Neurology Flashcards
Unconsciousness / coma
- Consciousness – state of wakefulness and awareness of self and surroundings
- Coma – unarousable unresponsiveness
define vegetative state, minimally conscious syndrome and locked-in syndrome
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
Brain-stem death
Neurological damage to brainstem resulting in irreversible loss of consciousness and loss of spontaneous ventilation
Pre-conditions for brain-stem death testing
- 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
who can perform brain stem death testing?
- 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)
how do you test for brainstem death?
- 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
stroke definiton
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
what is an ischaemic stroke? risk factors?
- 85%, thrombo/embolus, also shock, vasculitis
RFs - age, HTN, smoking, high cholesterol, DM, AF, cOCP, FHx, carotid artery stenosis
what is a haemorrhagic stroke? risk factors?
- 15%, intracerebral/subarach
- RFs - age, HTN, AV malformation, anticoagulation
stroke Px - general
- 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
Oxford Stroke Classification
Assess for:
- unilateral hemiparesis +/- hemisensory loss of face, arm, leg
- homonymous hemianopia
- higher cognitive dysfunction, eg dysphasia
TACS / PACS / LACS / POCS
Total anterior circulation infarct (TACS)
- 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)
Partial anterior circulation infarct (PACS)
- 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)
Lacunar syndrome (LACS)
- Arteries around internal capsule, thalamus, basal ganglia
1 of following
- Unilateral weakness +/- sensory deficit of face, arm, leg
- Pure sensory stroke
- Ataxic hemiparesis
Posterior circulation syndrome (POCS)
- Vertebrobasilar arteries
1 of following
- Cerebellar / brainstem syndromes
- Loss of consciousness
- Isolated homonymous hemianopia
Brainstem infarct
- quadriplegia
- locked-in syndrome (basilar artery)
Lateral medullary syndrome (PICA) - Wallenberg
- Ipsilateral - ataxia, nystagmus, dysphagia, facial numbness, CN palsy (Horner’s)
- Contralateral - limb sensory loss
Weber syndrome - branches of PCA that supply midbrain
- Ipsilateral CN3 palsy
- Contralateral weakness of upper + lower limbs
Anterior inferior cerebellar artery (AICA) - lateral pontine syndrome
- Similar to Wallenberg’s but:
- Ipsilateral - facial paralysis and deafness
Retinal/ophthalmic artery?
- amaurosis fugax
Stroke by areas: ACA, MCA, PCA
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
Stroke Ix
- 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
stroke initial Mx?
- 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
stroke secondary prevention?
- 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)
carotid artery assessment post TIA or stroke ?
Carotid endarterectomy - if stenosis >70%
- consider angioplasty / stenting
patient found to have AF after TIA/stroke ?
- warfarin / DOAC
- start immediately in TIA
- wait 2wks in acute stroke
Stroke mx of other issues
Fluids
- assess hydration
- oral hydration, IV saline (no dextrose)
Glycaemic control
- monitor BMs - aim for 4-11
- IV insulin / glucose infusions for diabetics
BP Mx
- use anti-HTN if hypertensive emergency
- IV labetalol, nicardipine….
Feeding
- screen for safe swallow
- NG feed
- nasal bridle tube / gastrostomy
- assess meds for NG feed
- nutritional support
Disability
- Barthel index to measure functional decline
TIA?
- transient episode of neurological dysfunction caused by focal brain/spinal cord/retinal ischaemia, without acute infarction
crescendo TIA?
2+ TIAs in 1wk - high risk of stroke
TIA Px
- Typically resolve in 1hr
- Unilateral weakness / sensory loss
- Aphasia / dysarthria
- Ataxia, vertigo, loss of balance
- Visual problems, amaurosis fugax, diplopia, homonymous hemianopia
TIA Ix
- CT
- MRI, inc diffusion-weighted / blood-sensitive
- Carotid imaging - doppler, CT/MR
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
Extradural haemorrhage (EDH)
- blood between skull + dura
- trauma, fracture to temporal/parietal bone, rupture of middle meningeal artery
EDH Px?
