Neuro Flashcards
Multiple sclerosis, myasthenia, TIA, guillain barre, epilepsy, stroke, charcot marie tooth, parkinsons,, myopathies, peripheral neuopathies
What is multiple sclerosis, dx. and how does it usually present
autoimmune disease causing demyelination of nerves - often has relapsing and remitting disease leading to progressive MS caused by incomplete healing of demyelination
- needs two discrete time and space attacks to diagnose (or 1 acute episode + MRI evidence of other one)
Presents
Neurological deficit >1 hour, more than 30 day between attacks. Usually one deficit
eg. weakness or paresthesia in limbs
- optic neuritis (pain on movement + reduced acuity)
- ataxia, dysarthria/ tremor
- urinary urgency/faecal incontinence
- CN symptoms, vertigo, dementia, seizures
+ depression,
Risk factors for MS + flare. Exam features
Family hx, caucasian, F>M, 20-40 yo, HLA DRB1, living far from equator
Risk for flare
- heat, infection/fever, exercise, post partum relapse
Exam - unilateral signs
- UMN weakness - spastic
- sensory loss of fine touch/vibration/proprioception (posterior column)
- cerebellar signs- nystagmus/ slurred speech/impaired coordination/dysmetria/dysdiadochokinesia
-cranial nerves: decreased VA, central scotoma, inter-nuclear opthalmoplegia
DDx for MS + investigations
DDx
- sarcoidosis
- small vessel ischaemia/multiple emboli/stroke/
- spinal cord compression in trauma/RA,
- spinal tumour,
- optic neuritis could be neuromyelitis optica
Ix
Renal function for drugs
-MRI head + spine +/- gadolinium, T1 T2 looking for demyelinated sites
- -high signal T2 lesions
periventricular plaques, dawson fingers
- evoked response testing - visual delayed because of demyelination but well preserved waveform
- somatosensory, auditory
- LP for CSF
- oligoclonal IgG bands = CNS inflammation - worse prognosis
- myelin basic protein - acute demylinaton
- WCC>100ml/L severe demyelination
- increased intrathecal synthesis of IgG (serum vs CSF IgG and albumin ratio
-ANA
Treatment of MS - pharm and non pharm - acute relapse, bladder spasticity, bladder dysfunction, facial spasm, tremor
- MDT (OT, physio), support groups
- Bed rest with nursing during relapses
- Splint if foot drop
- Bowel and bladder self catheterization
Regular exercise, stopping smoking and avoiding stress
Pharm
- Acute relapse/Flare treated with high dose prednisone or IV methyprednisolone if severe - doesn’t alter progress
- Iv interferons and monoclonal antibodies eg. Natalizumab, ocralizumab - reduce relapse frequency
- Or plasma exchange to get rid of other antibodies
- Other immunosuppressives - dimethyl fumarate (modulator), methotrexate, azathioprine
Symptomatic
Severe spasticity - baclofen (muscle relaxant)
Bladder dysfunction/spasm - oxybutynin (antciholin) + amitriptyline
Facial spasm - carbamazepine
Tremor - clonazepam or propanolol
what are the symptoms & past medical history to ask on history for myasthenia gravis
Symptoms
o Ocular – diplopia, drooping eyelids
o Bulbar – difficulty chewing and
swallowing, choking, dysarthria
o Limb girdle – proximal muscle
weakness, fatigue on exertion
- Weakness worsened by pregnancy, exercise, infection, change in climate, emotion, over treatment, drugs such as gentamicin, tetracycline, beta blockers.
- Past medical history
o Difficult anaesthesia (prolonged
muscle weakness after anaesthesia)
o Pneumonia
o Thymectomy
o Other auto-immune diseases – SLE,
rheumatoid arthritis - How much anticholinesterase required?
- Any admissions to hospital with myasthenic crisis? (resp distress)
what are the specific signs and investigations of myasthenia gravis on exam / ix
EXAMINATION
- Observation
o Ptosis
o Peek sign
o Smile – snarling expression
- Muscle fatigue
o Sustained upward gaze
o Counting aloud
o Hold arms above head - Weakness of neck flexion
- Thymectomy scar
- Reflexes intact, no sensory loss, muscle atrophy minimal
- diplopia
INVESTIGATIONS
- Antibodies
o Acetylcholine receptor antibodies
(anti-AChR) in 90%
o If seronegative, look for muscle specific kinase antibodies (MuSK)
- Neurophysiology
o Electromyogram – repetitive
stimulation at low frequencies with
decremental muscle response
o Single fibre EMG – increased jitter
and blocking - Thymoma investigation
o Chest X-ray
o Thoracic CT or MRI - Respiratory function tests
- Look for associated conditions
o TFT’s, RF, ANA
what is the symptomatic,relapse and disease suppression treatment of myasthenia gravis + SE of those treatments
Symptomatic
- Anticholinesterase
o E.g. Pyridostigmine 60-120mg PO
up to 6x daily
o SE: increased salivation, lacrimation,
vomiting, diarrhea
- Avoid drugs that impair NM transmission like gentamicin, procainamide
- Sudden worsening of respiratory symptoms can be life threatening and treated with mechanical ventilation and plasmapheresis.
