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Neurology Flashcards

1
Q

Unconsciousness / coma

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

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

A

Vegetative state
- widespread cortex damage, brainstem function normal

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

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

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3
Q

Brain-stem death

A

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

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4
Q

Pre-conditions for brain-stem death testing

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

who can perform brain stem death testing?

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

how do you test for brainstem death?

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

stroke definiton

A

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

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8
Q

what is an ischaemic stroke? risk factors?

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

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

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9
Q

what is a haemorrhagic stroke? risk factors?

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

stroke Px - general

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

Oxford Stroke Classification

A

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

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12
Q

Total anterior circulation infarct (TACS)

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

Partial anterior circulation infarct (PACS)

A
  • Smaller arteries of anterior circulation

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

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14
Q

Lacunar syndrome (LACS)

A
  • Arteries around internal capsule, thalamus, basal ganglia

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

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15
Q

Posterior circulation syndrome (POCS)

A
  • Vertebrobasilar arteries

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

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16
Q

Brainstem infarct

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

Lateral medullary syndrome (PICA) - Wallenberg

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

Weber syndrome - branches of PCA that supply midbrain

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

Anterior inferior cerebellar artery (AICA) - lateral pontine syndrome

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

Retinal/ophthalmic artery?

A
  • amaurosis fugax
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21
Q

Stroke by areas: ACA, MCA, PCA

A

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

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

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

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22
Q

Stroke Ix

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

stroke initial Mx?

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

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

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

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24
Q

stroke secondary prevention?

A
  • Clopidogrel 75mg once daily (alternatively aspirin plus dipyridamole)
  • Atorvastatin 20-80mg (not started immediately – usually delayed at least 48 hours)
  • Blood pressure and diabetes control
  • Addressing modifiable risk factors (e.g., smoking, obesity and exercise)
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25
Q

carotid artery assessment post TIA or stroke ?

A

Carotid endarterectomy - if stenosis >70%
- consider angioplasty / stenting

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26
Q

patient found to have AF after TIA/stroke ?

A
  • warfarin / DOAC
  • start immediately in TIA
  • wait 2wks in acute stroke
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27
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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28
Q

TIA?

A
  • transient episode of neurological dysfunction caused by focal brain/spinal cord/retinal ischaemia, without acute infarction
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29
Q

crescendo TIA?

A

2+ TIAs in 1wk - high risk of stroke

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30
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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31
Q

TIA Ix

A
  • CT
  • MRI, inc diffusion-weighted / blood-sensitive
  • Carotid imaging - doppler, CT/MR
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32
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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33
Q

Extradural haemorrhage (EDH)

A
  • blood between skull + dura
  • trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
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34
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
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35
Q

what is Cushing’s reflex?

A

response to acutely raised ICP:

  • bradycardia
  • irregular respirations
  • HTN / increase in pulse pressure (systolic up, diastolic down)
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36
Q

EDH Ix

A
  • bloods, inc G/S
  • non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
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37
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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38
Q

Subdural haemorrhage (SDH)

A
  • blood between dura + arachnoid layers
  • acute / subacute / chronic
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39
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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40
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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41
Q

SDH Ix

A
  • non-contrast CT head - hyperdense crescent (sickle) shaped collection
  • bloods etc
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42
Q

SDH Mx

A
  • A-E
  • neuroprotection
    Neurosurgery
  • Burr hole / open craniotomy, clot evacuation
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43
Q

Subarachnoid haemorrhage (SAH)

A

bleed into subarachnoid space - between arachnoid / pia (where CSF is)

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44
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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45
Q

SAH RFs

A
  • 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan’s, EDH, PKD
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46
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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47
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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48
Q

SAH Mx

A
  • nimodipine
    Neurosurgery
  • endovascular coiling
  • neurosurgical clipping
  • intracranial stents / balloons
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49
Q

SAH Cx

A
  • rebleed - 20%
  • cerebral ischaemia
  • hydrocephalus - VP shunt / EVD
  • hyponatraemia (SIADH) - fluid restriction
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50
Q

Intracerebral haemorrhage

A
  • bleed in brain tissue
  • spontaneous / secondary to ischaemic stroke, tumours, aneurysm rupture

Px
- similar to SAH
- stroke, focal neurology, reduced GCS

Ix
- non-contrast CT head - hyperdensity in substance of brain

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51
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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52
Q

dementia definition?

