 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
189
Q

Hydrocephalus Px

A
  • raised ICP sx
  • headache - worse in morning, lying down, Valsalva
  • N+V
  • papilloedema
  • coma
  • infants increased head circumference
190
Q

Hydrocephalus Ix

A
  • CT head
  • MRI
  • LP - drain CSF, measure opening pressure (do not use in obstructive causes)
191
Q

Hydrocephalus Mx

A
  • external ventricular drain (EVD)
  • ventriculoperitoneal shunt (VP shunt)
  • surgical tx for obstructive pathology
192
Q

Normal pressure hydrocephalus

A
  • reversible cause of dementia in elderly
  • reduced CSF absorption at arachnoid villi / head injury / SAH / meningitis
193
Q

Normal pressure hydrocephalus Px

A
  • urinary incontinence
  • dementia, bradyphrenia (slowed thinking)
  • gait abnormality
  • sx develop over a few months
194
Q

Normal pressure hydrocephalus Ix

A
  • CT head - hydrocephalus, ventriculomegaly, no sulcal enlargement
195
Q

Normal pressure hydrocephalus Mx

A
  • VP shunt
196
Q

Tuberous sclerosis

A
  • autosomal dominant condition causing features in multiple systems
  • development of hamartomas (benign neoplastic growths)
  • TSC1/2 mutations - proteins control size/growth of cells
197
Q

Tuberous sclerosis Px

A
  • 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
198
Q

Tuberous sclerosis Mx

A
  • supprotive
  • mTOR inhibitors
  • tx cx
199
Q

Aphasia

A

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
200
Q

Restless legs syndrome (RLS)

A
  • spontaneous, continuous lower limb movts, associated with paraesthesia

Causes

  • FHx, IDA, uraemia, DM, pregnancy
201
Q

RLS Px

A
  • 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)
202
Q

RLS Ix

A
  • Clinical dx
  • Ferritin - r/o iron deficiency anaemia
203
Q

RLS Mx

A
  • Walking, stretching, massaging
  • Iron
  • Dopamine agonist 1st line - pramipexole, ropinirole
  • Benzos
  • Gabapentin
204
Q

Reye’s syndrome

A
  • 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
205
Q

Lambert-Eaton myasthenic syndrome

A
  • paraneoplastic syndrome of reduced ACh release at NMJ
  • SCLC, breast, ovarian, also autoimmune
  • AB against presynaptic voltage-gated Ca channels in PNS
206
Q

Lambert-Eaton myasthenic syndrome Px

A
  • limb girdle weakness (lower limbs first)
  • hyporeflexia
  • exercise improves sx
  • autonomic - dry mouth, impotence, difficulty peeing
  • no ptosis (MG)
207
Q

Lambert-Eaton myasthenic syndrome Ix

A
  • EMG - incremental response to repetitive electrical stimulation
208
Q

Lambert-Eaton myasthenic syndrome Mx

A
  • tx cancer
  • immunosuppression - prednisolone / azathioprine
  • amifampridine / 3,4-diaminopyridine
  • pyridostigmine
  • IVIg, plasma exchange
209
Q

Neuropathic pain

A
  • 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
210
Q

Complex regional pain syndrome

A
  • 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
211
Q

Vestibular schwannoma

A
  • 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
212
Q

Pituitary apoplexy

A
  • 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
213
Q

Syringomyelia

A
  • collection of CSF in spinal cord
  • syringobulbia - fluid-filled cavity in medulla

Causes

  • chiari malformation
  • trauma
  • tumours
  • idiopathic
214
Q

Syringomyelia Px

A
  • 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
215
Q

Syringomyelia Ix

A
  • full spine MRI with contrast
  • brain MRI (exclude chiari)
216
Q

Syringomyelia Mx

A
  • tx cause
  • shunt if persistent
217
Q

Autonomic dysreflexia

A
  • 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
218
Q

Visual field defects

A
  • 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
219
Q

Arnold-Chiari malformation

A
  • Downward displacement / herniation of cerebellar tonsils through foramen magnum – congenital / acquired through trauma

Px

  • Hydrocephalus from obstruction of CSF outflow
  • Headache
  • Syringomyelia
220
Q

Erb’s palsy

A
  • 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
221
Q

Klumpke’s palsy

A
  • 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
222
Q

Axillary nerve palsy (C5-6)