Often precipitated by infection, so treat aggressively (not with aminoglycosides)
Disease Suppression
- Relapses treated with prednisolone. Often needed long term once anticholinesterases are inadequate.
- steroid SE- Give osteoporosis prophylaxis.
If failed steroids - immunosuppression
o Azathioprine
Methotrexate
o Rituximab in desperate cases
Thymectomy (because MG associated with
thymomas)
o If symptoms < 50 yrs, and ACH-antibody positive
What are the differential dx (proximal muscle weakness) and epidemiological features of myasthenia gravis
DDX
- Lambert-Eaton syndrome.Pre-synaptic
failure of release of acetylcholine caused by
Small CC of lung. Muscle weakness improves
on use.
- Polymyositis
- Acquired myopathy (hyperthyroid, SLE,
Cushing’s)
Epidemiological features of M Gravis
- LMN by autoabs to nicotinic aCH receptors - muscular weakness which worsens with use
- F (30s) > M70s
- extra ocular, bulbar, face, neck, limb girdle, trunk
TIA (deficit <24 hrs) causes - differentials -history
CAUSES
- Atherothromboembolism
o Most common
- Cardioembolism
o Mural thrombus post MI, AF, valve disease, prosthetic valves
- Hyperviscosity
o Polycythemia, elevated WCC, myeloma
DDx
- Hypoglycaemia
- Migraine aura
- Focal epileptic seizure
- Syncope and hypotension
- Hyperventilation
- Vertigo +/- secondary nausea and ataxia
- MS
- Somatization
HISTORY + EXAM FINDINGS
- As per stroke territories
- Global events such as syncope and dizziness
are not typical of TIA
- Amaurosis fugax (progressive loss of vision
in one eye “like a curtain descending”
-
exam and ix for TIA + stroke risk after TIA score
Exam: cardio / resp
Listen for carotid bruit, measure BP, listen
for murmurs, AF
Ix
Bloods
- FBC, ESR, U+E’s glucose, lipids
Imaging
- CXR
- Carotid USS +/- angiography
- CT or diffusion weighted MRI head
- Echo – foramen ovale or other holes?
- ECG - AF
STROKE risk after TIA (score 6-7 - observation in stroke clinic)
A – Age ≥ 60 (1)
B – Blood pressure ≥ 140/90 (1)
C – Clinical Features
Unilateral weakness (2)
Speech disturbance w/o weakness (1)
D – Duration of Symptoms
Lasting ≥ 1 hour (2)
Lasting 10 – 59 minutes (1)
D – Diabetes (1)
Management - non pharm, pharm and invasive for TIA
MANAGEMENT
Non-Pharmacological
- Diet, exercise
- Smoking cessation
- Avoid driving for 1/12
Pharmacological
- Control CV risk factors
o Cautiously lower blood pressure – target 140/85mmhg
o Statins
o Control DM
o Smoking cessation
- Antiplatelet
o Aspirin 300mg daily or Clopidogrel 75mg daily - Anticoagulation
o If cardiac emboli (e.g. from AF or mitral stenosis)
Invasive
- Consider carotid endarterectomy if > 70%
stenosed and operative risk is good.
Operating on 50 – 70% stenosis has some
value if operative risk very low.
- Should be performed within 2 weeks of first
presentation.
- Do not use antiplatelets beforehand.
- Carotid stenting is a good alternative if not good for surgery
Guillain Barre
- presentation - neuro- pain/motor/sensory, resp, speech, face findings
acute inflam demyelinating polyneuropathy triggered by antecedent infection
Motor - progressive muscle weakness over days - lower before upper, proximal before distal.
