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
2
Q
Brain-stem death
A
Neurological damage to brainstem resulting in irreversible loss of consciousness and loss of spontaneous ventilation
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
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
5
Q
Stroke
A
- CVA - sudden interruption in vascular supply of brain -> irreversible brain death
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
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
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
9
Q
Total anterior circulation infarct (TACS)
A
- ACA and MCA
- All 3 criteria above present
10
Q
Partial anterior circulation infarct (PACS)
A
- Smaller arteries of anterior circulation
- 2 of above criteria present
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
12
Q
Posterior circulation syndrome (POCS)
A
- Vertebrobasilar arteries
1 of following
- Cerebellar / brainstem syndromes
- Loss of consciousness
- Isolated homonymous hemianopia
13
Q
Brainstem infarct
A
- quadriplegia
- locked-in syndrome (basilar artery)
14
Q
Lateral medullary syndrome (PICA) - Wallenberg
A
- Ipsilateral - ataxia, nystagmus, dysphagia, facial numbness, CN palsy (Horner’s)
- Contralateral - limb sensory loss
15
Q
Weber syndrome - branches of PCA that supply midbrain
A
- Ipsilateral CN3 palsy
- Contralateral weakness of upper + lower limbs
16
Q
Anterior inferior cerebellar artery (AICA) - lateral pontine syndrome
A
- Similar to Wallenberg’s (above), but
- Ipsilateral - facial paralysis and deafness
17
Q
Retinal/ophthalmic artery
A
- amaurosis fugax
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
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
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
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
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
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
24
Q
TIA Ix
A
- CT
- MRI, inc diffusion-weighted / blood-sensitive
- Carotid imaging - doppler, CT/MRI
25
TIA Mx
- aspirin 300mg
- don't drive until seen by specialist
- clopidogrel / aspirin + dipyridamole
- carotid artery endarterectomy if stenosis >70%
- atorvastatin 80mg
Specialist review
- If >1 TIA / suspected cardioembolic source / severe carotid stenosis - ?admission / observation with stroke specialist
- Suspected TIA in last 7d – specialist review in 24hrs
- Suspected TIA >7d ago – specialist review asap within 7d
26
Extradural haemorrhage (EDH)
- blood between skull + dura
- trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
27
TBI patho
Primary brain injury
- at time of trauma - compression, tension, shearing of brain tissue, cell membrane disruption, vascular disruption, inflammation
Secondary brain injury
- hypoxia, hypotension, raised ICP -> further injury
Monro-Kellie doctrine
- brain, blood, CSF in cranium
- intial LOC, then CSF lost to compensate for increased bleed, then no more left, brain compressed - further LOC
- herniation - fixed dilated pupil due to compression of psym fibres of oculomotor nerve
28
EDH Px
- head trauma
- initial LOC / drowsiness
- lucid interval, later low GCS
- headache, N+V, confusion, seizures, focal neurology, fixed dilated pupil, deep coma
Cushing's reflex
- bradycardia
- irregular respirations
- HTN / increase in pulse pressure (systolic up, diastolic down)
29
EDH Ix
- bloods, inc G/S
- non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
30
EDH Mx
- A-E
Neuroprotection
- 30deg tilt head up
- oxygenation, loosen collars
- intubate + ventilate
- normostasis - normotension, normoglycaemia
- hyperventilate if coning
- IV mannitol
- hypertonic saline (3%)
- levetiracetam - seizures
Neurosurgery
- ICP bolt / EVD
- burr hole / open craniotomy
31
Subdural haemorrhage (SDH)
- blood between dura + arachnoid layers
- acute / subacute / chronic
32
SDH patho
- rupture of bridging veins between cortex + sinus
- infants (NAI), elderly/alcoholics (cerebral atrophy)
- bleed forms haematoma - when this breaks down, proteins become soluble - haematoma expands, ICP rises
33
SDH Px
- Head injury
- Lucid interval - can be days/wks/months
- Personality change, sleepiness, unsteadiness
- Fluctuation in consciousness, memory loss, cognitive impairment
- Focal neuro deficit
- Seizure, headache, coma, vomiting
- Fixed dilated pupil
34
SDH Ix
- non-contrast CT head - hyperdense crescent (sickle) shaped collection
- bloods etc
35
SDH Mx
- A-E
- neuroprotection
Neurosurgery
- Burr hole / open craniotomy, clot evacuation
36
Subarachnoid haemorrhage (SAH)
- bleed into subarachnoid space - between arachnoid / pia (where CSF is)
37
SAH causes
Traumatic
- most common cause of SAH
Spont
- Berry aneurysm - 85% spont cases - HTN, PKD, EDS, coarc aorta
- AV malformation
- pituitary apoplexy
- mycotic aneurysms
38
SAH RFs
- 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan's, EDH, PKD
39
SAH Px
- sentinel headache - less severe in wks before px
- sudden onset occipital headache - thunderclap
- N+V
- photophobia, neck stiffness, Kernig's, Brudzinski's
- coma
- seizures
- papilloedema, fixed dilated pupil, Cushing's
- maybe ST elevation
40
SAH Ix
Non-contrast CT head
- bright star-shaped lesions
- if CT head <6hrs of sx onset + normal - LP not indicated
- CT less reliable >6hrs - so if normal - consider LP
LP
- >12hrs after sx onset
- xanthrochromia
- raised opening pressure
- raised red cell count
CT intracranial angiogram +/- catheter angiogram
41
SAH Mx
- nimodipine
Neurosurgery
- endovascular coiling
- neurosurgical clipping
- intracranial stents / balloons
42
SAH Cx
- rebleed - 20%
- cerebral ischaemia
- hydrocephalus - VP shunt / EVD
- hyponatraemia (SIADH) - fluid restriction
43
Intracerebral haemorrhage
- bleed in brain tissue
- spontaneous / secondary to ischaemic stroke, tumours, aneurysm rupture
Px
- similar as above
- stroke, focal neurology, reduced GCS
Ix
- non-contrast CT head - hyperdensity in substance of brain
44
Head injury NICE criteria for CT head, >16yo
CT head <1hr if any of:
- GCS 12 or lower on initial assessment
- GCS <15 2hrs after injury
- ?open / depressed skull fracture
- Signs of basal skull fracture – haemotympanum, panda eyes, CSF oto/rhinorrhoea, Battle’s sign
- Seizure
- Focal neurology
- 1+ episodes vomiting
If none of the above, do CT head <8hrs of injury if there is LOC / amnesia, and any of:
- >65yo
- Bleeding / clotting disorders
- Dangerous MOI
- >30 mins retrograde amnesia of events just before injury
If no RFs of any category above, CT head <8hrs of injury if anticoagulated:
- Warfarin, DOACs, heparin, clopidogrel
45
Dementia
- irreversible, progressive decline of higher brain function - cognitive/behavioural sx - memory loss, reasoning/communication problems, change in personality, reduction in ADLs
RFs
- older, mild cognitive impairment, genetics, Parkinson's, CVA, smoking, DM, sedentary, obesity
46
Alzheimer's dementia
- type of dementia (50-75%)
- amyloid plaques / tau proteins in brain - reduce transmission, brain cell death
- widespread cerebral atrophy, esp cortex / hippocampus
47
Alzheimer's Px
- Steady decline
- Memory loss
- Loss of executive function / dysphasia
- Aphasia, apraxia, agnosia
- Changes in planning, reasoning, speech, orientation
48
Alzheimer's Mx
- wellbeing activities, cognitive stimulation therapy, group reminiscence therapy, cognitive rehab
- 1st - AChE inhibitors - donepezil, galantamine, rivastigmine
- 2nd - NMDA receptor antagonist - memantine
- antipsychotics - if risk of harming themselves
49
Vascular dementia (VD)
- type of dementia (<20%)
- Group of syndromes of cognitive impairment caused by ischaemia / haemorrhage secondary to cerebrovascular disease
- affects white matter of cerebral hemispheres, grey nuclei, thalamus, striatum
RFs
- previous stroke/TIA, older, AF, DM, lipids, smoking,....
Types
- stroke-related - multi/single-infarct
- subcortical - small vessel disease
- mixed dementia - VD + Alzheimer's
50
VD Px
- Stepwise progression - stability + acute decline
- Cognitive impairment
- Mood disturbance - psychosis, delusions, hallucinations, paranoia
- Gait, speech, emotional, memory disturbance
- Seizures
- Focal neurology
51
VD Ix
- screen for depression, psychomotor retardation
- formal cognitive screen
- MRI - infarcts, white matter changes
- Dx with NINDS-AIREN criteria
52
VD Mx
- cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy
- address pain, avoid overcrowding, clear communication
53
Lewy body dementia
- type of dementia (10-15%), >50yo
- lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
54
Lewy body Px
- progressive cognitive impairment - may fluctuate, early impairments to attention, executive function
- sleep disorder
- visual hallucinations
- Parkinson's like sx - bradykinesia, resting tremor, rigidity
55
Lewy body Mx
- AChE - donepezil, rivastigmine
- avoid neuroleptics (may develop Parkinsonism)
56
Frontotemporal dementia (FTD)
- type of dementia (2%), <65yo
- neuron damage/death at frontal/temporal lobes, atrophy due to tau protein deposition
57
FTD Px
Behavioural
- Change in behaviour / personality, eg disinhibited, impulsive, decline in social skills..
