Neuro

Question 1

What is multiple sclerosis, dx. and how does it usually present

Answer 1

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,

Question 2

Risk factors for MS + flare. Exam features

Answer 2

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

Question 3

DDx for MS + investigations

Answer 3

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

Question 4

Treatment of MS - pharm and non pharm - acute relapse, bladder spasticity, bladder dysfunction, facial spasm, tremor

Answer 4

• 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

Question 5

what are the symptoms & past medical history to ask on history for myasthenia gravis

Answer 5

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)

Question 6

what are the specific signs and investigations of myasthenia gravis on exam / ix

Answer 6

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

Question 7

what is the symptomatic,relapse and disease suppression treatment of myasthenia gravis + SE of those treatments

Answer 7

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

Question 8

What are the differential dx (proximal muscle weakness) and epidemiological features of myasthenia gravis

Answer 8

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

Question 9

TIA (deficit <24 hrs) causes - differentials -history

Answer 9

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”
-

Question 10

exam and ix for TIA + stroke risk after TIA score

Answer 10

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)

Question 11

Management - non pharm, pharm and invasive for TIA

Answer 11

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

Question 12

Guillain Barre
- presentation - neuro- pain/motor/sensory, resp, speech, face findings

acute inflam demyelinating polyneuropathy triggered by antecedent infection

Answer 12

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

Question 13

Ix for guillain barre

Answer 13

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

Question 14

diagnostic criteria for guillain barre and management - treatment and supportive

Answer 14

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

Question 15

guillian barre Monitoring, prognosis and complications

Answer 15

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

Question 16

epilepsy - different types of seizures

Answer 16

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

Question 17

What are the 4 categories of differentials causes for epilepsy with examples

Answer 17

1. Idiopathic (2/3 is familial)
2. Structural : Cortical scarring (post head injury)
   - Space occupying lesion
   - Stroke
3. Systemic : SLE, Sarcoidosis, Poly arteritis nodosa
4. Non-epileptic causes
   - Trauma, haemorrhage, increased ICP
   - alcohol, benzo
   - metabolic disturbance + fever
   -liver disease
   -infection of brain
   -drugs (TCA , cocaine, tramadol)
   -pseudo seizures

Question 18

history and exam of epilepsy

Answer 18

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

Question 19

Exam focus/ findings for epilepsy and diagnosis of epilepsy

Answer 19

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

Question 20

Ix for seizure and Differentials for transient LOC

Answer 20

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

Question 21

Non pharm + pharm management of seizures - generalised - atonic/myoclonic/tonic vs partial

Answer 21

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

Question 22

monitoring/ side effects with anticonvulsants - sodium valproate, lamotrigine, carbamazepine, phenytoin, levetiracetam, topiramate

Answer 22

1. SV - highest teratogenecity, pancreatitis, hepatic failure,
   - interact with other anticonvulsants, tca, warfarin, aspirin
2. 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
3. lamotrigine - safe in pregnancy <200mg. SE - allergic rash with increased dose - affected by other anticonvulsant (increased
4. phenytoin - worsen absence, myoclonic allergic rash, hirsuitism, hepatotoxicity - induces enzyme - E2, P2 ,warfarin, TCA, CCB, -
   - therapeutic drug monitoring
5. Topiramate - adjunctive therapy ataxia, weight loss, acute angle glaucoma, kidney stone, cognitive impairment
   - affect digoxin and cocp increase -

Question 23

What are the presentation and risk factors for stroke - ischaemic and haemorrhagic

Answer 23

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

Question 24

What are the investigations and when do you do them/ what is acute management of stroke - timing aims, inclusion/ exclusion criteria

Answer 24

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

Question 25

What is the NIH Stroke scale

Answer 25

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

Question 26

What are the aftercares after stroke - ischaemic vs haemorrhagic

Answer 26

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

Question 27

Primary and secondary prevention + complications of stroke

Answer 27

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

Question 28

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

Answer 28

• 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)

Question 29

Ix and management of charcot marie tooth

Progress
- Doesn’t affect lifespan, slow progression

Answer 29

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)

Question 30

hx and exam findings of different stroke territories
- ICA , ACA, MCA, (l and r).

Answer 30

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

Question 31

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).

Answer 31

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

Question 32

Parkinsons presentation/hx and risk factors

Answer 32

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

Question 33

Exam findings in parkinsons

Answer 33

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

Question 34

Ddx in parkinsons

Answer 34

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)

Question 35

IX in parkinsons and dx

Answer 35

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

Question 36

Management of parkinsons - pharm non pharm, SE - compare with word doc

Answer 36

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

Question 37

Complications of parkinsons specific to neuro/ psych effects and the treatment of these

Answer 37

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

Question 38

Parkinsons disease complications sleep, gi and postural hptn (3) + treatment

Answer 38

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

Question 39

hx and exam findings of different stroke territories
posterior circulation. vertebrobasilar arterial system, pons, medulla, cerebellum

Answer 39

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

Question 40

MN disease - pathophys, what are the differentiating factors,

Answer 40

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

Question 41

What are the features favouring myopathy(timing, distribution, exam finding)

How would you investigate myopathy

Answer 41

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)

Question 42

What is the heritability, age of onset, pathophys and presentation of facioscapulohumeral muscular dystrophy

Answer 42

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

Question 43

What is the heritability, age of onset, pathophys and presentation of Becker’s muscular dystrophy

Answer 43

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

Question 44

List 9 causes of peripheral neuropathies

Answer 44

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

Question 45

How to differentiate sensory , motor, cranial nerves or autonomic neuropathy

Answer 45

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.

Question 46

What are the ix and management of peripheral neuopathy

Answer 46

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

Question 47

Management of MN disease

Answer 47

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