Areflexia – absent knee/ankle, +/- plantars
- Paraesthesia in feet/hands (mild, before
weakness) - Autonomic dysfunction (sweating,
tachycardia, BP changes, arrhythmias - Pain: legs, back
- Resp: SOB-OE, resp muscle weakness
- Speech: facial & oropharyngeal weakness –
slurring - Face: facial droop, dysphagia
- Eyes: diplopia, extra-occular weakness, ptosis
Ix for guillain barre
INVESTIGATIONS
Nerve conduction studies:
- Slowing of velocities
LP:
- Raised CSF protein (>5.5g/L)
Bloods:
- Raised AST & ALT, slight bili rise
- Anti-ganglioside Ab; helps differentiate subtype (unknown in AIDP)
- Serology Campylobactor, CMV, EBV, Mycoplasma
Spirometry:
- 6hr intervals at bedside
- may show decreased Vc, maximal inspiratory P or expiratory P.
Imaging:
- MRI to rule out spinal cord pathology
diagnostic criteria for guillain barre and management - treatment and supportive
CRITERIA
1. Progressive muscle weakness in limbs
2. Areflexia (hypo-)
3. +/- Progressive over 2-4wks, symmetry,
mild sensory change, CN involved.
Recovery begins 2-4wks after plateau. Autonomic
changes, no fevers, CSF/EMG findings.
MANAGEMENT
Treatment:
- IV Ig 0.4g/kg/24hrs for 5 days (CI: IgA def, renal failure)
- Plasma exchange
Supportive:
- Ventilate (bulbar/bilateral CNVII palsy, dysautonomia) if resp involvement
- DVT prophylaxis
- Monitor vitals
- Analgesia: neuropathic type
- Physio input
guillian barre Monitoring, prognosis and complications
MONITORING
Progression over 2wks, plateau 2-4wks, then
recovery
F/U within 2/52 of acute phase, then 4-6wks for
6months, then at 6 months, then 1yr.
PROGNOSIS
85% good recovery. 20% mortality if ventilated.
Poorer outcomes if older age, severe Sx, rapid onset,
atrophy, need for ventilation, high antibody titre
COMPLICATIONS
Fatigue
Resp Failure (30%)
Bladder areflexia
Adynamic ileus
Paralysis 15% - strengthening exercises in acute rehab
DVT risk – 2 to immobilization
epilepsy - different types of seizures
Focal onset
- With or without awareness
- Motor or non-motor
- Focal to bilateral tonic-clonic
Generalized onset
- Always impaired awareness
- Motor
o Tonic-Clonic Seizures (LOC, limbs stiffen (tonic) then jerk (clonic). )
o Myoclonic Seizures (Sudden jerk of limb, face or trunk)
o Atonic Seizures (Sudden loss of muscle tone, no LOC)
- Non-motor
o Absence Seizures (Brief pauses ≤10s, carries on where left off) - Unknown onset
What are the 4 categories of differentials causes for epilepsy with examples
- Idiopathic (2/3 is familial)
- Structural : Cortical scarring (post head injury)
- Space occupying lesion
- Stroke - Systemic : SLE, Sarcoidosis, Poly arteritis nodosa
- Non-epileptic causes
- Trauma, haemorrhage, increased ICP
- alcohol, benzo
- metabolic disturbance + fever
-liver disease
-infection of brain
-drugs (TCA , cocaine, tramadol)
-pseudo seizures
history and exam of epilepsy
HX
- Details of seizure (ideally from eyewitness)
o Length of time, what it looked like, LOC, tongue biting, incontinence
- Ask about prodrome (change to mood or
behavior hours to days before seizure)
and aura (a partial focal seizure) - Post ictal period (headache, confusion,
myalgia, lethargy),
Todd’s paresis= focal
weakness after seizure - Triggers such as flickering lights, alcohol, TV
- Provocation (as per non epileptic causes)
- First episode? Previous funny turns or odd
behavior? - Family history
- Recent drug or alcohol use, lack of sleep=triggers
Exam focus/ findings for epilepsy and diagnosis of epilepsy
EXAMINATION
- Seek any clinical evidence of a focal brain lesion
- Exclude non-neurological causes
- Pay attention to signs of traumatic injury
- Check tongue
DIAGNOSIS
- Clinical diagnosis
- Positive findings on EEG (spikes) can help but cannot refute
- Make sure is truly a seizure
- Highly likely if:
o no provocation
o absence of syncopal prodrome
o Post-ictal drowsiness +/- confusion
Ix for seizure and Differentials for transient LOC
DIFFERENTIAL DIAGNOSIS FOR TRANSIENT LOC
- Syncope
o Reflex – vasovagal (stress, temp, pain) or carotid sinus. Reflex vasodilation or
bradycardia.