Semantic
- Lack of understanding word meanings, difficulty in name retrieval…
Non-fluent
- Breakdown in speech, apraxia, decline in literacy
58
FTD Mx
- maybe SSRIs / trazadone for behavioural sx
59
Dementia general Px
Cognitive impairment
- Memory loss, difficulty with reasoning / communication / decisions, dysphasia, coordinating movements, not orientated
Behavioural and psychological sx of dementia (BPSD)
- Psychosis – delusions / hallucinations, agitation / emotional lability, depression / anxiety, motor disturbance, sleep disturbance
Difficulties with ADLs
- Household tasks, toileting eating, walking
60
Dementia DDx
- CJD
- HIV-related cognitive impairment / dementia
- depression
- normal pressure hydrocephalus
- mild cognitive impairment
- delirium
- reversible causes - hypothyroid, Addison's, B12, folate, thiamine, syphilis, tumour, SDH, drugs,
61
Dementia Ix
Cognitive screen
NICE recommended
- 10-CS
- 6CIT
- 6-item screener
- memory impairment screen
- mini-cog
- Test your memory
Not NICE recommended
- AMTS
- GPCOG
- MMSE
Bloods
- Exclude reversible causes (eg hypothyroid) – FBC, U/E, LFTs, Ca, glucose, ESR/CRP, TFTs, vit B12, folate
- Maybe urine MC+S, CXR, ECG, syphilis serology, HIV testing
Imaging
- CT / MRI head
- consider SPECT scan, PET scan
62
Falls causes
- TLOC
- mechanical fall
- geriatric fall
- visual impairment
- BPPV
- drugs, polypharmacy
63
TLOC causes
- syncope
- seizure
- hypoglycaemia
- head injury
- narcolepsy
- stroke
64
Syncope
- transient LOC due to cerebral hypoperfusion - low BP after fall in CO / SVR
Reflex
- increase in vagal tone
- vasovagal - pain/emotions
- carotid sinus hypersensitivity
- situational syncope - micturition, defecation, cough
Orthostatic/postural hypotension
- fall in BP >20/10 on standing - blood pools in legs with gravity, reduced venous return, reduced preload/EDV/SV/CO (normally compensated for by sympathetic increase in HR/SVR
- Primary autonomic failure - older, Parkinson's, Lewy Body, MSA
- secondary - DM, adrenal insufficiency, hypothyroid
- drugs - anti-HTN, diuretics, alcohol
- hypovolaemia - D+V, diuresis, haemorrhage
Cardiac
- structural - MI, valvular heart disease, cardiomyopathy
- Arrhythmias - tachy/brady
65
Falls RFs
Leg weakness, vision problems, balance / gait disturbance, polypharmacy, incontinence, >65yo, depression, arthritis, psychoactive drugs, cognitive impairment
66
Meds and falls
Meds that cause postural hypotension
- Nitrates, diuretics, anticholinergics, antidepressants, BBs, levodopa, ACEi
Meds that cause falls by other mechanisms
- Benzos, antipsychotics, opiates, anti-epileptics, codeine, digoxin, sedatives
67
Falls Ix
- Hx - before, during, after, any LOC, injuries, anticoags, collateral hx, seizure sx, what were they doing at the time, ?self-mobilised, DHx, SHx...
- A-E
- Full cardio, resp, GI, neuro exams
- Check c-spine, pelvis, hips (?SLR),
- Obs, BMs, urine dip, ECG
- FBC, U/E, LFTs, bone profile, Mg, CRP, CK if long lie, coag, haematinics, vit D, TFTs
- XR of injured limbs
- CT head
- ECHO
- Lying standing BP
- Turn 180 tests / timed up and go test
- Tilt table test - measure HR / BP as table with pt on tilts - for postural hypotension
- Maybe short synacthen test for adrenal insufficiency
68
Falls Mx
- tx cause
- adjust meds
- IV fluids
- Cardiology referral if needed
- Maybe neuro obs
- Vasovagal / situational - reassure
- Inpatient – 1-1 nursing, non-slip socks, low rise beds / mattresses on floor
- Walking aids
- OT/PT review
- Package of care if going home
Postural hypotension
- Tx cause, review meds, ensure hydrated
- Fludrocortisone
- Midodrine – alpha agonist – for true autonomic dysfunction
- Falls clinic follow up
69
Multiple sclerosis (MS)
- chronic condition of demyelination in the CNS
- plaques of demyelination in brain + spinal cord
- autoimmune inflammatory response
- new myelin is less efficient
- genetics, EBV, low vit D, smoking, obesity
70
MS types
Clinically isolated syndrome
- First episode of demyelination
- May never have another episode, or go on to develop MS
Relapsing-remitting
- 85%
- Acute attacks (1-2mo), periods of remission
Secondary progressive
- Deterioration after relapsing-remitting – neuro signs between relapses
- 65% with relapsing-remitting go on to develop secondary progressive within 15yrs of dx
- See gait / bladder disorders
Primary progressive
- 10% pts
- Progressive deterioration from onset
- More common in older pts
71
MS Px
- sx can progress over >24hrs, last days-wks, then improve
Visual
- optic neuritis - unilateral reduced vision, central scotoma, pain with eye movt, impaired colour vision, RAPD
- optic atrophy
- Uhthoff's - vision worse with warm body temp
- internuclear ophthalmoplegia - impaired adduction ipsilaterally, nystagmus contralaterally, conjugate lateral gaze disorder
Sensory
- paraesthesia, numbness, trigeminal neuralgia, Lhermitte's syndrome
Motor
- spastic weakness - legs
Cerebellar
- ataxia - sensory / cerebellar
- tremor
Others
- urinary incontinence
- sexual dysfunction
- intellectual dysfunction
- transverse myelitis
72
MS Dx
- 2+ relapses of different parts of CNS - disseminated in space and time
- Or objective clinical evidence of one lesion + reasonable hx evidence of previous relapse
73
MS Ix
- exclude ddx - FBC, CRP/ESR, U/E, LFTs, glucose, HIV, auto-ABs, Ca, vit B12
- MRI - high-signal T2 lesions, periventricular plaques, Dawson fingers
- LP - oligoclonal bands
- electrophysiology - visual evoked potential studies delayed
74
MS Mx
Acute relapses
- high dose oral / IV steroids - methyl/prednisolone
Disease-modifying therapies
- natalizumab, ocrelizumab, fingolimod...