A
  • irreversible, progressive decline of higher brain function - cognitive/behavioural sx - memory loss, reasoning/communication problems, change in personality, reduction in ADLs
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53
Q

RFx for dementia?

A
  • older, mild cognitive impairment, genetics, Parkinson’s, CVA, smoking, DM, sedentary, obesity
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54
Q

what is 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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55
Q

how does Alzheimer’s present?

A
  • Steady decline
  • Memory loss
  • Loss of executive function / dysphasia
  • Aphasia, apraxia, agnosia
  • Changes in planning, reasoning, speech, orientation
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56
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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57
Q

vascular dementia?

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

Types
- stroke-related - multi/single-infarct
- subcortical - small vessel disease
- mixed dementia - VD + Alzheimer’s

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58
Q

RFx for vascular dementia?

A
  • previous stroke/TIA, older, AF, DM, lipids, smoking
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59
Q

vascular dementia Px?

A
  • Stepwise progression - stability + acute decline
  • Cognitive impairment
  • Mood disturbance - psychosis, delusions, hallucinations, paranoia
  • Gait, speech, emotional, memory disturbance
  • Seizures
  • Focal neurolog
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60
Q

vascular dementia Ix?

A
  • screen for depression, psychomotor retardation
  • formal cognitive screen
  • MRI - infarcts, white matter changes
  • Dx with NINDS-AIREN criteria
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61
Q

vascular dementia Mx?

A
  • cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy
  • address pain, avoid overcrowding, clear communication
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62
Q

what is Lewy body dementia?

A
  • type of dementia (10-15%), >50yo
  • lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
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63
Q

how does Lewy body present?

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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64
Q

how is lewy body Mx?

A
  • AChE - donepezil, rivastigmine
  • avoid neuroleptics (may develop Parkinsonism)
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65
Q

what is 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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66
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 literac

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67
Q

FTD Mx

A
  • maybe SSRIs / trazadone for behavioural sx
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68
Q

general management of dementia?

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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69
Q

Ddx of dementia

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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70
Q

dementia Ix

A
  • cognitive screen
  • bloods:
  • Exclude reversible causes (eg hypothyroid) – FBC, U/E, LFTs, Ca, glucose, ESR/CRP, TFTs, vit B12, folate
  • Maybe urine MC+S, CXR, ECG, syphilis serology, HIV testing

Imaging
- CT / MRI head
- consider SPECT scan, PET scan

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71
Q

what is a cognitive screen?

A

NICE recommended
- 10-CS
- 6CIT
- 6-item screener
- memory impairment screen
- mini-cog
- Test your memory

Not NICE recommended
- AMTS
- GPCOG
- MMSE

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72
Q

causes of falls?

A
  • TLOC
  • mechanical fall
  • geriatric fall
  • visual impairment
  • BPPV
  • drugs, polypharmacy
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73
Q

TLOC causes

A
  • syncope
  • seizure
  • hypoglycaemia
  • head injury
  • narcolepsy
  • stroke
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74
Q

define syncope?

A
  • transient LOC due to cerebral hypoperfusion - low BP after fall in CO / SVR
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75
Q

define orthostatic / postural hypotension

A
  • 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
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76
Q

cardiac syncope?

A
  • structural - MI, valvular heart disease, cardiomyopathy
  • Arrhythmias - tachy/brady
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77
Q

reflex syncope?

A
  • increase in vagal tone
  • vasovagal - pain/emotions
  • carotid sinus hypersensitivity
  • situational syncope - micturition, defecation, cough
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78
Q

RFx for falls?

A

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

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79
Q

medication that can contribute to 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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80
Q

how to investigate falls?

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
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81
Q

managing falls?

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
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82
Q

management of postural hypotension?

A
  • Tx cause, review meds, ensure hydrated
  • Fludrocortisone
  • Midodrine – alpha agonist – for true autonomic dysfunction
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83
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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84
Q

what types of MS are there/

A
  • clinically isolated
  • relapsing remitting
  • secondary progressive
  • primary progressive
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85
Q

clinically isolated syndrome in context of MS?

A
  • First episode of demyelination
  • May never have another episode, or go on to develop MS
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86
Q

relapsing remitting MS?

A
  • 85%
  • Acute attacks (1-2mo), periods of remission
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87
Q

secondary progressive MS?