- head trauma
- initial LOC / drowsiness
- lucid interval, later low GCS
- headache, N+V, confusion, seizures, focal neurology, fixed dilated pupil, deep coma
what is Cushing’s reflex?
response to acutely raised ICP:
- bradycardia
- irregular respirations
- HTN / increase in pulse pressure (systolic up, diastolic down)
EDH Ix
- bloods, inc G/S
- non-contrast CT head - biconvex (lemon) hyperdense collection +/- mass effect
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
Subdural haemorrhage (SDH)
- blood between dura + arachnoid layers
- acute / subacute / chronic
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
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
SDH Ix
- non-contrast CT head - hyperdense crescent (sickle) shaped collection
- bloods etc
SDH Mx
- A-E
- neuroprotection
Neurosurgery - Burr hole / open craniotomy, clot evacuation
Subarachnoid haemorrhage (SAH)
bleed into subarachnoid space - between arachnoid / pia (where CSF is)
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
SAH RFs
- 45-70yo, F, black, HTN, smoking, alcohol, FHx, cocaine, SCA, Marfan’s, EDH, PKD
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
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
SAH Mx
- nimodipine
Neurosurgery - endovascular coiling
- neurosurgical clipping
- intracranial stents / balloons
SAH Cx
- rebleed - 20%
- cerebral ischaemia
- hydrocephalus - VP shunt / EVD
- hyponatraemia (SIADH) - fluid restriction
Intracerebral haemorrhage
- bleed in brain tissue
- spontaneous / secondary to ischaemic stroke, tumours, aneurysm rupture
Px
- similar to SAH
- stroke, focal neurology, reduced GCS
Ix
- non-contrast CT head - hyperdensity in substance of brain
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
dementia definition?
- irreversible, progressive decline of higher brain function - cognitive/behavioural sx - memory loss, reasoning/communication problems, change in personality, reduction in ADLs
RFx for dementia?
- older, mild cognitive impairment, genetics, Parkinson’s, CVA, smoking, DM, sedentary, obesity
what is Alzheimer’s dementia ?
- type of dementia (50-75%)
- amyloid plaques / tau proteins in brain - reduce transmission, brain cell death
- widespread cerebral atrophy, esp cortex / hippocampus
how does Alzheimer’s present?
- Steady decline
- Memory loss
- Loss of executive function / dysphasia
- Aphasia, apraxia, agnosia
- Changes in planning, reasoning, speech, orientation
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
vascular dementia?
- type of dementia (<20%)
- Group of syndromes of cognitive impairment caused by ischaemia / haemorrhage secondary to cerebrovascular disease
- affects white matter of cerebral hemispheres, grey nuclei, thalamus, striatum
Types
- stroke-related - multi/single-infarct
- subcortical - small vessel disease
- mixed dementia - VD + Alzheimer’s
RFx for vascular dementia?
- previous stroke/TIA, older, AF, DM, lipids, smoking
vascular dementia Px?
- Stepwise progression - stability + acute decline
- Cognitive impairment
- Mood disturbance - psychosis, delusions, hallucinations, paranoia
- Gait, speech, emotional, memory disturbance
- Seizures
- Focal neurolog
vascular dementia Ix?
- screen for depression, psychomotor retardation
- formal cognitive screen
- MRI - infarcts, white matter changes
- Dx with NINDS-AIREN criteria
vascular dementia Mx?
- cognitive stimulation syndromes, multisensory stimulation, music/art therapy, animal therapy
- address pain, avoid overcrowding, clear communication
what is Lewy body dementia?
- type of dementia (10-15%), >50yo
- lewy body proteins deposited in brain - SN, paralimbic, neocortical areas
how does Lewy body present?