Symptomatic tx
- fatigue - amantadine, mindfulness, CBT
- spasticity - baclofen/gabapentin, diazepam, physio
- bladder dysfunction - USS, ISC, anticholinergics (solifenacin)
- oscillopsia - gabapentin/memantine
75
MND
- progressive destruction of motor neurons
- cause unknown, 5% have SOD1 mutation
- possibly mitochondrial dysfunction - oxidative stress, ROS damages stuff
- Frontotemporal dementia in 25%
76
MND types
Amyotrophic lateral sclerosis (ALS)
- <80% - most common
- Loss of motor neurons in motor cortex and anterior horn of cord – UMN + LMN
Progressive bulbar palsy (PBP)
- 10-20%
- CN9-12 – LMN
- Affects muscles of talking + swallowing, tongue also
Progressive muscular atrophy (PMA)
- <10%
- Anterior horn lesion – LMN
- Affects distal muscle groups before proximal
Primary lateral sclerosis (PLS)
- Loss of bets cells in motor cortex – UMN
- Marked spastic leg weakness, pseudobulbar palsy, no cognitive decline
77
MND Px
- Insidious, progressive weakness – often first in upper limbs
- Fatigue when exercising
- Clumsiness, dropping things
- Dysarthria – slurred speech
- dysphagia, fluid regurg, choking
LMN signs
- Muscle wasting
- Reduced tone – flaccid paralysis
- Fasciculations
- Reduced reflexes
UMN signs
- Increased tone – spastic paralysis
- Brisk reflexes
- Upgoing plantar reflex
more in notes on px by location
78
MND Ix
- clinical dx
- NCS - normal
- EMG - reduced no of APs, increased amplitude
- MRI - exclude myelopathy etc
79
MND Mx
- riluzole
- NIV - resp support
- baclofen
- PEG
- benzos
- advanced directives
80
Guillain-Barre syndrome (GBS)
- immune mediated demyelination of PNS
- triggered by infection - C jejuni
- ABs against infection attack Schwann cells -> demyelination
81
GBS Px
- hx of GE
- sx peak in 2-4wks, recovery years
- back/leg pain
- progressive, symmetrical weakness of limbs - ascending, reflexes reduced, mild paraesthesia, neuropathic pain, hypotonia
- CNs - diplopia, bl facial nerve palsy, oropharyngeal weakness
- resp muscle weakness
- autonomic - diarrhoea, urinary retention
82
GBS Ix
- LP - raised protein, normal WCC
- NCS - decreased velocity
- spirometry - monitor FVC
83
GBS Mx
- FVC <80% - mechanical ventilation
- IV Ig 1st line
- plasma exchange
- VTE prophylaxis
84
Myasthenia gravis (MG)
- autoimmune disorder with ABs against AChR in NMJ
- ABs block receptors, prevent ACh stimulation,
- thymoma link
- autoimmune link - thyroid, pernicious anaemia, RA, SLE, thymic hyperplasia
85
MG Px
- muscle weakness - worse with activity, improves with rest
- extraocular - diplopia
- proximal muscle weakness - face, neck, limb girdle
- ptosis
- dysphagia
- slurred speech, jaw fatigue
Tests to elicit fatigability
- Count to 50, voice quietens
- Prolonged upward gazing exacerbates diplopia
- Repeated blinking -> ptosis
- Repeated abduction of one arm 20x -> unilateral weakness on that side
86
MG Ix
- ABs - AChR, MuSK, LRP4
- CK normal
- CT / MRI thorax - thymus
- Single fibre electromyography
87
MG Mx
- AChE inhibitors - pyridostigmine
- immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate
- thymectomy
- rituximab
88
Myasthenic crisis
- life-threatening cx of MG
- acute worsening of sx - often triggered by illness - eg resp infection
- monitor FVC - resp failure - NIV / mechanical ventilation
Mx
- IV Ig
- plasmapheresis
89
Huntington's
- Inherited neurodegenerative disorder which causes chorea - jerky, semi-purposeful movements
- autosomal dominant
- CAG repeats, may see anticipation
- lack of GABA + ACh, decreased inhibition of dopamine release -> excessive movts
90
Huntington's Px
- Sx develop >35yo
- Starts with mild psychotic / behavioural sx - cognitive, psych, mood problems
- Chorea - random, irregular, abnormal body movts
- Personality changes - irritable, apathy, depression, intellectual impairment
- Dystonia
- Rigidity
- Speech / swallowing difficulties
- Saccadic eye movts
91
Huntington's Ix
- genetic testing, genetic counselling
92
Huntington's Mx
- MDT, physio, SALT
- chorea - benzos, tetrabenazine, sulpiride
- antidepressants
- advanced directives
93
Parkinson's
- Progressive degenerative movt disorder caused by degeneration of dopaminergic neurons in substantia nigra (in basal ganglia)
- dementia, depression associations
- from mitochondrial dysfunction / oxidative stress
94
Parkinson's Px
Tremor
- worse at rest, 4-6Hz
- worse stressed/tired
- improves on voluntary movt
- pill-rolling
Bradykinesia
- hypokinesia/poverty of movt
- short, shuffling steps, reduced arm swinging
- difficulty initiating movt
- micrographia / monotonous hypophonic speech
Rigidity
- increased tone in limbs/trunk
- lead pipe - throughout ROM
- cogwheel - superimposed tremor
Other sx
- hypomimia - reduced facial movts
- flexed posture
- saliva drooling
- psych - depression, dementia, psychosis, sleep disturbance, cognitive impairment, memory problems
- anosmia
- REM sleep behaviour disorder
- fatigue
- postural hypotension
95
Parkinson's DDx
Causes of Parkinsonism
- Parkinson's disease, drug induced (antipsychotics, metoclopramide, domperidone), PSP, MSA, Wilson's, CO poisoning
Drug-induced parkinsonism
- rapid onset motor sx, bilateral
- rigidity/resting tremor uncommon
Benign essential tremor
- symmetrical, 6-12Hz, improves at rest, worse with voluntary movts, improves with alcohol
96
Parkinson's Ix
- clinical dx
- CT / MRI head
- Use UK Parkinson's Disease Society Brain Bank Clinical Diagnostic Criteria
- SPECT scan
97
Parkinson's Mx
First line
- if motor sx affecting QoL - levodopa
- if not - dopamine agonist / levodopa / MAO-B
Levodopa
- synthetic dopamine, less effective over time
- add decarboxylase inhibitor - prevent peripheral metabolism
- co-beneldopa (Madopa)
- co-careldopa (Sinemet)
- S/Es - dyskinesia (amantadine to tx), on-off effect, end-of-dose wearing off, postural hypotension, arrhythmias, N+V, psychosis....
Dopamine agonist
- bromocriptine, cabergoline, pergolide
- mimic dopamine action
- S/Es - pulm/cardiac fibrosis, impulse control disorders
MAO-B inhibitors
- selegiline, rasagiline
- inhibit MAO-B - break down dopamine, serotonin, adrenaline
- can use with levodopa to reduce end of dose sx worsening
COMT inhibitors
- entacapone
- inhibits enzyme that breaks down levodopa
- take with levodopa to slow its breakdown
Neuropsych sx
- anti-depressants, anti-psychotics
Anti-muscarinics
- block cholinergic receptors - drug-induced Parkinsonism
- procyclidine
98
Parkinson-plus syndromes
- group of neurodegenerative diseases with classical sx of Parkinson's + additional features
Multiple system atrophy (MSA)
- neurons of brain degenerate, inc basal ganglia
- Parkinsonism, autonomic dysfunction, cerebellar dysfunction
Progressive supranuclear palsy (PSP)
- postural instability, falls, impairment of vertical gaze
- parkinsonism, cognitive impairment
Corticobasal degeneration (CBD)
Dementia with Lewy bodies (DLB)
Frontotemporal dementia (Pick's disease)
99
Epilepsy
neurological condition characterised by recurrent seizures
Associated conditions
- cerebral palsy
- tuberous sclerosis
- mitochondrial diseases
100
Seizure
excessive, abnormal electrical activity in the brain
101
Causes of seizures
- epilepsy
- febrile convulsions
- alcohol withdrawal
- non-epileptic
- idiopathic
- stroke, haemorrhage
- head injury
- Alzheimer's, dementia
- tumour
- metabolic disturbance - electrolytes
102
Non-epileptic seizure px
- Situational
- Longer
- Closed eyes / mouth during tonic clonic
- No incontinence / tongue biting
- May have psychological cause
- Don't occur during sleep
- Preserved consciousness
- Purposeful movements
- Pelvic thrusting
- crying afterwards
- don't occur when alone
103
Types of seizures
Focal (partial) seizures - one hemisphere, specific area
- simple - no LOC
- complex - impaired awareness
- secondary generalised - evolve into bl, convulsive seizure
Generalised seizure - widespread, both hemispheres, LOC
- Tonic-clonic - muscle tensing + muscle jerking
- Absence - blank/unresponsive for 10-20s
- Myoclonic - rapid, brief muscle jerks
- Atonic - sudden loss of muscle tone
Paeds
- infantile spasms / West syndrome - tx with ACTH / vigabatrin
- Lennox-Gastaut syndrome
- Benign rolandic epilepsy
- Juvenile myoclonic epilepsy
104
Epilepsy Px
Focal
- Motor – Jacksonian march
- Sensory – déjà vu, jamais vu, strange smells / tastes / sight / sound
Aura
- Pt aware
- Strange gut feeling, déjà vu, strange smells, flashing lights
- Implies focal seizure, but not necessarily in temporal lobe
Tonic clonic
- Tonic clonic
- LOC
- Tongue biting
- Incontinence
- Groaning
Post-ictal
- Confused, tired, irritable, low
- Todd’s paralysis – temporary weakness after seizure
- May have dysphasia – from focal seizure in temporal lobe
Absence
- Stop activity, vacant, 20-30s, carries on
Myoclonic
- Sudden isolated jerk of muscle
Tonic seizure
- Sudden increased tone, intense stiffness, no jerking
- Characteristic cry / grunt
Atonic
- Loss of muscle tone, fall
105
Epilepsy px by lobe
Temporal lobe (memory, emotion, speech understanding)
- Rising epigastric sensation
- Aura – déjà vu, jamais vu, auditory hallucinations, funny smells, fear
- Anxiety, out-of-body experience, automatisms – eg lip smacking, chewing, fiddling
Frontal lobe (motor and thought processing)
- Motor features – posturing, peddling movts of leg
- Jacksonian march (seizure ‘marches’ up/down motor homunculus starting in face/thumb)
- Post-ictal Todd’s paralysis – paralysis of limbs involved in seizure
Parietal lobe (interprets sensations)
- Paraesthesia
Occipital lobe (vision)
- Visual phenomena – spots, lines, flashes
106
Epilepsy DDx
- Vasovagal syncope
- Non-epileptic attacks
- Cardiac syncope
- Hypoglycaemia
- Hemiplegic migraine
- TIA
107
Epilepsy Ix
- clinical dx - >2 unprovoked seizures >24hrs apart
- bloods - FBC, U/E, bone profile, Mg, LFTs, glucose
- ECG
- ?infective cause - blood/urine cultures, LP, CXR
- CT / MRI head
- EEG - support dx
108
Epilepsy Mx overview
- most neurologists start AEDs after 2nd seizure
- start after 1st seizure if - neuro deficit, structural abnormality, EEG unequivocal of epilepsy, family find further seizure unacceptable
- prescribe by brand (not generic)
- seizure free for >12mo before driving
109
Generalised tonic clonic Mx
M - sodium valproate
F - lamotrigine / levetiracetam
110
Focal seizure Mx
1st - lamotrigine / levetiracetam
2nd - carbamazepine
111
Absence seizure Mx
1st - ethosuximide
2nd - M - sodium valproate
- F - lamotrigine / levetiracetam
112
Myoclonic seizures Mx
M - sodium valproate
F - levetiracetam
113
Tonic/ atonic seizures Mx
M - sodium valproate
F - lamotrigine
114
Pregnancy in epilepsy
- folic acid 5mg OD
- carbamazepine least teratogenic
- Phenytoin - cleft palate, give vit K in last month of pregnancy also
- Lamotrigine - congenital malformation rate low
- Breastfeeding - safe for mothers on antiepileptics
- No sodium valproate - significant risk of neurodevelopmental delay
115
Contraception and epilepsy
If on phenytoin, carbamazepine, barbiturates, primidone, topiramate, oxcarbazepine:
- UKMEC 3: the COCP and POP
- UKMEC 2: implant
- UKMEC 1: Depo-Provera, IUD, IUS
If on lamotrigine
- UKMEC 3: the COCP
- UKMEC 1: POP, implant, Depo-Provera, IUD, IUS
If cOCP chosen, should contain minimum 30mcg ethinylestradiol
If on levetiracetam - can use all contraceptive types
116
Epilepsy cx
- aspiration pneumonia
- sudden unexpected death in epilepsy (SUDEP)
117
Status epilepticus
- seizure > 5mins
- or multiple seizures without regaining consciousness in between
118
Status epilepticus Ix
- A-E
- Check pupils
- BMs
- VBG, seizure bloods
- ?Toxins
- Check for focal neurology, meningism, temp, tongue biting, incontinence etc
- ?CT head
- ?LP
- EEG – and to check seizures controlled after mx
119
Status epilepticus Mx
- Secure airway - NPA, adjuncts etc
- Recovery position
- 15L O2
- Get IV access (or IO)
At 5 mins – benzodiazepine
- Buccal midazolam 10mg
- Rectal diazepam 10mg
- IV lorazepam 4mg
At 10 mins – benzodiazepine
At 15 mins – IV phenytoin / levetiracetam, call ITU
At 20 mins – RSI
120
Headache
Primary
- no underlying cause
- tension, migraine, cluster
Secondary
- underlying cause
- eg GCA, glaucoma, haemorrhage, meds....