o Orthostatic – failure of homeostatic maintenance of BP on postural change,
dehydration, antihypertensives,
autonomic failure e.g. Parkinson’s
o Arrhythmia – transient compromise of cardiac output “Stokes-Adams attack”
o Cardiac - structural heart disease especially LV outflow obstruction, MI,
PE, AS
- Hypoglycaemia
INVESTIGATIONS
- FBC, glucose, electrolytes, Ca, Mg, Creatinine, LFTs
- Urine toxicology screen
- CXR
- Consider LP
- CT head esp. if fever, focal neuro signs, slow recovery
- EEG/MRI in selected patients, usually as an OP
Non pharm + pharm management of seizures - generalised - atonic/myoclonic/tonic vs partial
MANAGEMENT
Non-Pharmacological
- Avoid triggers
- Avoid driving until seizure free for 1 year
(this can be reduced if a clear and nonrecurring cause is found)
- Counselling regarding swimming, heights,
heavy machinery
- Enlist help of epilepsy nurse specialist
- If first seizure, refer to first seizure clinic
Pharmacological
- Do not start treatment after 1st seizure unless
structural brain lesion, focal neurological deficit, or unequivocal epileptiform EEG
- Neurologist to commence drug therapy after 2nd seizure
- If only 1-2 seizures a year and no need of driving, then they may want to take no meds
- (start low, go slow)
- Generalised (tonic clonic, myoclonic, absence, atonic)
1.Sodium Valproate (first line)
o Lamotrigine (second line)
o Can also consider levetiracetam,
carbamazepine, topiramate,
oxycarbazepine
o AVOID carbamazepine and
oxycarbazepine in tonic, atonic and
myoclonic seizures
- Partial +/- secondary generalization
o Carbamazepine (first line) - If pregnant or breastfeeding:
o Lamotrigine
Invasive
- Neurosurgical resection if clear
epileptogenic focus and refractory to
medication
monitoring/ side effects with anticonvulsants - sodium valproate, lamotrigine, carbamazepine, phenytoin, levetiracetam, topiramate
- SV - highest teratogenecity, pancreatitis, hepatic failure,
- interact with other anticonvulsants, tca, warfarin, aspirin - carbamazepine - worsen absence, myoclonic seizures - allergic rash, hyponatremia (<125 bad), hepatotoxicity . Ok in pregnancy
- toxicity risk w azoles, macrolide, ssri,
- reduces e2, warfarin, Ca2+ blockers, statins - lamotrigine - safe in pregnancy <200mg. SE - allergic rash with increased dose - affected by other anticonvulsant (increased
- phenytoin - worsen absence, myoclonic allergic rash, hirsuitism, hepatotoxicity - induces enzyme - E2, P2 ,warfarin, TCA, CCB, -
- therapeutic drug monitoring - Topiramate - adjunctive therapy ataxia, weight loss, acute angle glaucoma, kidney stone, cognitive impairment
- affect digoxin and cocp increase -
What are the presentation and risk factors for stroke - ischaemic and haemorrhagic
Presentation
Ischaemic 85%
- Large artery atherosclerosis + thrombosis
- Cardioembolic
- Lacunar thrombus in situ
Haemorrhagic
-Hypertension
-Coagulopathy APLS
-AV malformation
-Berry aneurysm
Risk factors
- For Ischaemic: carotid bruit, AF , past TIA, IHD
- For Haemorrhagic: meningism, severe headache and coma within hours
- General risk factors – smoking, HTN, T2DM, lipids
What are the investigations and when do you do them/ what is acute management of stroke - timing aims, inclusion/ exclusion criteria
Acute event
1. Protect airway ABCD
2. Call code stroke
3. Stroke symptoms <24 hours ago - Order CT head non contrast
- parenchymal or subarachnoid haemorrhage ?- yes then CTA circle of willis,
- No - CTA carotids + circle of willis + perfusion
4. Bloods – FBC, U&E, glucose, coags, trops.
Thrombophilia screen in <50yo
5. ECG + obs / neuro monitoring
Acute ischaemic
- aim thrombolysis within 45 minutes of arrival (max 4.5 hrs post event)
– if previously independent.’