A
  • Deterioration after relapsing-remitting – neuro signs between relapses
  • 65% with relapsing-remitting go on to develop secondary progressive within 15yrs of dx
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88
Q

primary progressive MS?

A
  • 10% pts
  • Progressive deterioration from onset
  • More common in older pts
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89
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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90
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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91
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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92
Q

management of MS

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/memantin

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93
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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94
Q

types of MND?

A
  • amyotrophic lateral sclerosis (ALS)
  • progressive bulbar palsy (PBP)
  • progressive muscular atrophy (PMA)
  • Primary lateral sclerosis (PLS)
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95
Q

Amyotrophic lateral sclerosis (ALS)

A
  • <80% - most common
  • Loss of motor neurons in motor cortex and anterior horn of cord – UMN + LMN
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96
Q

Progressive bulbar palsy (PBP)

A
  • 10-20%
  • CN9-12 – LMN
  • Affects muscles of talking + swallowing, tongue also
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97
Q

Progressive muscular atrophy (PMA)

A
  • <10%
  • Anterior horn lesion – LMN
  • Affects distal muscle groups before proximal
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98
Q

Primary lateral sclerosis (PLS)

A
  • Loss of bets cells in motor cortex – UMN
  • Marked spastic leg weakness, pseudobulbar palsy, no cognitive decline
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99
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

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100
Q

LMN signs?

A
  • Muscle wasting
  • Reduced tone – flaccid paralysis
  • Fasciculations
  • Reduced reflexes
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101
Q

UMN signs?

A
  • Increased tone – spastic paralysis
  • Brisk reflexes
  • Upgoing plantar reflex
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102
Q

MND Ix?

A
  • clinical dx
  • NCS - normal
  • EMG - reduced no of APs, increased amplitude
  • MRI - exclude myelopathy etc
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103
Q

MND Mx?

A
  • riluzole
  • NIV - resp support
  • baclofen
  • PEG
  • benzos
  • advanced directives
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104
Q

Guillain-Barre syndrome (GBS) - definition

A
  • immune mediated demyelination of PNS
  • triggered by infection - C jejuni
  • ABs against infection attack Schwann cells -> demyelination
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105
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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106
Q

GBS Ix?

A
  • LP - raised protein, normal WCC
  • NCS - decreased velocity
  • spirometry - monitor FVC
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107
Q

GBS Mx?

A
  • FVC <80% - mechanical ventilation
  • IV Ig 1st line
  • plasma exchange
  • VTE prophylaxis
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108
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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109
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
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110
Q

what tests can you do to elicit fatiguability

A

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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111
Q

MG Ix

A
  • ABs - AChR, MuSK, LRP4
  • CK normal
  • CT / MRI thorax - thymus
  • Single fibre electromyography
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112
Q

MG Mx

A
  • AChE inhibitors - pyridostigmine
  • immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate
  • thymectomy
  • rituximab
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113
Q

myasthenia 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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114
Q

what is 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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115
Q

how does Huntington’s present?

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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116
Q

Huntington’s Ix

A
  • genetic testing, genetic counselling
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117
Q

Huntington’s Mx

A
  • MDT, physio, SALT
  • chorea - benzos, tetrabenazine, sulpiride
  • antidepressants
  • advanced directives
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118
Q

Parkinson’s - definition

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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119
Q

key Parkinson’s triad?

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

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120
Q

other potential Parkinson’s sx?

A
  • 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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121
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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122
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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123
Q

Parkinson’s Mx?

A

First line
- if motor sx affecting QoL - levodopa
- if not - dopamine agonist / levodopa / MAO-B

Neuropsych sx
- anti-depressants, anti-psychotics

Anti-muscarinics
- block cholinergic receptors - drug-induced Parkinsonism
- procyclidine

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124
Q

what is levodopa?

A
  • synthetic dopamine, less effective over time
  • add decarboxylase inhibitor - prevent peripheral metabolism
  • co-beneldopa (Madopa)
  • co-careldopa (Sinemet)
  • S/Es - dyskinesia (amantadine to tx), on-off effect, end-of-dose wearing off, postural hypotension, arrhythmias, N+V, psychosis
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125
Q

what is a dopamine agonist?