121
Headache red flags
- Fever, photophobia, neck stiffness - meningitis, encephalitis, brain abscess
- Focal neurology
- New cognitive disturbance
- Impaired GCS
- Visual disturbance - eg GCA, glaucoma, tumour
- Red eye - glaucoma
- Sudden-onset headache - 'thunderclap'
- Worse on coughing, straining
- Postural, worse standing / lying / bending over
- Vomiting
- Hx of trauma
- Hx of malignancy
- Pregnancy - pre-eclampsia
- Immunocompromised - HIV, immunosuppressants
- <20yo and hx of malignancy
- Seizures
122
Tension headache
- common primary headache - most common cause of primary headache in adults
- infrequent/frequent episodic, chronic
Causes
- eg stress, depression, alcohol, skipping meals, dehydration
123
Tension headache Px
- Episodic, 30 mins - 7d
- Like tight band around head
- No aura, N+V, not aggravated by routine physical activity
- May have photophobia / phonophobia
124
Tension headache Mx
- paracetamol, NSAIDs, aspirin
- limit analgesia to <6d/mo
- acupuncture
- physio, exercise, CBT, relaxation
- trial of amitriptyline
125
Migraine
- Complex neuro condition causing episodes of headaches + associated sx
- most common type in paeds/teenagers
Types
- migraine +/- aura
- silent migraine - aura, no headache
- hemiplegic migraine
126
Migraine patho
- likely combination of structural, functional, chemical, vascular, inflammatory factors
Triggers - CHOCOLATE
- Chocolate
- Hangovers
- Orgasms
- Cheese, caffeine
- Oral contraceptives
- Lie-ins
- Alcohol
- Tumult – loud noise (also bright lights)
- Exercise
127
Migraine Px
Prodrome
- Can begin several days before
- Yawning, fatigue, mood change
Headache
- 4-72hrs
- Unilateral
- Throbbing / pounding
- Aggravated by routine physical activity
- Photophobia
- Phonophobia
- N+V
Aura
- <60mins
- Vision – lines, loss of visual fields (eg scotoma)
- Sensation – tingling, numbness
- Language - dysphasia
Resolution stage
- Headache may fade away / relieved abruptly by vomiting / sleeping
Postdromal / recovery phase
Hemiplegic
- Unilateral limb weakness
- Ataxia
- Impaired consciousness
- Mimic stroke / TIA
128
Migraine dx criteria (5,4,3,2,1)
A - at least 5 attacks fulfilling criteria B-D
B - last 4-72hrs
C - at least 2 of:
- Unilateral
- Pulsating
- Moderate / severe pain
- Aggravated by routine activity
D - during headache, at least 1 of:
- N +/- V
- Photophobia, phonophobia
E - no other cause
129
Migraine Mx
Acute
- NSAIDs, paracetamol
- triptans - sumatriptan - stimulate serotonin receptors
- antiemetics - prochlorperazine / metoclopramide
- no opiates
Prevention
- avoid triggers
- propranolol
- topiramate
- amitriptyline
- specialist - candesartan, valproate, MAbs
- menstrual migraines - prophylactic triptans, mefenamic acid, aspirin
Pregnancy
- paracetamol
- NSAIDs 1st/2nd trim
- no aspirin/opioids
- safe to prescribe HRT
130
Cluster headache
- most disabling type of primary headache
- potential triggers - alcohol, strong smells, exercise
131
Cluster headache Px
- Occur in clusters which last several weeks, occur once a year
- Intense sharp, stabbing pain / headache around one eye
- Occurs 1-2 times/day, episode lasts 15mins-2hrs
- Restless during attack
- Redness, lacrimation, lid swelling, swelling
- Nasal stuffiness
- Miosis / ptosis in minority
132
Cluster headache Ix
- MRI with gadolinium contrast
133
Cluster headache Mx
- neurology referral
Acute
- 15L O2
- S/C triptan (or intranasal)
Prophylaxis
- verapamil
- occipital nerve block, prednisolone, lithium
134
Medication overuse headache Px
- Present for >15d per month
- Developed/worsened whilst taking regular symptomatic medication
- Pts using opioids and triptans at most risk
- May be psychiatric comorbidity
135
Medication overuse headache Mx
- withdraw simple analgesics + triptans abruptly (may worsen headache initially)
- gradually withdraw opioids
Withdrawal sx
- Vomiting, hypotension, tachycardia, restlessness, sleep disturbance, anxiety
136
Post-LP headache
- due to leak of CSF after dural puncture
Px
- 24-48hrs after LP, can be <1wk later
- Can last several days
- Worsens when upright, improves with recumbent position
Mx
- supportive - analgesia, rest
- if pain >72hrs, tx to prevent SDH - blood patch / epidural saline / IV caffeine
137
Sinusitis
- Inflammation of paranasal sinuses in face
- Pain / pressure after viral URTI
- May have swelling / tenderness of affected areas
- Most resolve in 2-3wks
- >10d - steroid nasal spray, abx (phenoxymethylpenicillin)
138
Cervical spondylosis
- Caused by degenerative changes in c-spine
- Neck pain, worse on movt, often also headaches
139
Hormonal headache
- low oestrogen
- similar to migraines - unilateral, pulsatile headache, nausea
- eg 2d before and first 3d of menstrual period / early pregnancy / perimenopause
- triptans / NSAIDs (mefenamic acid) to tx
140
Brain tumour
- primary / secondary (10x more common)
141
Brain tumour patho
- can be benign/malignant, high/low grade
Primary
- adults - majority supratentorial, children infratentorial
- various types (see notes)
Secondary
- NSCLC - most common
- SCLC
- breast
- melanoma
- renal cell
- GI
- SOL, CSF lost to compensate, eventually no more CSF, ICP rises acutely -> worsening in sx, eventually coning, death
142
Brain tumour Px
Progressive focal neurology
- location dependent
- eg personality change - frontal lobe
- sensory loss, motor change, speech changes, visual field defects, DANISH for cerebellar
Raised ICP
- Papilloedema
- Headache, worse lying down / waking / at night / coughing
- Vomiting
- CN3/6 palsies
- Unilateral ptosis
- Drowsiness
Seizures
- focal seizures more common
143
Brain tumours Ix
- CT with contrast
- MRI brain
- Biopsy - during surgery
- LP CI'd (raised ICP)
144
Brain tumours Mx
- surgery
- chemo / radio
- dexamethasone
- levetiracetam
- palliative care
145
Meningitis
- inflammation of meninges
- viral most common, presume bacterial until otherwise, also fungal, non-infective
- meningococcal septicaemia - bacterial infection spreads to bloodstream - non-blanching rash
146
Meningitis causes
Bacterial
0-3mo - GBS, E coli, listeria
3mo-6yo - N meningitidis, S pneumoniae, H influenzae
6-60yo - N meningiditis, S pneumoniae
>60yo - S pneumoniae, N meningiditis, Listeria
Immuno - listeria
Viral
- coxsackie, HSV, VZV, CMV, HIV, measles, mumps
Non-infective
- malignancy, chemicals, meds, sarcoidosis, SLE
147
Meningitis Px
- fever
- neck stiffness
- vomiting
- headache
- photophobia
- altered consciousness
- seizures
- non-blanching rash
- Kernig's
- Brudzinski's
148
Meningitis LP interpretation
- send for - bacterial culture, viral PCR, cell count, protein, glucose, lactate, ?TB Ix
- Bacterial - cloudy, high protein, low glucose, high WCC (neutrophils), culture growth
- Viral - clear, mildly high protein, normal glucose, high WCC (lymphocytes), negative culture
- TB - slightly cloudy, low glucose, high protein, PCR to dx
- delay LP if - sepsis/rash, B/C compromise, bleeding risk, raised ICP
149
Meningitis Ix
- LP
- bloods - cultures, meningococcal PCR, FBC, U/E, glucose, lactate, coag, VBG