- progressive cognitive impairment - may fluctuate, early impairments to attention, executive function
- sleep disorder
- visual hallucinations
- Parkinson’s like sx - bradykinesia, resting tremor, rigidity
how is lewy body Mx?
- AChE - donepezil, rivastigmine
- avoid neuroleptics (may develop Parkinsonism)
what is Frontotemporal dementia (FTD)?
- type of dementia (2%), <65yo
- neuron damage/death at frontal/temporal lobes, atrophy due to tau protein deposition
FTD Px?
Behavioural
- Change in behaviour / personality, eg disinhibited, impulsive, decline in social skills..
Semantic
- Lack of understanding word meanings, difficulty in name retrieval…
Non-fluent
- Breakdown in speech, apraxia, decline in literac
FTD Mx
- maybe SSRIs / trazadone for behavioural sx
general management of dementia?
Cognitive impairment
- Memory loss, difficulty with reasoning / communication / decisions, dysphasia, coordinating movements, not orientated
Behavioural and psychological sx of dementia (BPSD)
- Psychosis – delusions / hallucinations, agitation / emotional lability, depression / anxiety, motor disturbance, sleep disturbance
Difficulties with ADLs
- Household tasks, toileting eating, walking
Ddx of dementia
- CJD
- HIV-related cognitive impairment / dementia
- depression
- normal pressure hydrocephalus
- mild cognitive impairment
- delirium
- reversible causes - hypothyroid, Addison’s, B12, folate, thiamine, syphilis, tumour, SDH, drugs,
dementia Ix
- cognitive screen
- bloods:
- Exclude reversible causes (eg hypothyroid) – FBC, U/E, LFTs, Ca, glucose, ESR/CRP, TFTs, vit B12, folate
- Maybe urine MC+S, CXR, ECG, syphilis serology, HIV testing
Imaging
- CT / MRI head
- consider SPECT scan, PET scan
what is a cognitive screen?
NICE recommended
- 10-CS
- 6CIT
- 6-item screener
- memory impairment screen
- mini-cog
- Test your memory
Not NICE recommended
- AMTS
- GPCOG
- MMSE
causes of falls?
- TLOC
- mechanical fall
- geriatric fall
- visual impairment
- BPPV
- drugs, polypharmacy
TLOC causes
- syncope
- seizure
- hypoglycaemia
- head injury
- narcolepsy
- stroke
define syncope?
- transient LOC due to cerebral hypoperfusion - low BP after fall in CO / SVR
define orthostatic / postural hypotension
- fall in BP >20/10 on standing - blood pools in legs with gravity, reduced venous return, reduced preload/EDV/SV/CO (normally compensated for by sympathetic increase in HR/SVR
- Primary autonomic failure - older, Parkinson’s, Lewy Body, MSA
- secondary - DM, adrenal insufficiency, hypothyroid
- drugs - anti-HTN, diuretics, alcohol
- hypovolaemia - D+V, diuresis, haemorrhage
cardiac syncope?
- structural - MI, valvular heart disease, cardiomyopathy
- Arrhythmias - tachy/brady
reflex syncope?
- increase in vagal tone
- vasovagal - pain/emotions
- carotid sinus hypersensitivity
- situational syncope - micturition, defecation, cough
RFx for falls?
Leg weakness, vision problems, balance / gait disturbance, polypharmacy, incontinence, >65yo, depression, arthritis, psychoactive drugs, cognitive impairment
medication that can contribute to falls
Meds that cause postural hypotension
- Nitrates, diuretics, anticholinergics, antidepressants, BBs, levodopa, ACEi
Meds that cause falls by other mechanisms
- Benzos, antipsychotics, opiates, anti-epileptics, codeine, digoxin, sedatives
how to investigate falls?