A
  • bromocriptine, cabergoline, pergolide
  • mimic dopamine action
  • S/Es - pulm/cardiac fibrosis, impulse control disorders
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126
Q

MAO-B inhibitors

A
  • selegiline, rasagiline
  • inhibit MAO-B - break down dopamine, serotonin, adrenaline
  • can use with levodopa to reduce end of dose sx worsening
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127
Q

COMT inhibitors

A
  • entacapone
  • inhibits enzyme that breaks down levodopa
  • take with levodopa to slow its breakdown
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128
Q

Parkinson’s plus syndrome?

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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129
Q

epilepsy definiton?

A

neurological condition characterised by recurrent seizures

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130
Q

definition of a seizure?

A

Disordered electrical activity in the brain leading to clinical manifestationsexcessive, abnormal electrical activity in the brain

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131
Q

causes of seizure

A
  • epilepsy
  • febrile convulsions
  • alcohol withdrawal
  • non-epileptic
  • idiopathic
  • stroke, haemorrhage
  • head injury
  • Alzheimer’s, dementia
  • tumour
  • metabolic disturbance - electrolytes
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132
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
133
Q

types of seizure?

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

134
Q

types of seizure in paeds?

A
  • infantile spasms / West syndrome - tx with ACTH / vigabatrin
  • Lennox-Gastaut syndrome
  • Benign rolandic epilepsy
  • Juvenile myoclonic epilepsy
135
Q

epilepsy presentation

A

Tonic clonic
- Tonic clonic
- LOC
- Tongue biting
- Incontinence
- Groaning

Focal
- Motor – Jacksonian march
- Sensory – déjà vu, jamais vu, strange smells / tastes / sight / sound
Absence
- Stop activity, vacant, 20-30s, carries on

Myoclonic
- Sudden isolated jerk of muscle

Tonic seizure
- Sudden increased tone, intense stiffness, no jerking
- Characteristic cry / grunt

Atonic
- Loss of muscle tone, fall

136
Q

what is an aura in epilepsy?

A
  • Pt aware
  • Strange gut feeling, déjà vu, strange smells, flashing lights
  • Implies focal seizure, but not necessarily in temporal lobe
137
Q

what is post ical period in epilepsy?

A
  • Confused, tired, irritable, low
  • Todd’s paralysis – temporary weakness after seizure
  • May have dysphasia – from focal seizure in temporal lobe
138
Q

how does epilepsy present: temporal 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

139
Q

how does epilepsy present: frontal lobe?

A

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

140
Q

how does epilepsy present: parietal lobe?

A

Parietal lobe (interprets sensations)
- Paraesthesia

141
Q

how does epilepsy present: occipital lobe?

A

Occipital lobe (vision)
- Visual phenomena – spots, lines, flashes

142
Q

DDx for blackouts?

A

Vasovagal syncope.
Cardiac syncope.
Non-epileptic attacks.
Intermittent hydrocephalus (rare).
Migraine (if no loss of awareness).

143
Q

Ix for epilepsy?

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

144
Q

epilepsy general Mx guide

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)
145
Q

how long do you need to be seizure free before driving/

146
Q

Generalised tonic clonic Mx

A

M - sodium valproate
F - lamotrigine / levetiracetam

147
Q

Focal seizure Mx

A

1st - lamotrigine / levetiracetam
2nd - carbamazepine

148
Q

Absence seizure Mx

A

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

149
Q

Myoclonic seizures Mx

A

M - sodium valproate
F - levetiracetam

150
Q

Tonic/ atonic seizures Mx

A

M - sodium valproate
F - lamotrigine

151
Q

epilepsy in pregnancy? KEY POINTS

A
  • folic acid 5mg OD
  • carbamazepine is least teratogenic
  • Breastfeeding is safe for mothers on antiepileptics

Phenytoin = cleft palate, give vit K in last month of pregnancy also
Lamotrigine = congenital malformation rate low
Sodium valproate - significant risk of neurodevelopmental delay

152
Q

contraception and epilepsy?

A

on levetiracetam? can use all contraceptive types

on lamotrigine? UKMEC 3: cocp. UKMEC 1; POP, implant, depo, IUD, IUS

on phenytoin, carbamazepine, barbiturates, primidone, topiramate, oxycarbazepine? UKMEC 3: cocp. UKMEC 2: implant. UKMEC1: depo, IUD, IUS

153
Q

epilepsy complications

A
  • aspiration pneumonia
  • sudden unexpected death in epilepsy (SUDEP)
154
Q

Status epilepticus

A
  • seizure > 5mins
  • or multiple seizures without regaining consciousness in between
155
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
156
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

157
Q

headache types: primary versus secondary

A

Primary
- no underlying cause
- tension, migraine, cluster
Secondary
- underlying cause
- eg GCA, glaucoma, haemorrhage, meds

158
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
159
Q

What is a tension headache and what can cause them?