- maybe throat swab
- CT not normally indicated
150
Meningitis Mx
Community - meningococcal:
- IM benzylpenicillin
Broad spec abx
- <3mo – IV cefotaxime + amoxicillin
- 3mo-50yo – IV cefotaxime / ceftriaxone
- >50yo – cefotaxime / ceftriaxone + amoxicillin
Focused abx tx
Meningococcal meningitis - IV benzylpenicillin / cefotaxime / ceftriaxone
Pneumococcal meningitis
- IV cefotaxime / ceftriaxone - add vancomycin if risk of penicillin-resistant pneumococcal infection – recent foreign travel / prolonged abx exposure
H influenzae meningitis - IV cefotaxime / ceftriaxone
Listeria meningitis - IV amoxicillin + gentamicin
- If previous penicillin allergy, consider chloramphenicol
- IV dexamethasone - avoid <3mo, shock/meningococcal
- IV fluids
Viral
- self-limiting
- acyclovir if ?secondary to HSV
Mx of contacts - close contact <7d sx onset
- oral ciprofloxacin single dose, rifampicin 2nd line
- offer meningococcal vaccination/booster
151
Meningitis Cx
- Sensorineural hearing loss - most common
- Seizures
- Focal neuro deficit
- Cognitive impairment
- Sepsis, cerebral abscess
- Brain herniation, hydrocephalus
- With meningococcal meningitis - Waterhouse-Friderichsen syndrome - adrenal insufficiency secondary to adrenal haemorrhage
152
Encephalitis
- infection / inflammation of brain parenchyma
- mostly viral
- typically temporal / inferior frontal lobes
153
Encephalitis causes
Viral
- HSV1 (cold sores), HSV2 (genital herpes), VZV, CMV, EBV, HIV, mumps, measles
- HSV encephalitis - tends to affect temporal lobes
Non-viral
- bacterial, TB, malaria, polio, mumps, rubella, measles
154
Encephalitis Px
- Fever
- Headache
- Psych sx, unusual behaviour
- Altered cognition, consciousness
- Seizures, often focal
- Vomiting
- Focal neurology, eg aphasia
155
Encephalitis Ix
- LP - high lymphocytes/protein, send for PCR, CI if low GCS, unstable, seizing
- CT (contrast enhanced) / MRI - petechial haemorrhages in medial temporal/inferior frontal lobes
- EEG - lateralised periodic discharges at 2Hz
- HIV testing, swabs elsewhere
156
Encephalitis Mx
- IV acyclovir
157
Facial nerve palsy
- dysfunction of facial nerve -> unilateral facial weakness
- each side of forehead has UMN innervation from both sides of brain
UMN lesion
- forehead spared
- strokes, tumours
- bilateral - MND, pseudobulbar palsies
LMN lesion
- forehead not spared (paralysed)
- Bell's, Ramsay Hunt, OM, OE, HIV....
158
Bell's palsy
- acute, unilateral idiopathic facial nerve paralysis
- cause unknown, maybe HSV involvement
159
Bell's palsy Px
- Unilateral facial muscle weakness, forehead affected (LMN facial palsy)
- Rapid onset <72hrs
- Post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis (sounds seem louder), eye not closing, drooling
160
Bell's palsy dx
- dx made when no other cause for paralysis found
161
Bell's palsy Mx
- oral prednisolone within 72hrs
- eye care - drops, tape closed at night
- refer if atypical sx / worsening / no improvement
162
Ramsay Hunt syndrome
- reactivation of VZV in geniculate ganglion of CN7
Px
- auricular pain
- facial nerve palsy
- vesicular rash around ear
- vertigo, tinnitus
Mx
- oral acyclovir
- corticosteroids
163
Brain abscess
- pus-filled swelling in brain
Causes
- from middle ear / sinuses
- trauma
- surgery to scalp
- penetrating head injuries
- emboli from IE
164
Brain abscess Px
- headache - dull, persistent
- fever
- focal neurology - site dependent
- raised ICP - nausea, papilloedema, seizures
165
Brain abscess Ix
- CT head
166
Brain abscess mx
- surgery - craniotomy, debride
- IV abx - ceftriaxone + metronidazole
- dexamethasone
167
Charcot-Marie-Tooth disease
- hereditary peripheral neuropathy - predominantly motor - majority autosomal dominant
Px
- Hx of frequently sprained ankles
- Foot drop
- High-arched feet (pes cavus)
- Hammer toes
- Distal muscle weakness / atrophy – inverted champagne bottle legs
- Hyporeflexia
- Stork leg deformity
- Peripheral sensory loss
Mx
- OT/PT, podiatry
168
DDx peripheral neuropathy
- alcohol
- B12 deficiency
- cancer (myeloma), CKD
- diabetes, drugs (amiodarone, isoniazid, nitrofurantoin, metronidazole)
- vasculitis
169
Essential tremor
- autosomal dominant condition - tremor usually affecting upper limbs/hands
170
Essential tremor Px
- postural tremor (worse on movt) 6-12Hz
- symmetrical
- improved with alcohol/rest
- worse when tired, stressed, caffeine
- absent during sleep
- titubation - head tremor
171
Essential tremor Mx
- propranalol
- primidone
172
Idiopathic intracranial HTN (IIH)
- build up of pressure around brain
RFs
- female, obese, pregnancy, drugs...
173
IIH Px
- headache, worse in morning/coughing/straining
- nausea
- blurred vision
- papilloedema
- enlarged blind spot
- sixth nerve palsy
174
IIH Ix
- assess neurology, eyes, vision
- CT / MRI
- LP
175
IIH Mx
- wt loss
- acetazolamide / topiramate - act as diuretics
- repeated LP
- surgery - eg shunt / optic nerve sheath decompression
176
Narcolepsy
- long term brain condition causing pt to fall asleep suddenly at inappropriate times
- low levels orexin (hypocretin)
177
Narcolepsy Px
- Onset in teenage years
- Hypersomnolence - sleepy during day, difficult to stay awake
- Sleep attacks - fall asleep suddenly / w/o warning
- Cataplexy - sudden loss of muscle tone triggered by emotion
- Sleep paralysis
- Vivid hallucinations on going to sleep / waking up
178
Narcolepsy DDx
- sleep apnoea
- restless legs in bed
- hypothyroid
179
Narcolepsy Ix
- multiple sleep latency EEG
- CSF orexin levels - low
180
Narcolepsy Mx
- Daytime stimulants - modafinil
- Night time - sodium oxybate
181
Trigeminal neuralgia
- pain syndrome - severe unilateral pain in face
- affects any branch of trigeminal nerve - ophthalmic, maxillary, mandibular
- idiopathic / nerve compression
- possible triggers - light touch, cold weather, spicy food, caffeine, citrus
182
Trigeminal neuralgia Px
- intense facial pain, seconds-hrs
- electricity-like
- attacks worsen over time
183
Trigeminal neuralgia red flags for serious underlying cause
- Sensory changes
- Deafness, ear problems
- Skin / oral lesions
- Pain only in ophthalmic division, or bilaterally
- Optic neuritis
- MS FHx
- <40yo
184
Trigeminal neuralgia Mx
- carbamazepine
- neurology referral if atypical features, eg <50yo
- surgery to decompress/intentionally damage nerve
185
Neurofibromatosis
- genetic condition that causes nerve tumours (neuromas) to develop throughout nervous system
- tumours benign, but cause neuro/structural problems
- NF1>NF2
186
NF1
- gene on c17 - codes for neurofibromin - autosomal dominant
Dx - need 2/7 - CRABBING
C – café-au-lait spots (>6), >5mm in children or >15mm in adults
R – relative with NF1
A – axillary / inguinal freckles
BB – bony dysplasia (eg Bowing of long bone or sphenoid wing dysplasia)
I – Iris hamartomas (Lisch nodules) (>2) – yellow brown spots on iris
N – neurofibromas (>2) or 1 plexiform neurofibroma
G – glioma of optic nerve
Ix
- clinical dx
- genetic testing
- XR - bone lesions
- CT / MRI
Mx
- no tx
- mx sx / tx cx (various...)