- Exclusion – if bleeding on CT, BP >185/110, recent stroke, MI, surgery or trauma within 30 days
—Alteplase 0.9mg/kg max 90 mg. 10% over 1 minute, 90% in 60minute infusion
- Manage SBP 150-170 during infusion/ for first 24hrs –eg. Labetalol, GTN, hydralazine
OR transfer for thrombectomy within 90 min of hospital arrival (max 6 hrs post event for AC, 24hrs PC)
- If independent mobility ADLS prior to stroke, no haemorrhage evidence of clot
- Discuss with OCNeuroSMO
Acute Haemorrhagic
- Stop antithrombotic
Reverse anticoagulation –call haem
Neurosurgical referral
What is the NIH Stroke scale
level of consciousness,
knows month and age
blink eyes & squeeze hands
horizontal extraocular movements
visual fields
facial palsy
left & right arm & leg motor drift (each separate)
Limb ataxia in how many limbs
sensation
language /aphasia - describe scene
dysarthria
extinction/ inattention
What are the aftercares after stroke - ischaemic vs haemorrhagic
Ischaemic
Clopidogrel (300mg loading,75mg daily) + aspirin (300 loading 100-150mg daily)
- within 24hrs if not for reperfusion or 24hours after thrombolysis after repeat CT looking for haemorrhagic transformation
NBM until swallow assessment - +/- IV fluids
Driving restriction
Monitor
-Blood glucose – treat >10 mmol
- Blood pressure
- Neuro-obs as per protocol
- Temperature
Haemorrhagic
Blood pressure control if >220/120 mmHg – reduction only by 20% in first 24 hours max
Primary and secondary prevention + complications of stroke
Primary prevention is management of CVS risk
Secondary Prevention
- Smoking cessation, exercise, diet
- Clopidogrel (P2Y12 antagonist) 75mg daily (better than aspirin)
- Control HTN – continue regular meds, delay new meds until 72 hours.
- Statins 40mg regardless of lipids
-Consider carotid endarterectomy if >70% stenosed
-If in AF, consider anticoagulation after 2 weeks.
Complications
- Falls
- UTI
- Pneumonia – swallow assessment – prevent aspiration
- Pressure sores
- Depression
- Shoulder Pain
- DVT / PE - Prophylaxis
charcot marie tooth - presentation (motor, deformity, sensory, others)+ risk factors
- Hereditary motor + sensory neuropathy of the peripheral nervous system
- Genetic mutations affecting myelin sheath of axons – 7 types (present differently)
o Constant cycle of demyleination and remyelination around nerves
- Middle childhood – 30s/40s – progressive
- Initially asymptomatic 2 years before presentation
Motor
- Weakness and atrophy in peroneal and distal leg muscles – leading to
- Foot drop – weakness in tibialis anterior/ dorsiflexion – disruption of deep peroneal nerve L4/5
- Weakness in hands and forearms as disease progresses
Deformity
- Stork leg deformity – wasting of the calves
- High arched feet (pes cavus) leads to sprained ankle
- flat arched feet (pes planus)
- Hammer toe or claw hands – contraction of muscles
Sensory
- Pain and temperature > Vibration, sensation decreases in a glove stocking pattern
- Deep tendon reflexes absent
- Early and late onset forms – painful spasmodic muscular contractions. Overuse of affected part can activate symptoms
Other features
- Scoliosis, hip socket malformation, involuntary teeth grinding
Risk factors
- Family hx of neuropathy – autosomal dominant inheritance
- Exacerbated by drugs (vincristine, anaesthesia, pregnancy due to progesterone, and phenytoin and carbamazepine)
Ix and management of charcot marie tooth
Progress
- Doesn’t affect lifespan, slow progression
Investigations – Dx by neurology
1. Nerve conduction studies – reduced nerve conduction
2. Sural nerve biopsy showing onion bulb formation around nerves
3. Genetic testing – off blood (or antenatal – chorionic villous sampling) -
Management:
1. Orthotics – splint for walking/stiff boots à stabilizes ankles
2. Physiotherapy to increase muscle strength, flexibility of muscles and reduce risk of ankle fractures
3. OT – education on energy conservation strategies and ADLS
4. Podiatrist – assistance triming nails or removing calluses due to poor sensory reception
5. Muscle/joint pain: NSAIDs
6. Neuropathic pain: amitriptyline, gabapentin or pregabalin
7. Surgical – ankle fusion, straightening and pinning toes, lowering arch.
8. Avoid drugs like vincristine, anaesthetics, phenytoin, carbamazepine (affect sensory side)
hx and exam findings of different stroke territories
- ICA , ACA, MCA, (l and r).