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

Causes
- eg stress, depression, alcohol, skipping meals, dehydration

160
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
161
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
162
Q

Tension headache management?

A
  • paracetamol, NSAIDs, aspirin
  • limit analgesia to <6d/mo
  • acupuncture
  • physio, exercise, CBT, relaxation
  • trial of amitriptyline
163
Q

what is a 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

164
Q

Name some possible migraine triggers

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

165
Q

Migraine Px ( 5 stages of migraine: prodrome, headache, aura)

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

166
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

167
Q

Hemiplegic migrane

A
  • Unilateral limb weakness
  • Ataxia
  • Impaired consciousness
  • Mimic stroke / TIA
168
Q

Hemiplegic migrane

A
  • Unilateral limb weakness
  • Ataxia
  • Impaired consciousness
  • Mimic stroke / TIA
169
Q

Migraine Mx - acute

A

Acute
- NSAIDs, paracetamol
- triptans - sumatriptan - stimulate serotonin receptors
- antiemetics - prochlorperazine / metoclopramide
- no opiates

170
Q

Migraine Mx - prevention

A

avoid triggers
- propranolol
- topiramate
- amitriptyline
- specialist - candesartan, valproate, MAbs
- menstrual migraines - prophylactic triptans, mefenamic acid, aspirin

171
Q

Migraine Mx - prevention

A

avoid triggers
- propranolol
- topiramate
- amitriptyline
- specialist - candesartan, valproate, MAbs
- menstrual migraines - prophylactic triptans, mefenamic acid, aspirin

172
Q

Migraine Mx - in pregnancy

A

Pregnancy
- paracetamol
- NSAIDs 1st/2nd trim
- no aspirin/opioids

173
Q

Migraine Mx - in pregnancy

A

Pregnancy
- paracetamol
- NSAIDs 1st/2nd trim
- no aspirin/opioids

174
Q

Cluster headache - definition + triggers?

A
  • most disabling type of primary headache
  • potential triggers - alcohol, strong smells, exercise
175
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
176
Q

Cluster headache Ix?

A
  • MRI with gadolinium contrast
177
Q

Cluster headache Mx

A
  • neurology referral

Acute
- 15L O2
- S/C triptan (or intranasal)

Prophylaxis
- verapamil
- occipital nerve block, prednisolone, lithium

178
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
179
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
180
Q

Post-LP headache - what is it and how does it present?

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

181
Q

Post-LP headache - Mx

A
  • supportive - analgesia, rest
  • if pain >72hrs, tx to prevent SDH - blood patch / epidural saline / IV caffeine
182
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
183
Q

Cervical spondylosis

A
  • Caused by degenerative changes in c-spine
  • Neck pain, worse on movt, often also headaches
184
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
185
Q

brain tumour

A
  • primary / secondary (10x more common)
186
Q

brain tumour pathophysiology

A
  • can be benign/malignant, high/low grade

Primary
- adults - majority supratentorial, children infratentorial

Secondary
- NSCLC - most common
- SCLC
- breast
- melanoma
- renal cell
- GI

  • SOL, CSF lost to compensate, eventually no more CSF, ICP rises acutely -> worsening in sx, eventually coning, death
187
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

188
Q

Brain tumours Ix

A
  • CT with contrast
  • MRI brain
  • Biopsy - during surgery
  • LP CI’d (raised ICP)
189
Q

Brain tumours Mx

A
  • surgery
  • chemo / radio
  • dexamethasone
  • levetiracetam
  • palliative care
190
Q

meningitis

A
  • inflammation of meninges
  • viral most common, presume bacterial until otherwise, also fungal, non-infective
191
Q

meningococcal septicaemia

A

bacterial infection spreads to bloodstream - non-blanching rash

192
Q

bacterial causes of meningitis

A

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

193
Q

viral causes of meningitis

A
  • coxsackie, HSV, VZV, CMV, HIV, measles, mumps
194
Q

non infective causes of meningitis

A
  • malignancy, chemicals, meds, sarcoidosis, SLE
195
Q

Meningitis Px

A
  • fever
  • neck stiffness
  • vomiting
  • headache
  • photophobia
  • altered consciousness
  • seizures
  • non-blanching rash
  • Kernig’s
  • Brudzinski’s
196
Q

kernig’s sign?