- Hx - before, during, after, any LOC, injuries, anticoags, collateral hx, seizure sx, what were they doing at the time, ?self-mobilised, DHx, SHx…
- A-E
- Full cardio, resp, GI, neuro exams
- Check c-spine, pelvis, hips (?SLR),
- Obs, BMs, urine dip, ECG
- FBC, U/E, LFTs, bone profile, Mg, CRP, CK if long lie, coag, haematinics, vit D, TFTs
- XR of injured limbs
- CT head
- ECHO
- Lying standing BP
- Turn 180 tests / timed up and go test
- Tilt table test - measure HR / BP as table with pt on tilts - for postural hypotension
managing falls?
- tx cause
- adjust meds
- IV fluids
- Cardiology referral if needed
- Maybe neuro obs
- Vasovagal / situational - reassure
- Inpatient – 1-1 nursing, non-slip socks, low rise beds / mattresses on floor
- Walking aids
- OT/PT review
- Package of care if going home
management of postural hypotension?
- Tx cause, review meds, ensure hydrated
- Fludrocortisone
- Midodrine – alpha agonist – for true autonomic dysfunction
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
what types of MS are there/
- clinically isolated
- relapsing remitting
- secondary progressive
- primary progressive
clinically isolated syndrome in context of MS?
- First episode of demyelination
- May never have another episode, or go on to develop MS
relapsing remitting MS?
- 85%
- Acute attacks (1-2mo), periods of remission
secondary progressive MS?
- Deterioration after relapsing-remitting – neuro signs between relapses
- 65% with relapsing-remitting go on to develop secondary progressive within 15yrs of dx
primary progressive MS?
- 10% pts
- Progressive deterioration from onset
- More common in older pts
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
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
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
management of MS
Acute relapses
- high dose oral / IV steroids - methyl/prednisolone
Disease-modifying therapies
- natalizumab, ocrelizumab, fingolimod…
Symptomatic tx
- fatigue - amantadine, mindfulness, CBT
- spasticity - baclofen/gabapentin, diazepam, physio
- bladder dysfunction - USS, ISC, anticholinergics (solifenacin)
- oscillopsia - gabapentin/memantin
MND
- progressive destruction of motor neurons
- cause unknown, 5% have SOD1 mutation
- possibly mitochondrial dysfunction - oxidative stress, ROS damages stuff
- Frontotemporal dementia in 25%
types of MND?
- amyotrophic lateral sclerosis (ALS)
- progressive bulbar palsy (PBP)
- progressive muscular atrophy (PMA)
- Primary lateral sclerosis (PLS)
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
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
LMN signs?
- Muscle wasting
- Reduced tone – flaccid paralysis
- Fasciculations
- Reduced reflexes
UMN signs?
- Increased tone – spastic paralysis
- Brisk reflexes
- Upgoing plantar reflex
MND Ix?
- clinical dx
- NCS - normal
- EMG - reduced no of APs, increased amplitude
- MRI - exclude myelopathy etc
MND Mx?
- riluzole
- NIV - resp support
- baclofen
- PEG
- benzos
- advanced directives
Guillain-Barre syndrome (GBS) - definition
- immune mediated demyelination of PNS
- triggered by infection - C jejuni
- ABs against infection attack Schwann cells -> demyelination
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
GBS Ix?
- LP - raised protein, normal WCC
- NCS - decreased velocity
- spirometry - monitor FVC
GBS Mx?
- FVC <80% - mechanical ventilation
- IV Ig 1st line
- plasma exchange
- VTE prophylaxis
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
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
what tests can you do to elicit fatiguability
Tests to elicit fatigability
- Count to 50, voice quietens
- Prolonged upward gazing exacerbates diplopia
- Repeated blinking -> ptosis
- Repeated abduction of one arm 20x -> unilateral weakness on that side
MG Ix
- ABs - AChR, MuSK, LRP4
- CK normal
- CT / MRI thorax - thymus
- Single fibre electromyography
MG Mx
- AChE inhibitors - pyridostigmine
- immunosuppression - prednisolone, azathioprine, cyclosporin, mycophenolate
- thymectomy
- rituximab
myasthenia crisis?