A

Causes

  • Anterior shoulder dislocation
  • Fracture surgical neck of humerus

Motor

  • Deltoid – impaired abduction, atrophy
  • Teres minor – impaired external rotation

Sensory

  • Lateral shoulder
223
Q

Musculocutaneous nerve palsy (C5-7)

A

Causes

  • Trauma, Erb’s

Motor

  • Impaired elbow flexion – brachialis / coracobrachialis
  • Impaired forearm supination – biceps brachii

Sensory

  • Lateral forearm, elbow to base of thumb
224
Q

Median nerve palsy (C5-T1)

A

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
Q

Radial nerve palsy (C5-T1)

A

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
Q

Ulnar nerve palsy (C7-T1)

A

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
227
Q

Long thoracic nerve palsy

A

Causes

  • Axillary surgery, stab wound, carrying backpack for long time

Motor

  • Serratus anterior – scapula winging, impaired abduction of arm beyond 90 degrees
228
Q

Superior gluteal nerve palsy (L4-S1)

A

Causes

  • IM injection

Motor

  • Paralysis of glut medius / minimus – impaired hip abduction. Positive Trendelenburg (lateral pelvic tilt towards opposite side)
229
Q

Inferior gluteal nerve palsy (L5-S2)

A

Causes

  • Posterior hip dislocation

Motor

  • Paralysis of glut maximus – impaired thigh extension – difficulty standing up, climbing stairs
230
Q

Femoral nerve palsy (L2-4)

A

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)
231
Q

Lateral femoral cutaneous palsy (L2-3)

A

Causes

  • Compression at level of inguinal ligament – increased abdo pressure, external compression, tumours

Sensory

  • Pain, paraesthesia – lateral surface of anterior thigh
232
Q

Obturator nerve palsy (L2-3)

A

Causes

  • Pelvic ring fracture, obturator hernia

Motor

  • Paralysis of hip adductors

Sensory

  • Medial thigh
233
Q

Sciatic nerve palsy (L4-S3)

A

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
Q

Tibial nerve palsy (L4-S3)

A

Causes

  • Tibial fracture, tarsal tunnel syndrome

Motor

  • Impaired foot inversion / plantarflexion – can’t walk on toes, cannot invert foot (TIPPED)

Sensory

  • Sole of foot
235
Q

Common peroneal nerve palsy (L4-S2)

A

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
236
Q

Sural nerve palsy (L4-S3)

A

Causes

  • Achilles tendon rupture, entrapment

Sensory

  • Posterolateral lower leg, lateral border of foot, small area under heel
237
Q

Olfactory (I)

A
  • Palsy – change in smell
238
Q

Optic (II)

A
  • Palsy – change in sight – see visual field defects
239
Q

Oculomotor (III)

A
  • All eye muscles except LR and SO. Pupil constriction, accommodation, eyelid opening
  • Palsy – ptosis, down + out eye, fixed dilated pupil
240
Q

Trochlear (IV)

A
  • Superior oblique
  • Palsy – defective downward gaze – vertical diplopia – noticed when looking down – head tilt to compensate
241
Q

Trigeminal (V)

A
  • 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
Q

Abducens (VI)

A
  • Lateral rectus
  • Palsy – defective abduction – horizontal diplopia
243
Q

Facial (VII)

A
  • 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
Q

Vestibulocochlear (VIII)

A
  • Hearing, balance
  • Palsy – hearing loss, vertigo, nystagmus. Acoustic neuromas – Schwann cell tumours of cochlear nerve
245
Q

Glossopharyngeal (IX)

A
  • Taste posterior 1/3 tongue. Salivation, swallowing, mediates input from carotid body + sinus
  • Palsy – hypersensitive carotid sinus reflex, loss of gag reflex (afferent)
246
Q

Vagus (X)

A
  • Phonation, swallowing, innervates viscera
  • Palsy – uvula deviates away from site of lesion, loss of gag reflex (efferent)
247
Q

Accessory (XI)

A
  • Head + shoulder movt
  • Palsy – weakness turning head to contralateral side, weakness shrugging shoulder
248
Q

Hypoglossal (XII)