A

Position the patients supine with their hips flexed to 90°.
This test is positive if there is pain on passive extension of the knee

197
Q

brudzinski’s sign?

A

Position the patients supine and passively flex their neck.
This test is positive if this manoeuvre causes reflex flexion of the hip and knee

198
Q

Meningitis LP interpretation: bacterial

A
  • cloudy
  • high protein
  • low glucose
  • high WCC (neutrophils)
  • culture growth
199
Q

Meningitis LP interpretation: viral

A
  • clear
  • mildly high protein
  • normal glucose
  • high WCC (lymphocytes)
  • negative culture
200
Q

Meningitis LP interpretation: TB

A
  • slightly cloudy
  • low glucose
  • high protein
  • PCR to dx
201
Q

meningitis Ix?

A
  • LP
  • bloods - cultures, meningococcal PCR, FBC, U/E, glucose, lactate, coag, VBG
  • maybe throat swab
  • CT not normally indicated
202
Q

meningitis Mx in community

A
  • IM benzylpenicillin
203
Q

meningitis Mx?

A
  • <3mo – IV cefotaxime + amoxicillin
  • 3mo-50yo – IV cefotaxime / ceftriaxone
  • > 50yo – cefotaxime / ceftriaxone + amoxicillin

NB
Vancomycin added if risk of penicillin resistant pneumococcal infection e.g. recent foreign travel or prolonged antibiotic exposure

Steroids used in bacterial meningitis to reduce the frequency and severity of hearing loss and neurological damage - dexamethasone QDS for 4 days if >3m

204
Q

Mx of meningitis contacts?

A

Mx of contacts
- close contact <7d sx onset
- oral ciprofloxacin single dose, rifampicin 2nd line
- offer meningococcal vaccination/booster

205
Q

complications of meningitis?

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
206
Q

encephalitis?

A
  • infection / inflammation of brain parenchyma
  • mostly viral
  • typically temporal / inferior frontal lobes
207
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

208
Q

Encephalitis Px

A
  • Fever
  • Headache
  • Psych sx, unusual behaviour
  • Altered cognition, consciousness
  • Seizures, often focal
  • Vomiting
  • Focal neurology, eg aphasia
209
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
210
Q

Encephalitis Mx

A
  • IV acyclovir
211
Q

facial nerve palsy ?

A
  • dysfunction of facial nerve -> unilateral facial weakness
  • each side of forehead has UMN innervation from both sides of brain
212
Q

facial nerve palsy: UMN lesion

A
  • forehead spared
  • strokes, tumours
  • bilateral - MND, pseudobulbar palsies
213
Q

facial nerve palsy: LMN lesion

A
  • forehead not spared (paralysed)
  • Bell’s, Ramsay Hunt, OM, OE, HIV….
214
Q

Bell’s palsy?

A
  • acute, unilateral idiopathic facial nerve paralysis
  • cause unknown, maybe HSV involvement
215
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
216
Q

Bell’s palsy dx

A
  • dx made when no other cause for paralysis found
217
Q

Bell’s palsy Mx

A
  • oral prednisolone within 72hrs
  • eye care - drops, tape closed at night
  • refer if atypical sx / worsening / no improvement
218
Q

Ramsay Hunt syndrome: Px and Mx

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

219
Q

brain abscess?

A
  • pus-filled swelling in brain

Causes
- from middle ear / sinuses
- trauma
- surgery to scalp
- penetrating head injuries
- emboli from IE

220
Q

brain abscess px?

A
  • headache - dull, persistent
  • fever
  • focal neurology - site dependent
  • raised ICP - nausea, papilloedema, seizures
221
Q

brain abscess ix ?