- life-threatening cx of MG
- acute worsening of sx - often triggered by illness - eg resp infection
- monitor FVC - resp failure - NIV / mechanical ventilation
Mx
- IV Ig
- plasmapheresis
what is Huntington’s?
- Inherited neurodegenerative disorder which causes chorea - jerky, semi-purposeful movements
- autosomal dominant
- CAG repeats, may see anticipation
- lack of GABA + ACh, decreased inhibition of dopamine release -> excessive movts
how does Huntington’s present?
- Sx develop >35yo
- Starts with mild psychotic / behavioural sx - cognitive, psych, mood problems
- Chorea - random, irregular, abnormal body movts
- Personality changes - irritable, apathy, depression, intellectual impairment
- Dystonia
- Rigidity
- Speech / swallowing difficulties
- Saccadic eye movts
Huntington’s Ix
- genetic testing, genetic counselling
Huntington’s Mx
- MDT, physio, SALT
- chorea - benzos, tetrabenazine, sulpiride
- antidepressants
- advanced directives
Parkinson’s - definition
- Progressive degenerative movt disorder caused by degeneration of dopaminergic neurons in substantia nigra (in basal ganglia)
- dementia, depression associations
- from mitochondrial dysfunction / oxidative stress
key Parkinson’s triad?
Tremor
- worse at rest, 4-6Hz
- worse stressed/tired
- improves on voluntary movt
- pill-rolling
Bradykinesia
- hypokinesia/poverty of movt
- short, shuffling steps, reduced arm swinging
- difficulty initiating movt
- micrographia / monotonous hypophonic speech
Rigidity
- increased tone in limbs/trunk
- lead pipe - throughout ROM
- cogwheel - superimposed tremor
other potential Parkinson’s sx?
- hypomimia - reduced facial movts
- flexed posture
- saliva drooling
- psych - depression, dementia, psychosis, sleep disturbance, cognitive impairment, memory problems
- anosmia
- REM sleep behaviour disorder
- fatigue
- postural hypotension
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
Parkinson’s Ix
- clinical dx
- CT / MRI head
- Use UK Parkinson’s Disease Society Brain Bank Clinical Diagnostic Criteria
- SPECT scan
Parkinson’s Mx?
First line
- if motor sx affecting QoL - levodopa
- if not - dopamine agonist / levodopa / MAO-B
Neuropsych sx
- anti-depressants, anti-psychotics
Anti-muscarinics
- block cholinergic receptors - drug-induced Parkinsonism
- procyclidine
what is levodopa?
- synthetic dopamine, less effective over time
- add decarboxylase inhibitor - prevent peripheral metabolism
- co-beneldopa (Madopa)
- co-careldopa (Sinemet)
- S/Es - dyskinesia (amantadine to tx), on-off effect, end-of-dose wearing off, postural hypotension, arrhythmias, N+V, psychosis
what is a 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
Parkinson’s plus syndrome?
- group of neurodegenerative diseases with classical sx of Parkinson’s + additional features
Multiple system atrophy (MSA)
- neurons of brain degenerate, inc basal ganglia
- Parkinsonism, autonomic dysfunction, cerebellar dysfunction
Progressive supranuclear palsy (PSP)
- postural instability, falls, impairment of vertical gaze
- parkinsonism, cognitive impairment
Corticobasal degeneration (CBD)
Dementia with Lewy bodies (DLB)
Frontotemporal dementia (Pick’s disease)
epilepsy definiton?
neurological condition characterised by recurrent seizures
definition of a seizure?
Disordered electrical activity in the brain leading to clinical manifestationsexcessive, abnormal electrical activity in the brain
causes of seizure
- epilepsy
- febrile convulsions
- alcohol withdrawal
- non-epileptic
- idiopathic
- stroke, haemorrhage
- head injury
- Alzheimer’s, dementia
- tumour
- metabolic disturbance - electrolytes