A
  • Tongue movt
  • Palsy – tongue deviates towards side of lesion
249
Q

ALS

A
  • Affects corticospinal tracts (UMN) and LMNs - upper and lower motor neuron signs
250
Q

Poliomyelitis

A

Affects anterior horns - LMN signs

251
Q

Brown-Sequard syndrome

A
  • 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
Q

Friedrich’s ataxia

A
  • Same as above
  • Other features of cerebellar disease as well as ataxia - eg intention tremor
253
Q

Anterior cord syndrome

A
  • Eg from anterior spinal artery occlusion, flexion/extension
  • Lateral corticospinal tracts – bilateral spastic paresis
  • Lateral spinothalamic tracts – bilateral loss of pain + temp sensation
254
Q

Central cord syndrome

A
  • 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
Q

Posterior cord syndrome

A
  • 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
256
Q

Neurosyphilis

A

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)

257
Q

Subacute combined generation of spinal cord

A
  • 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
Q

Cerebral venous sinus thrombosis (CVST)

A
  • thrombosis in brain’s venous sinuses - prevents blood draining
  • 50% isolated sagittal sinus
  • also cavernous sinus thrombosis / lateral sinus
259
Q

CVST Px

A
  • sudden headache
  • N+V
  • reduced GCS
260
Q

CVST Ix

A
  • MRI venography, CT alternative
  • D dimer elevated maybe
261
Q

CVST Mx

A
  • anticoagulation - LMWH, then warfarin
262
Q

CVST specific syndromes

A

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
Q

Degenerative cervical myelopathy (DCM)

A
  • stenosis of c-spine leading to spinal cord compression, neurology
  • age-related osteoarthritis
264
Q

DCM Px

A
  • 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
265
Q

DCM Ix

A

MRI c spine

266
Q

DCM Mx

A
  • refer for spinal services assessment
  • decompressive surgery
267
Q

Ataxia telangiectasia

A
  • 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
Q

Friedreich’s ataxia

A
  • 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
Q

Foot drop causes

A
  • 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
Q

Internuclear ophthalmoplegia

A
  • 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
271
Q

Myotonic dystrophy

A
  • 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
Q

Creutzfeldt-Jakob disease (CJD)

A
  • 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
Q

Dystrophinopathies

A
  • x-linked recessive
  • due to mutation in dystrophin gene - dystrophin connects muscle membrane to actin, part of cytoskeleton
274
Q

Duchenne muscular dystrophy (DMD)

A
  • 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
Q

Becker muscular dystrophy

A
  • Non-frameshift insertion – both binding sites preserved – milder form

Px

  • After 10yo
  • Intellectual impairment much less common
276
Q

Cataplexy

A
  • 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
Q

Thoracic outlet syndrome (TOS)

A
  • 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
Q

TOS Px

A

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
Q

TOS Ix

A
  • CXR, c-spine XR
  • CT / MRI
  • venography / angiography
  • anterior scalene block - confirm neurogenic TOS
280
Q

TOS Mx

A
  • Conservative mx - education, rehab, physio, taping
  • Surgical decompression
  • Botox?
281
Q

Multiple system atrophy (MSA)

A
  • Parkinsonism
  • Autonomic disturbance - erectile dysfunction, postural hypotension, atonic bladder
  • Cerebellar signs
282
Q

Cavernous sinus

A
  • 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
Q

Paroxysmal hemicrania

A
  • Attacks of severe, unilateral headache - usually orbital, supraorbital, temporal region
  • Autonomic features also
  • Last 30 mins, multiple times a day
  • Tx with indomethacin
284
Q

Reflexes

A
  • 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
Q

Radiculopathy

A
  • 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
Q

Neuropathy

A

pathological process affecting nerve(s)

287
Q

Mononeuropathy

A
  • single nerve affected
  • eg CTS
288
Q

Mononeuritis multiplex

A

Several individual nerves affected

Systemic causes - WARDS PLC
- Wegener’s granulomatosis
- AIDs/amyloid
- RA
- DM
- Sarcoidosis
- PAN (polyarteritis nodosa)
- Leprosy
- Carcinoma

289
Q

Polyneuropathy

A
  • 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
Q

Spontaneous intracranial hypotension

A
  • 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
291
Q

Von Hippel-Lindau syndrome

A
  • 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 (30 decks)

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