187
NF2
- gene on c22 - codes for merlin - lack of leads to Schwannomas - autosomal dominant
Px
- acoustic neuromas - hearing loss, tinnitus, balance issues
- schwannoma
Mx
- surgery to resect
188
Hydrocephalus
- excessive volume of CSF in ventricular system in brain - production/absorption imbalance
Obstructive
- tumours, haemorrhage, aqueduct stenosis
Non-obstructive
- choroid plexus tumour, reabsorption failure, normal pressure hydrocephalus
189
Hydrocephalus Px
- raised ICP sx
- headache - worse in morning, lying down, Valsalva
- N+V
- papilloedema
- coma
- infants increased head circumference
190
Hydrocephalus Ix
- CT head
- MRI
- LP - drain CSF, measure opening pressure (do not use in obstructive causes)
191
Hydrocephalus Mx
- external ventricular drain (EVD)
- ventriculoperitoneal shunt (VP shunt)
- surgical tx for obstructive pathology
192
Normal pressure hydrocephalus
- reversible cause of dementia in elderly
- reduced CSF absorption at arachnoid villi / head injury / SAH / meningitis
193
Normal pressure hydrocephalus Px
- urinary incontinence
- dementia, bradyphrenia (slowed thinking)
- gait abnormality
- sx develop over a few months
194
Normal pressure hydrocephalus Ix
- CT head - hydrocephalus, ventriculomegaly, no sulcal enlargement
195
Normal pressure hydrocephalus Mx
- VP shunt
196
Tuberous sclerosis
- autosomal dominant condition causing features in multiple systems
- development of hamartomas (benign neoplastic growths)
- TSC1/2 mutations - proteins control size/growth of cells
197
Tuberous sclerosis Px
- Classical px – child with epilepsy + skin features of tuberous sclerosis
Skin signs
- Ash leaf spots – depigmented areas
- Shagreen patches – thickened, dimpled, pigmented patches of skin
- Angiofibromas – small skin-coloured / pigmented papules – on nose / cheeks
- Subungual fibromata – fibromas in nail bed – circular, painless lumps, displace nail
- Café-au-lait spots – light brown (coffee + milk) flat lesions on skin
- Poliosis – patch of white hair on head, eyebrows, eyelashes, beard
Neuro signs
- Epilepsy
- LD, developmental delay
Other sx
- Rhabdomyoma in heart
- Gliomas (tumour of brain + spinal cord)
- Polycystic kidneys
- Lymphangioleiomyomatosis – abnormal growth in smooth muscle, often affecting lungs (lung cysts)
- Retinal hamartomas
198
Tuberous sclerosis Mx
- supprotive
- mTOR inhibitors
- tx cx
199
Aphasia
Wernicke’s (receptive)
- Lesion of superior temporal gyrus, typically supplied by inferior division of left MCA
- Forms speech before sending it to Broca’s
- Sentences make no sense, word substitution, neologisms, but speech fluent (word salad)
- Comprehension impaired
Broca’s (expressive)
- Lesion of inferior frontal gyrus, superior division of left MCA
- Speech non-fluent, laboured, halting, repetition impaired
- Comprehension normal
Conduction aphasia
- From stroke affecting arcuate fasciculus – connection between Wernicke’s and Broca’s
- Speech fluent, repetition poor
- Comprehension normal
Global aphasia
- Large legion affecting all three areas above
- Severe expressive and receptive aphasia
200
Restless legs syndrome (RLS)
- spontaneous, continuous lower limb movts, associated with paraesthesia
Causes
- FHx, IDA, uraemia, DM, pregnancy
201
RLS Px
- Akathisia - uncontrollable urge to move legs - worse at night initially, then progresses to during day, sx worse at rest
- Crawling / throbbing sensations
- Noted in sleep by partner - periodic limb movts of sleeps (PLMS)
202
RLS Ix
- Clinical dx
- Ferritin - r/o iron deficiency anaemia
203
RLS Mx
- Walking, stretching, massaging
- Iron
- Dopamine agonist 1st line - pramipexole, ropinirole
- Benzos
- Gabapentin
204
Reye's syndrome
- Severe progressive encephalopathy affecting children
- Associated with fatty infiltration of liver, kidneys, pancreas
- Known association with aspirin, ?viral cause also
Px
- Preceding viral illness
- Confusion, seizures, cerebral oedema, coma
- Fatty infiltration of liver, kidneys, pancreas
- Hypoglycaemia
Mx
- Supportive
205
Lambert-Eaton myasthenic syndrome
- paraneoplastic syndrome of reduced ACh release at NMJ
- SCLC, breast, ovarian, also autoimmune
- AB against presynaptic voltage-gated Ca channels in PNS
206
Lambert-Eaton myasthenic syndrome Px
- limb girdle weakness (lower limbs first)
- hyporeflexia
- exercise improves sx
- autonomic - dry mouth, impotence, difficulty peeing
- no ptosis (MG)
207
Lambert-Eaton myasthenic syndrome Ix
- EMG - incremental response to repetitive electrical stimulation
208
Lambert-Eaton myasthenic syndrome Mx
- tx cancer
- immunosuppression - prednisolone / azathioprine
- amifampridine / 3,4-diaminopyridine
- pyridostigmine
- IVIg, plasma exchange
209
Neuropathic pain
- pain which arises following damage/disruption to nervous system
Px
- burning, tingling, pins/needles, electric shocks, loss of sensation to touch
Ix
- DN4 questionnaire
Mx
- amitriptyline / duloxetine / gabapentin / pregabalin
- tramadol for exacerbations
- topical capsaicin
- pain clinic
- physiotherapy
210
Complex regional pain syndrome
- Areas are affected by abnormal nerve functioning -> neuropathic pain, abnormal sensations
- Usually one limb, often triggered by injury to area
- Painful, hypersensitive (even to clothing), swell, change colour/temp, flush, sweat
- Tx as neuropathic, also under pain specialist
211
Vestibular schwannoma
- acoustic neuroma
- 90% cerebellopontine angle tumours
- Bl in NF2
Px
- CN8 – vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
- CN5 – absent corneal reflex
- CN7 – facial palsy
Ix
- Urgent ENT referral
- MRI of cerebellopontine angle
- Audiometry
Mx
- Surgery
- Radiotherapy
- Watch and wait
212
Pituitary apoplexy
- sudden enlargement of pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage/infarction
- eg HTN, pregnancy, trauma, anticoags
Px
- sudden onset thunderclap headache
- vomiting, neck stiffness
- Bitemporal superior quadrantic visual field defect
- Extraocular nerve palsies
- Features of pituitary insufficiency – eg hypotension / hyponatraemia secondary to hypoadrenalism
Ix
- MRI
Mx
- steroid replacement (loss of ACTH)
- careful fluid balance
- surgery
213
Syringomyelia
- collection of CSF in spinal cord
- syringobulbia - fluid-filled cavity in medulla
Causes
- chiari malformation
- trauma
- tumours
- idiopathic
214
Syringomyelia Px
- cape-like distribution - neck, shoulders, arms
- loss of temp sensation
- light touch, proprioception, vibration preserved
- spastic weakness - lower limbs
- neuropathic pain
- upgoing plantars
- bladder/bowel dysfunction
- Horner's syndrome (rare)
- scoliosis years later
215
Syringomyelia Ix
- full spine MRI with contrast
- brain MRI (exclude chiari)
216
Syringomyelia Mx
- tx cause
- shunt if persistent
217
Autonomic dysreflexia
- clinical syndrome with spinal cord injury at/above T6
- Afferent signals (most commonly from faecal impaction / urinary retention) cause sympathetic spinal reflex via thoracolumbar outflow
- The usual centrally-mediated parasympathetic response prevented by cord lesion
- Results in unbalanced physiological response – extreme HTN, flushing, sweating above level of cord lesion, agitation, haemorrhagic stroke
Mx
- Remove / control stimulus
- Tx HTN +/- bradycardia
218
Visual field defects
- Left homonymous hemianopia means visual field defect to left, ie lesion of right optic tract
- Homonymous quadrantanopias – PITS
- Congruous defect – complete / symmetrical visual field loss. Incongruous – incomplete / asymmetric
- Incongruous defects – optic tract lesion, congruous defects – optic radiation lesion / occipital cortex
Homonymous hemianopia
- Incongruous – optic tract lesion
- Congruous – optic radiation / occipital cortex
- Macula sparing – occipital cortex
Homonymous quadrantanopias
- Superior – lesion of inferior optic radiations, temporal lobe, Meyer’s loop
- Inferior – superior optic radiations, parietal lobe, Baum’s
Bitemporal hemianopia
- Lesion of optic chiasm
- Upper quadrant > lower – inferior chiasmal compression – pituitary tumour
- Lower > upper – superior compression - craniopharyngioma
219