222
Q

brain abscess Mx

A
  • surgery - craniotomy, debride
  • IV abx - ceftriaxone + metronidazole
  • dexamethasone
223
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

224
Q

DDx of peripheral neuropathy

A
  • alcohol
  • B12 deficiency
  • cancer (myeloma), CKD
  • diabetes, drugs (amiodarone, isoniazid, nitrofurantoin, metronidazole)
  • vasculitis
225
Q

essential tremor

A
  • autosomal dominant condition - tremor usually affecting upper limbs/hands
226
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
227
Q

essential tremor Mx

A
  • propranalol
  • primidone
228
Q

Idiopathic intracranial HTN (IIH)

A
  • build up of pressure around brain
    RFs
  • female, obese, pregnancy, drugs
229
Q

IIH Px

A
  • headache, worse in morning/coughing/straining
  • nausea
  • blurred vision
  • papilloedema
  • enlarged blind spot
  • sixth nerve palsy
230
Q

IIH Ix

A
  • assess neurology, eyes, vision
  • CT / MRI
  • LP
231
Q

IIH Mx

A
  • wt loss
  • acetazolamide / topiramate - act as diuretics
  • repeated LP
  • surgery - eg shunt / optic nerve sheath decompression
232
Q

narcolepsy

A
  • long term brain condition causing pt to fall asleep suddenly at inappropriate times
  • low levels orexin (hypocretin)
233
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
234
Q

narcolepsy Ix

A
  • multiple sleep latency EEG
  • CSF orexin levels - low
235
Q

narcolepsy Mx

A
  • Daytime stimulants - modafinil
  • Night time - sodium oxybate
236
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
237
Q

Trigeminal neuralgia Px

A
  • intense facial pain, seconds-hrs
  • electricity-like
  • attacks worsen over time
238
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
239
Q

Trigeminal neuralgia Mx

A
  • carbamazepine
  • neurology referral if atypical features, eg <50yo
  • surgery to decompress/intentionally damage nerve
240
Q

Neurofibromatosis

A
  • genetic condition that causes nerve tumours (neuromas) to develop throughout nervous system
  • tumours benign, but cause neuro/structural problems
  • NF1>NF2
241
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

242
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

243
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

244
Q

hydrocephalus Px

A
  • raised ICP sx
  • headache - worse in morning, lying down, Valsalva
  • N+V
  • papilloedema
  • coma
  • infants increased head circumference
245
Q

hydrocephalus Ix and Mx

A

Ix
- CT head
- MRI
- LP - drain CSF, measure opening pressure (do not use in obstructive causes)

Mx
- external ventricular drain (EVD)
- ventriculoperitoneal shunt (VP shunt)
- surgical tx for obstructive pathology

246
Q

Normal pressure hydrocephalus

A
  • reversible cause of dementia in elderly
  • reduced CSF absorption at arachnoid villi / head injury / SAH / meningitis
247
Q

Normal pressure hydrocephalus Px

A
  • urinary incontinence
  • dementia, bradyphrenia (slowed thinking)
  • gait abnormality
  • sx develop over a few months
248
Q

Normal pressure hydrocephalus Ix

A
  • CT head - hydrocephalus, ventriculomegaly, no sulcal enlargement
249
Q

Normal pressure hydrocephalus Mx

250
Q

wernicke’s aphasia?

A
  • 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
251
Q

Broca’s aphasia?

A
  • expressive
  • Lesion of inferior frontal gyrus, superior division of left MCA
  • Speech non-fluent, laboured, halting, repetition impaired
  • Comprehension normal
252
Q

conduction aphasia

A
  • From stroke affecting arcuate fasciculus – connection between Wernicke’s and Broca’s
  • Speech fluent, repetition poor
  • Comprehension norma
253
Q

restless legs syndrome: definition and causes?

A
  • spontaneous, continuous lower limb movts, associated with paraesthesia
    Causes
  • FHx, IDA, uraemia, DM, pregnancy
254
Q

restless legs 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
255
Q

restless legs Ix

A
  • Clinical dx
  • Ferritin - r/o iron deficiency anaemia
256
Q

restless legs Mx

A
  • Walking, stretching, massaging
  • Iron
  • Dopamine agonist 1st line - pramipexole, ropinirole
  • Benzos
  • Gabapentin
257
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

258
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
259
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)
260
Q

Lambert-Eaton myasthenic syndrome Ix

A
  • EMG - incremental response to repetitive electrical stimulation
261
Q

Lambert-Eaton myasthenic syndrome Mx

A
  • tx cancer
  • immunosuppression - prednisolone / azathioprine
  • amifampridine / 3,4-diaminopyridine
  • pyridostigmine
  • IVIg, plasma exchange
262
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

263
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 - pain specialist
264
Q

what is a vestibular schwannoma?

A
  • acoustic neuroma
  • 90% cerebellopontine angle tumours
  • Bl in NF2
265
Q

how might vestibular schwannoma present?