Anterior Circulation
- Internal Carotid Artery
o Hemiparesis and hemianaesthesia on
opposite side of body
o Homonymous hemianopia
o Dysphasia - Anterior Cerebral Artery
o Hemiparesis L > A
o Sensory loss leg only
o Change in personality, mood, behavior
and social inhibition. - Middle Cerebral Artery
o Left MCA – R sided weakness involving
lower part of face, arm > leg with dysphasia
o Right MCA – L sided weakness involving
lower part of face, arm > leg with visual and/or sensory neglect
History for polyneuropathies :
Disorders of peripheral or cranial nerves; usually
symmetrical & widespread, often with distal weakness and ‘glove & stocking’ sensory loss.
Classified by course (acute/chronic), by function
(sensory/motor/autonomic/mixed), or by pathology (demyelinating/axonal
degeneration/both).
Time course
Nature of Sx
- Preceding or associated events (D&V before
GBS, wt loss cancer, arthralgia CT disease)
- Travel, drugs, sexual infections
- FMHx
- Palpable nerve thickening (leprosy, CMT)
- Other cues: ?CLD
Parkinsons presentation/hx and risk factors
Presentation
Premotor symptoms
- REM sleep disorder
- Hyposmia/ anosmia
- Non specific fatigue, depression
- Restless legs
Autonomic dysfunction
- Postural hypotension
- Urinary incontinence
- Gastroparesis - > constipation
Neurodenegerative
- Dementia, hallucinations
Key Motor features
- Usually asymmetrical onset
- Slowness, stiffness, tremor
- Freezing, dyskinesias, wearing off
Drugs
- Current and past
- Ask about how many times taking Sinemet daily
- Effect wearing off early
ACP/EPOA
Support
- Formal
- Informal, including PD society etc
Risk factors
- Family history
- Vascular
- age , drugs
Exam findings in parkinsons
EXAMINATION
Aim is to demonstrate that patient has PD and not
PD plus.
General Inspection
- Masked facies
- Flexed posture – cannot lie flat with head off
pillow and simian stance on standing
- Tremor and titubation (tremor of head)
- Dribbling
- Speech – slow monotonous speech
Core Features (TRAP)
- Tremor
o Pill rolling at rest
o should increase on distraction with
movement of other hand
o should decrease on asking patient to
hold hands out front
o ΔΔ Flapping (liver, respiratory or
renal failure), Intention (cerebellar),
postural (benign essential)
- Rigidity
o Look for cogwheel rigidity
o If not obvious, distract patient by
asking them to tap other knee
- Akinesia (more accurately bradykinesia)
o Finger tapping and piano playing
o Slow movement, decreased RAM,
shuffle walk
- Postural Instability
o Ask pt to rise from chair, walk to the
other side of the room, turn around
and come back.
o Hesitancy, shuffling gait, loss of arm
swing
o (Retropulsion)
Extra Tests
- Glabellar Tap
o Failure of attenuation of blink
response
- Ocular Movements
o Weakness of upward gaze in PD (cf.
loss of downward gaze in PSP)
- Lying and standing BP
Function
- Undo a button
- Write name and address - micrographia
Ddx in parkinsons
DIFFERENTIAL DIAGNOSIS OF PARKINSONISM
Parkinson’s-plus Syndromes
- Progressive Supranuclear Palsy
o Early postural instability, vertical gaze
palsy, rigidity trunk > limbs, pseudobulbar palsy, symmetrical onset, tremor
unusual.
- Multisystem Atrophy
o Early autonomic features, cerebellar
signs, rigidity > tremor.
- Cortico-basal degeneration
o Akinetic rigidity affecting one limb,
apraxia, astereognosis
- Lewy body dementia
o Early dementia with fluctuating
cognition and hallucinations
- Vascular Parkinsonism
o Legs > arms. Prominent gait
abnormality.
Drug Induced
- Antipsychotics- haloperidol and lithium
- Dopamine receptor antagonists
o Prochlorperazine and
Metoclopramide
Toxin Induced
- Copper (Wilson’s disease)
IX in parkinsons and dx
INVESTIGATIONS
- CT or MRI if no tremor to exclude brain
lesion
- Younger patients should have slit lamp for
KF rings (Wilson’s) or serum copper
- Olfactory testing (also reduced in MSA)
- Review by neurologist if young or unusual
presentation
DIAGNOSIS
- Clinical. Refer to specialist without
medication
- Bradykinesia plus one other clinical sx,
progressive and alternate dx less likely and
response to levodopa
Management of parkinsons - pharm non pharm, SE - compare with word doc
MANAGEMENT
Non-Pharmacological
- Educations
- Exercise - PT- strength/speed/ROM/reduce
falls, OT
- Multidisciplinary approach. Neurologist, PD
nurse, SW, GP, respite care, SLTdysphagia/voice volume, psych
Pharmacological- w advice from geriatrician or
neurologist
- Treatment of non-motor symptoms
o Depression SSRI’s
o Psychosis quetiapine or
olanzapine
- Sinemet
o Levodopa + dopadecarboxylase
(dopamine metabolizer) inhibitor.