Arnold-Chiari malformation
- Downward displacement / herniation of cerebellar tonsils through foramen magnum – congenital / acquired through trauma
Px
- Hydrocephalus from obstruction of CSF outflow
- Headache
- Syringomyelia
220
Erb's palsy
- Damage to C5,6
- From excessive lateral flexion of neck – trauma / birth injury
Px
- Sensory loss in C5,6 dermatomes
- Weak elbow flexion, wrist extension, arm abduction + external rotation -> flexed wrist, extended forearm, internally rotated + adducted arm (waiter’s tip posture)
- Asymmetric moro reflex in infants
221
Klumpke's palsy
- Damage to lower trunk of brachial plexus – T1 (C8-T1)
- Due to hyperabduction of arm – trauma / birth injury, or compression – Pancoast tumour / rib
Px
- Sensory loss in C8, T1 dermatomes
- Weakness of intrinsic hand muscles -> total claw hand
- Absent grasp reflex in infants
222
Axillary nerve palsy (C5-6)
Causes
- Anterior shoulder dislocation
- Fracture surgical neck of humerus
Motor
- Deltoid – impaired abduction, atrophy
- Teres minor – impaired external rotation
Sensory
- Lateral shoulder
223
Musculocutaneous nerve palsy (C5-7)
Causes
- Trauma, Erb’s
Motor
- Impaired elbow flexion – brachialis / coracobrachialis
- Impaired forearm supination – biceps brachii
Sensory
- Lateral forearm, elbow to base of thumb
224
Median nerve palsy (C5-T1)
Causes
- Proximal – supracondylar humerus fracture
- Distal – carpal tunnel syndrome, wrist lac
Motor
- Supplies LOAF – lateral 2 lumbricals, opponens pollicis, abductor pollicis brevis, flexor pollicis brevis
- Median claw, ape hand
- Can’t make OK sign
- Thenar muscle atrophy
- Proximal damage – unable to pronate, weak wrist flexion, wrist ulnar deviation
Sensory
- Palmar thumb, index, middle fingers, lateral ring finger
225
Radial nerve palsy (C5-T1)
Causes
- Axilla – crutch, Saturday night palsy
- Midshaft fracture of humerus
- Wrist fracture, tight bracelets
Motor
- Impaired elbow extension
- Wrist drop – impaired extension
Sensory
- Dorsal thumb, index, middle, and lateral ring finger
226
Ulnar nerve palsy (C7-T1)
Causes
- Proximal – fracture of medial epicondyle of humerus
- Distal – ulnar tunnel syndrome, cycling, hook of hamate fracture
Motor
- Motor to medial 2 lumbricals, adductor pollicis, interossei, hypothenar, flexor carpi ulnaris
- Ulnar claw – hyperextension of MCPJ, flexion at distal and proximal IPJ of 4/5th digits
- Wasting + paralysis of intrinsic hand muscles (except lateral two lumbricals)
- Wasting + paralysis of hypothenar muscles
- Damage at elbow – as above + radial deviation of wrist
Sensory
- Medial half of ring finger, little finger, dorsal and palmar
227
Long thoracic nerve palsy
Causes
- Axillary surgery, stab wound, carrying backpack for long time
Motor
- Serratus anterior – scapula winging, impaired abduction of arm beyond 90 degrees
228
Superior gluteal nerve palsy (L4-S1)
Causes
- IM injection
Motor
- Paralysis of glut medius / minimus – impaired hip abduction. Positive Trendelenburg (lateral pelvic tilt towards opposite side)
229
Inferior gluteal nerve palsy (L5-S2)
Causes
- Posterior hip dislocation
Motor
- Paralysis of glut maximus – impaired thigh extension – difficulty standing up, climbing stairs
230
Femoral nerve palsy (L2-4)
Causes
- Trauma, prolonged pressure
Motor
- Impaired hip flexion, knee extension
Sensory
- Anteromedial thigh (anterior cutaneous branches), medial lower leg/edge of foot (saphenous nerve lesion)
231
Lateral femoral cutaneous palsy (L2-3)
Causes
- Compression at level of inguinal ligament – increased abdo pressure, external compression, tumours
Sensory
- Pain, paraesthesia – lateral surface of anterior thigh
232
Obturator nerve palsy (L2-3)
Causes
- Pelvic ring fracture, obturator hernia
Motor
- Paralysis of hip adductors
Sensory
- Medial thigh
233
Sciatic nerve palsy (L4-S3)
Causes
- Iatrogenic, IM injection
- Trauma, hip dislocation, total hip arthroplasty
Motor
- Impaired knee flexion (hamstrings), motor deficits of tibial nerve and common peroneal
Sensory
- Lower leg + foot
234
Tibial nerve palsy (L4-S3)
Causes
- Tibial fracture, tarsal tunnel syndrome
Motor
- Impaired foot inversion / plantarflexion – can’t walk on toes, cannot invert foot (TIPPED)
Sensory
- Sole of foot
235
Common peroneal nerve palsy (L4-S2)
Causes
- Fracture of fibular head
Motor
- Deep peroneal nerve – paralysis of dorsiflexors – foot drop, high-stepping gait
- Superficial nerve – impaired pronation / eversion of foot (TIPPED)
Sensory
- Deep – flip-flop zone between first and second toes
- Superficial – lateral lower leg, dorsum of foot
236
Sural nerve palsy (L4-S3)
Causes
- Achilles tendon rupture, entrapment
Sensory
- Posterolateral lower leg, lateral border of foot, small area under heel
237
Olfactory (I)
- Palsy – change in smell
238
Optic (II)
- Palsy – change in sight – see visual field defects
239
Oculomotor (III)
- All eye muscles except LR and SO. Pupil constriction, accommodation, eyelid opening
- Palsy – ptosis, down + out eye, fixed dilated pupil
240
Trochlear (IV)
- Superior oblique
- Palsy – defective downward gaze – vertical diplopia – noticed when looking down – head tilt to compensate
241
Trigeminal (V)
- Facial sensation, mastication
- Palsy – trigeminal neuralgia, loss of corneal reflex (afferent), loss of facial sensation, paralysis of mastication muscles, deviation to jaw to weak side
242
Abducens (VI)
- Lateral rectus
- Palsy – defective abduction – horizontal diplopia
243
Facial (VII)
- Facial movt, taste anterior 2/3 tongue, lacrimation, salivation
- Palsy – flaccid paralysis of upper + lower face, loss of corneal reflex (efferent), loss of taste, hyperacusis
244
Vestibulocochlear (VIII)
- Hearing, balance
- Palsy – hearing loss, vertigo, nystagmus. Acoustic neuromas – Schwann cell tumours of cochlear nerve
245
Glossopharyngeal (IX)
- Taste posterior 1/3 tongue. Salivation, swallowing, mediates input from carotid body + sinus
- Palsy – hypersensitive carotid sinus reflex, loss of gag reflex (afferent)
246
Vagus (X)
- Phonation, swallowing, innervates viscera
- Palsy – uvula deviates away from site of lesion, loss of gag reflex (efferent)
247
Accessory (XI)
- Head + shoulder movt
- Palsy – weakness turning head to contralateral side, weakness shrugging shoulder
248
Hypoglossal (XII)
- Tongue movt
- Palsy – tongue deviates towards side of lesion
249
ALS
- Affects corticospinal tracts (UMN) and LMNs - upper and lower motor neuron signs
250
Poliomyelitis
Affects anterior horns - LMN signs
251
Brown-Sequard syndrome
- Hemi-section of spinal cord
Lateral corticospinal tract – ipsilateral spastic paresis below lesion
- Decussates at medulla
DCML – ipsilateral loss of proprioception and vibration, fine touch
- Decussates at medulla
Lateral spinothalamic – contralateral loss pain and temp sensation
- Decussates almost immediately in spinal cord (1-2 levels below lesion)
252
Friedrich's ataxia
- Same as above
- Other features of cerebellar disease as well as ataxia - eg intention tremor
253
Anterior cord syndrome
- Eg from anterior spinal artery occlusion, flexion/extension
- Lateral corticospinal tracts – bilateral spastic paresis
- Lateral spinothalamic tracts – bilateral loss of pain + temp sensation
254
Central cord syndrome
- Eg from hyperextension
- Small lesions - spinothalamic. Large lesions - spinothalamic, DCML, corticospinal
- Weakness and numbness - more so in upper limbs than lower limbs - cape-like distribution
255
Posterior cord syndrome
- Eg from B12 deficiency, MS, tumour compression, neck hyperextension, posterior spinal artery infarct
- DCML – loss of fine touch, vibration, proprioception
- Wide steppage gait – sensory ataxia. Positive Romberg’s
256
Neurosyphilis
Dorsal columns - loss of proprioception, vibration
(o Eg from B12 deficiency, MS, tumour compression, neck hyperextension, posterior spinal artery infarct
o DCML – loss of fine touch, vibration, proprioception
Wide steppage gait – sensory ataxia. Positive Romberg’s)
257
Subacute combined generation of spinal cord
- B12 deficiency, and E
- Dorsal columns and lateral corticospinal tracts affected