A
  • CN8 – vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
  • CN5 – absent corneal reflex
  • CN7 – facial palsy
266
Q

how would you investigate and manage vestibular schwannoma

A

Ix
- Urgent ENT referral
- MRI of cerebellopontine angle
- Audiometry

Mx
- Surgery
- Radiotherapy
- Watch and wait

267
Q

what is pituitary apoplexy?

A
  • sudden enlargement of pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage/infarction
  • eg HTN, pregnancy, trauma, anticoags

Ix - MRI

Mx
- steroid replacement (loss of ACTH)
- monitor fluid balance
- surgery

268
Q

Syringomyelia

A

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

Causes
- chiari malformation
- trauma
- tumours
- idiopathic

269
Q

syringomyelia Ix and Mx

A
  • full spine MRI with contrast
  • brain MRI (exclude chiari)
  • tx cause
  • shunt if persistent
270
Q

what is 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

271
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
272
Q

homonymous hemianopia

A
  • Incongruous – optic tract lesion
  • Congruous – optic radiation / occipital cortex
  • Macula sparing – occipital cortex
273
Q

Homonymous quadrantanopias

A
  • Superior – lesion of inferior optic radiations, temporal lobe, Meyer’s loop
  • Inferior – superior optic radiations, parietal lobe, Baum’s
274
Q

bitemporal hemianopia

A
  • Lesion of optic chiasm
  • Upper quadrant > lower – inferior chiasmal compression – pituitary tumour
  • Lower > upper – superior compression - craniopharyngioma
275
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

277
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

278
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

279
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

280
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

281
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

282
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

283
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

284
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)

285
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

286
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)

287
Q

Obturator nerve palsy (L2-3)

A

Causes
- Pelvic ring fracture, obturator hernia
Motor
- Paralysis of hip adductors
Sensory
- Medial thigh

288
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

289
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

290
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

291
Q

Olfactory (I)

A

Palsy – change in smell

292
Q

Optic (II)

A

Palsy – change in sight

293
Q

Oculomotor (III)

A
  • All eye muscles except LR and SO. Pupil constriction, accommodation, eyelid opening
  • Palsy – ptosis, down + out eye, fixed dilated pupi
294
Q

Trochlear (IV)

A
  • Superior oblique
  • Palsy – defective downward gaze – vertical diplopia – noticed when looking down – head tilt to compensate
295
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
296
Q

Abducens (VI)

A
  • Lateral rectus
  • Palsy – defective abduction – horizontal diplopia
297
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
298
Q

Vestibulocochlear (VIII)

A
  • Hearing, balance
  • Palsy – hearing loss, vertigo, nystagmus. Acoustic neuromas – Schwann cell tumours of cochlear nerve
299
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)
300
Q

Vagus (X)

A
  • Phonation, swallowing, innervates viscera
  • Palsy – uvula deviates away from site of lesion, loss of gag reflex (efferent)
301
Q

Accessory (XI)

A
  • Head + shoulder movt
  • Palsy – weakness turning head to contralateral side, weakness shrugging shoulder
302
Q

Hypoglossal (XII)

A
  • Tongue movt
  • Palsy – tongue deviates towards side of lesion
303
Q

poliomyelitis

A

Affects anterior horns - LMN signs

304
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)

305
Q

friedrich’s ataxia

A
  • Other features of cerebellar disease as well as ataxia - eg intention tremor
306
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
307
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
308
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
309
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
310
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
311
Q

CVST Px

A
  • sudden headache
  • N+V
  • reduced GCS
312
Q

CVST Ix and Mx

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

Mx - anticoagulation - LMWH, then warfarin

313
Q

Degenerative cervical myelopathy (DCM)

A
  • stenosis of c-spine leading to spinal cord compression, neurology
  • age-related osteoarthritis
314
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
315
Q

DCM Ix and Mx

A

MRI c spine
- refer for spinal services assessment
- decompressive surgery

316
Q

causes of foot drop?

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
317
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

318
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
319
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
320
Q

Becker muscular dystrophy

A
  • Non-frameshift insertion – both binding sites preserved – milder form
    Px
  • After 10yo
  • Intellectual impairment much less common
321
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
322
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)
323
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
324
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
325
Q

neuropathy?

A

pathological process affecting nerve(s)

326
Q

mononeuropathy

A
  • single nerve affected
  • eg CTS
327
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

328
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

finals (19 decks)

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