Initial response dramatic, efficacy
reduces with time. SE: dyskinesia,
painful dystonias, nausea, psychosis.
- Dopamine agonists
o Ropinirole. Used as monotherapy to
delay starting L-dopa in early stages.
o Apomorphine as a continuous
infusion or as a rescue pen for
freezing
- Anticholinergics (Benzotropine)
o Help with tremor. SE: confusion, dry
mouth, dizziness, urinary retention.
Other things stop dopamine levels being metab
- Selegiline (maob inhib- risk of serotonin syndrome)
- Entacapone (comt inhib)
Invasive
- Deep brain stimulation – for younger
patients
- Surgical ablation of overactive basal ganglia
Complications of parkinsons specific to neuro/ psych effects and the treatment of these
Complications
Meds
- Warn with large dose of dopamine ( Sinemet) : impulse disorder unable to resist impulsive activities eat, sex, gambling, porn.
- Tardive dyskinesia with high doses of Sinemet /long term use
Falls risk / Non Pharm
- PT – safe falling, walking aids, strength and balance programme – physical activity slows progression
- OT – equipment, optimise function
- SLT – voice training for hypophonia, assessment of dysphagia
- SW – refer to parkinson’s support group, mobility parking card, financial assistance (disability allowance), medic alert bracelet , respite care
Dyskinesia
- Amantadine 100mg daily with food – in conjunction with levodopa
o CI: epilepsy, gastric ulceration, pregnancy
Tremor
- Anticholinergics (however not started in elderly as can cause urinary retention, hallucination and delirium.
Mental health
o Delusion and hallucination maybe more drug induced but common.’
- Neuropsychiatric degeneration: development of Parkinson disease dementia - executive function goes but memory stays.
- Health psychologist
- SSRI for depression,
- Psychosis – quetiapine at low doses or olanzapine – not for non troubling hallucinations – caution due to worsening motor symptoms
Progression
* Expect 11 years from time of dx to death
* Expect plateauing of Sinemet efficacy after 10 years
Parkinsons disease complications sleep, gi and postural hptn (3) + treatment
Postural Hypotension
- Try high salt diet, thigh high compression stockings ?, r/v of antihypertensive medications
- Fludracortisone – mineralocorticoid mimetic (50-100 mcg daily)
- Minodrine – alpha agonist - (up to 30mg)
GI
- Drooling – 1% atropine eye drops sublingual, radiotherapy
- Dysphagia – diet and SLT,
- Gastroparesis – domperidone 10-20 mg 3-4x daily . Nausea improve posture and small meals
- Constipation – diet – increase fibre + fluids and avoid antimuscarinics. Use bisacodyl suppository in the morning or docusate sodium 100-150mg BD
Sleep
- Sleep hygiene – screen for depression, nocturia.
- Insomnia Nocturnal dose of dopamine
- REM sleep disorder clonazepam 1mg daily
hx and exam findings of different stroke territories
posterior circulation. vertebrobasilar arterial system, pons, medulla, cerebellum
Posterior Circulation
- Posterior Cerebral Artery
o Homonymous hemianopia
o Parietal deficits – spatial skills, recognition
o Temporal deficits – memory, mood, aggression
- Vertebrobasilar arterial System
o Midbrain
3rd and 4th CN deficits same side. Weakness and sensory loss opp side
o Pons
5th and 6th CN deficits same side. Weakness and sensory loss opp side
o Medulla
9th, 10th, 11th CN deficits same side. Weakness and sensory loss opp side
o Cerebellum
DASHING
Dysdiadochokinesia and dysmetria,
ataxia, slurred speech, hypotonia,
intention tremor, nystagmus, gait abnormality
MN disease - pathophys, what are the differentiating factors,
Progressive degenerate of motor neurons - U& LMN but no sensory loss. Never affects eye movements . Rare with mean age of onset 60
EXAM
Upper and lower neuro and CN exam (look for UMN and LMN signs)
- UMN signs – spasticity, brisk reflexes,
upgoing plantars
- LMN signs – wasting, fasciculations
- Dysphagia and dysphasia
- Weakness!