- Joint position + vibration lost first, then distal paraesthesia
- UMN signs in legs – upgoing plantars, brisk knee, absent ankle jerk
- If untreated – stiffness + weakness persist
Lateral corticospinal tracts – bilateral spastic paresis
DCML – bilateral loss of proprioception + vibration
Spinocerebellar tracts – bilateral limb ataxia
258
Cerebral venous sinus thrombosis (CVST)
- thrombosis in brain's venous sinuses - prevents blood draining
- 50% isolated sagittal sinus
- also cavernous sinus thrombosis / lateral sinus
259
CVST Px
- sudden headache
- N+V
- reduced GCS
260
CVST Ix
- MRI venography, CT alternative
- D dimer elevated maybe
261
CVST Mx
- anticoagulation - LMWH, then warfarin
262
CVST specific syndromes
Sagittal sinus thrombosis
- Seizures, hemiplegia
- Parasagittal biparetial / bifrontal haemorrhagic infarctions sometimes seen
- Empty delta sign on venography
Cavernous sinus thrombosis
- Other causes – local infection (sinusitis), neoplasia, trauma
- Periorbital oedema
- Ophthalmoplegia – 6th nerve damage, then 3/4th
- Trigeminal nerve involvement – hyperaesthesia of upper face, eye pain
- Central retinal vein thrombosis
Lateral sinus thrombosis
- 6/7th CN palsies
263
Degenerative cervical myelopathy (DCM)
- stenosis of c-spine leading to spinal cord compression, neurology
- age-related osteoarthritis
264
DCM Px
- subtle early sx, progressive
- pain - neck, upper/lower limbs
- loss of motor function - dexterity, impaired gait
- loss of sensory function - numbness
- autonomic - urinary/faecal incontinence, impotence
- Hoffman's sign positive
265
DCM Ix
MRI c spine
266
DCM Mx
- refer for spinal services assessment
- decompressive surgery
267
Ataxia telangiectasia
- Autosomal recessive disorder – defect in ATM gene – codes for DNA repair enzymes
- An inherited combined immunodeficiency disorder
Px
- Early childhood
- Cerebellar ataxia
- Telangiectasia (spider angiomas)
- IgA deficiency – recurrent chest infections
- 10% risk of malignancy, lymphoma, leukaemia, non-lymphoid tumours
268
Friedreich's ataxia
- Autosomal recessive, trinucleotide repeat disorder (GAA repeat in X25 gene on c9)
- Does not demonstrate anticipation
Px
- 10-15yo
- Gait ataxia
- Kyphoscoliosis
- Neuro sx - absent ankle jerks / extensor plantars, cerebellar ataxia, optic atrophy, spinocerebellar tract degeneration
- HOCM
- DM
- High-arched palate
269
Foot drop causes
- Common peroneal nerve lesion - eg fracture of fibular head, compression
- L5 radiculopathy - would also show weakness of hip abduction
- Sciatic nerve lesion
- Superficial / deep peroneal nerve lesion
- Central nerve lesions, eg stroke - other features would be present
270
Internuclear ophthalmoplegia
- a cause of horizontal dysconjugate eye movt
- due to lesion in medial longitudinal fasciculus (MLF)
Causes
- MS
- vascular disease
Px
- Impaired adduction of eye on same side as lesion
- Horizontal nystagmus of abducting eye on contralateral eye
271
Myotonic dystrophy
- inherited myopathy, sx develop ~20-30yo, affects skeletal, cardiac, smooth muscle
- autosomal dominant trinucleotide repeat disorder, DM1/2 types - distal/proximal weakness more prominent
Px
- Myotonic facies – long, haggard appearance
- Frontal balding
- Bilateral ptosis
- Cataracts
- Dysarthria
- Myotonia (tonic spasm)
- Weakness of arms + legs, distal initially
- Mild mental impairment
- DM
- Testicular atrophy
- Cardiac – HB, cardiomyopathy
- Dysphagia
272
Creutzfeldt-Jakob disease (CJD)
- rapidly progressive neuro condition - prion proteins - induce formation of amyloid folds
- sporadic / new variant
Px
- dementia - rapid onset
- myoclonus
Ix
- CSF - normal
- EEG – biphasic, high amplitude sharp waves (only in sporadic CJD)
- MRI – hyperintense signals in basal ganglia and thalamus
273
Dystrophinopathies
- x-linked recessive
- due to mutation in dystrophin gene - dystrophin connects muscle membrane to actin, part of cytoskeleton
274
Duchenne muscular dystrophy (DMD)
- Frameshift mutation – one/both binding sites lost – more severe form
Px
- Progressive proximal muscle weakness from 5yo
- Calf pseudohypertrophy
- Gower’s sign – child uses arms to stand from squatting
- 30% - LD
275
Becker muscular dystrophy
- Non-frameshift insertion – both binding sites preserved – milder form
Px
- After 10yo
- Intellectual impairment much less common
276
Cataplexy
- Sudden / transient loss of muscular tone caused by strong emotion – eg laughter, being frightened. 2/3 pts who have narcolepsy have cataplexy
Px
- Buckling knees
- Collapse
277
Thoracic outlet syndrome (TOS)
- Compression of brachial plexus, subclavian artery, or vein at site of thoracic outlet (space between clavicle and first rib)
- neurogenic (90%) / vascular
- pts usually thin young women, long neck, drooping shoulder
- after neck trauma, may have cervical rib
278
TOS Px
Neuro
- painless muscle wasting of hand muscles - weak grasp
- numbness/tingling
- autonomic nerves - cold hands, blanching, swelling
Vascular
- vein - diffuse arm swelling, distended veins
- artery - claudication, ulceration/gangrene if severe
279
TOS Ix
- CXR, c-spine XR
- CT / MRI
- venography / angiography
- anterior scalene block - confirm neurogenic TOS
280
TOS Mx
- Conservative mx - education, rehab, physio, taping
- Surgical decompression
- Botox?
281
Multiple system atrophy (MSA)
- Parkinsonism
- Autonomic disturbance - erectile dysfunction, postural hypotension, atonic bladder
- Cerebellar signs
282
Cavernous sinus
- On body of sphenoid bone - runs from superior orbital fissure to petrous temporal bone
- Medial - is pituitary fossa, sphenoid sinus
- Lateral - temporal lobe
Lateral wall components - from top to bottom
- Oculomotor nerve
- Trochlear nerve
- Ophthalmic nerve
- Maxillary nerve
Contents of sinus - from medial to lateral
- Internal carotid artery (and sympathetic plexus)
- Abducens nerve
Blood supply
- Ophthalmic vein, superficial cortical veins, basilar plexus of veins posteriorly.
- Drains into the internal jugular vein via: the superior and inferior petrosal sinuses
283
Paroxysmal hemicrania
- Attacks of severe, unilateral headache - usually orbital, supraorbital, temporal region
- Autonomic features also
- Last 30 mins, multiple times a day
- Tx with indomethacin
284
Reflexes
- S1-2 - ankle
- L3-4 - knee
- C5-6 - biceps
- C7-8 - triceps
- S1,2 tie shoe, L3,4 kick the door, C5,6 pick up sticks, C7,8 shut gate
285
Radiculopathy
- compression of nerve root / LMN
- eg pinched nerve
- degenerative disc disease, OA, facet joint degeneration, spondylosis
- radicular pain, weakness in limbs, numbness / paraesthesia
- MRI, maybe EMG
- neuropathic pain meds, tx cause, surgery
286
Neuropathy
pathological process affecting nerve(s)
287
Mononeuropathy
- single nerve affected
- eg CTS
288
Mononeuritis multiplex
Several individual nerves affected
Systemic causes - WARDS PLC
- Wegener's granulomatosis
- AIDs/amyloid
- RA
- DM
- Sarcoidosis
- PAN (polyarteritis nodosa)
- Leprosy
- Carcinoma
289
Polyneuropathy
- many nerves affected
- diffuse, symmetrical
- Can be motor, sensory, sensorimotor, autonomic
- Demyelination / axonal degeneration
- Often distal – glove + stocking
Mostly motor
- GBS
- Porphyria
- Lead poisoning
- Hereditary sensorimotor neuropathies (HSMN) – Charcot-Marie-Tooth
- Chronic inflammatory demyelinating polyneuropathy (CIDP)
- Diphtheria
Mostly sensory
- Diabetes
- Uraemia
- Leprosy
- Alcoholism
- B12 deficiency - subacute combined degeneration of spinal cord
- Amyloidosis
Px
- Sensory deficit
- Weakness, muscle cramps, fasciculations, atrophy
- Poor balance, ataxia
Mx
- Tx cause
- Neuropathic pain meds if needed
290
Spontaneous intracranial hypotension
- CSF leak causes headaches
- Leak typically from thoracic nerve root sleeves
- RFs - Marfan's
- Headache worse upright
- Ix - MRI with gadolinium
- Mx - conservative, epidural blood patch
291
Von Hippel-Lindau syndrome
- Autosomal dominant condition – predisposes to neoplasia
- Abnormality in VHL gene on c3
Features
- Cerebellar haemangiomas – cause SAHs
- Retinal haemangiomas
- Renal cysts, and elsewhere
- Phaeochromocytoma
- RCC