- Memory impairment
What are the features favouring myopathy(timing, distribution, exam finding)
How would you investigate myopathy
FEATURES favoring MYOPATHY (cf neuropathy)
- Gradual onset
- Symmetrical proximal weakness (difficulty combing hair, climbing stairs)
- Preserved tendon reflexes
- Pain at rest, local tenderness (inflam myopathy)
- Oddly firm muscles (DMD)
- Lumps - tendon rupture, muscle herniation
INVESTIGATIONS
- Bloods: ESR, CK, AST, LDH, TSH
- EMG
- DNA analysis (muscle Bx)
What is the heritability, age of onset, pathophys and presentation of facioscapulohumeral muscular dystrophy
Autosominal dominant. Onset 12-14 years old. Pathophys is mutation of DUX4 gene leading to expression in myocytes which is toxic
exam findings
- Facial weakness - ironed out, unable to puff cheeks
- Scapular winging, difficulty raising arms over head, deltoids spared
- Horizontal clavicles, anterior axillary folds
-Scoliosis, foot drop
What is the heritability, age of onset, pathophys and presentation of Becker’s muscular dystrophy
X-linked recessive, onset 8-25 mutation in dystrophin gene which usually protects muscle
Muscle wasting in hip pelvis –> thighs and shoulders -difficulty walking and standing
-Resp failure
- scoliosis
- elevated CK
- o Pseudohypertrophy in calves
List 9 causes of peripheral neuropathies
Metabolic
- DM, Renal failure, hypothyroidism, hypoglycaemia,
Nutritional
- Vit B1, B12 in alcohol abuse, Vit E, folate, B6
Drugs
- Isoniazaid, phenytoin, nitrofurantoin, metronidazole, chemo
Vasculitis
- Poly arteritis nodosa, Wegners
Malignancy
Paraneoplastic, Polycythaemia RV
Inherited
- C-M-T Syndrome, Refsum’s, Porphyria
Inflam
- GBS, sarcoidosis
Infection
- HIV, syphilis, lyme disease
Other
- alc, lead, arsenic poising, amyloidosis, paraproteinaemias
How to differentiate sensory , motor, cranial nerves or autonomic neuropathy
SENSORY NEUROPATHY:
- Numbness, tingling
- Burning pain (alc, DM)
- Glove & stocking
- Check functional ability
- Signs: Tm, joint deformity
MOTOR NEUROPATHY:
- Often progressive
- Weak clumsy hands
- Difficulty walking (falls)
- Difficulty breathing (reduced VC)
- Signs: LMN signs- wasting, weakness distally (foot/hand drop), reduced/absent reflexes
CRANIAL NERVES:
- swallowing or speaking difficulty
AUTONOMIC NEUROPATHY: (SNS & PSNS)
- Postural hypotension (S)
- Erectile dysfunction (P)/Ejaculatory failure (S)
- Decreased sweating (S)
- Constipation, nocturnal diarrhea, urine retention (P)
- Horners (S)
- Homes Adie pupil (P)
*Primary autonomic failure can occur alone (autoimmune) or part of multisystem atrophy (MSA) or with PD.
What are the ix and management of peripheral neuopathy
INVESTIGATIONS
Bloods:
- FBC, ESR, glc, U&E, TSH, B12/folate, ANA,ANCA
CXR
Urine analysis
Consider LP +/- specific studies
Nerve conduction studies: demyelinating vs axonal
MANAGEMENT
- Treat cause
- PT, OT
- Foot care & show choice education
- Joint splinting to prevent contractures
- GBS/CIDP IV Ig; Vasculitis steroids
- Neuropathic pain: amitriptyline, gabapentin
Management of MN disease
Non-Pharmacological
- Multidisciplinary approach
o Neurologist
o Palliative nurse and hospice
o Physio, OT
o SLT, dieticians
o Social services
Pharmacological
- Antiglutamatergic drugs
o Riluzole prolongs life by
approximately 3 months and is very
expensive.
o Causes LFT derangement so
monitor. SE: vomiting, weakness,
somnolence, headache, dizziness,
pain.
- Drooling
o amitriptyline
- Dysphagia
o blend food
o consider NG or PEG but may prolong
life.
- Spasticity
o baclofen, diazepam, dantrolene,
tizanidine
o Start low, go slow.
- Joint pains and distress
o analgesic ladder
- Respiratory failure
o noninvasive ventilation at home for
palliation
