1
Q
Pathogenesis of MS
Cells Effected in MS
A
Multifactorial
- Inflammation
- Demyelination
- Axonal degeneration
Mostly effecting Oligodendracyte
2
Q
Diagnosis of MS
A
MacDonald Criteria
- Separation of Time
- >2 clinical neurological events @ different time intervals
- Separated on MRI gadolinium scan - Separation of Space
- >2 different clinical neurological features
- >2 lesions on MRI in deferent areas
3
Q
Subtypes of MS
A
- Relapsing and remitting
- 90%
- Return to some residual deficit
- F > M - Secondary progressive
- Primary Progressive
- 10%
- M > F - Progressive Relapse
4
Q
Treatment of MS
- limited head to head studies
A
Interferon
- monitor LFTs
- Flu like symptoms
#
5
Q
Dx of MS
A
oligoclonal bands in the cerebrospinal fluid
6
Q
syringomyelia
A
Cyst in the middle of spinal cord
White T2 Image
7
Q
GBS
A
Campylobacter jejuni
8
Q
Aetiology of epilepsy
A
- Genetic: direct result of a known or presumed genetic defect that is the core symptom of the disor-der
- Structural or metabolic: structural or metabolic condition or disease that has demonstrated to be associated with a substantially increased risk of developing epilepsy
- Gentic: tuberous sclerosis or malformations of cortical development
- Aquired: stroke, trauma or infection
- Autoimmune
9
Q
Epilepsy in elderly
Aetiology
A
- CVA
- Dementia
- Intracranial tumors
- Unknown one-third to half
10
Q
Dystonia
A
- Sustained or intermittent muscle contraction causing abnormal movements and/ or postures
- Often repetitive
- Twisting pattern
- Worsened by voluntary action
11
Q
Chorea
A
- Involuntary movements limbs, trunk, neck or face; which rapidly flit from region to region Irregular pattern, unpreductable manner ‘no sense of abnormal muscle contraction’
- Not repetitive
- ?caudate nucleus & putamen
12
Q
Myoclonus
A
Fast hyperkinetic disorder
Sudden increase in muscle tone
Focal, multifocal, segmental or generalised
13
Q
Dyskinesia
A
Overactivity of movement
ie. Over treatment in Parkinsons (motor fluctuation)
14
Q
Resting tremor
A
When the limbs are completely at rest
15
Q
Postural tremor: when the arms are held up by the patient
A
- Kinetic tremor: during movement, may significantly worsen toward the end of
movement - Intention: coarse terminal tremor when limb approaches a target
- Action tremor: postural + kinetic
16
Q
Essential tremor
A
- Bilateral, mildly asymmetrical involving the hands and forearms
- May have concomitant head and jaw tremor
- Can be at rest but posture predominant
- Slow progression
- Begins 60’s
- ETOH improves
Family history - 30-50% familial form
Treatment (May not require treatment) - First line: propranolol & primidone
- Second line: clonazepam, topiramte, gabapentin and nimodipine
- Others: botulinum toxin & DBS
17
Q
Physiologic tremor
A
- Bilateral symmetrical
- Onset any age
- Worsened by anxiety & fatigue
- No family history
- Possible identification of enhancing cause
18
Q
Dystonic tremor
A
- Irregular and jerky oscillatory
- Other signs of dystonia
19
Q
Phenytoin
A
- 90% bound to albumin
- 10% free
- Free is important to levels - active
- only need to change dose if symptomatic
20
Q
Stroke management
A
1.
21
Q
Migraine
A
# acute: triptan + NSAID or triptan + paracetamol # prophylaxis: topiramate or propranolol - if 2 or more attacks per month
22
Q
Visual field defects:
A
- left homonymous hemianopia means visual field defect to the left, i.e. lesion of right optic tract
homonymous quadrantanopias: PITS (Parietal-Inferior, Temporal-Superior) - incongruous defects = optic tract lesion; congruous defects= optic radiation lesion or occipital cortex
23
Q
Homonymous hemianopia
A
- incongruous defects: lesion of optic tract
- congruous defects: lesion of optic radiation or occipital cortex
- macula sparing: lesion of occipital cortex
24
Q
Homonymous quadrantanopias*
A
- superior: lesion of temporal lobe
- inferior: lesion of parietal lobe
- mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
25
Bitemporal hemianopia
- lesion of optic chiasm
- upper quadrant defect > lower quadrant defect = inferior - chiasmal compression, commonly a pituitary tumour
- lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
26
Cataplexy
sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy.
27
Eclampsia
- condition seen after 20 weeks gestation
- pregnancy-induced hypertension
- proteinuria
Mx
- IV Magnesium Sulphate (to prevent and treat seizures
- Fluid restriction - prevent fluid overload
28
Bells Palsy
- LMN - affects forehead
- Often associated with pain around the ear (Ramsey-Hunt syndrome), altered taste, dry eyes, hyperacusis
Mx
- Prednisalone
- Aciclovir adds no benefit
Note UMN (Stroke) - spares forhead
29
Myasthenia gravis
- The key feature is muscle fatigability
- Antibodies to acetylcholine receptors are seen in 85-90% of cases*
Management
- long-acting anticholinesterase e.g. pyridostigmine
- immunosuppression: prednisolone initially
- thymectomy
Management of myasthenic crisis
- plasmapheresis
- intravenous immunoglobulins
30
Guillain-Barre syndrome
- immune mediated demyelination of the peripheral nervous system
- Often triggered by an infection (classically Campylobacter jejuni).
Management
- Plasma exchange
- IVIG - as effective as plasma exchange
- No benefit in combining treatments
- steroids and immunosuppressants have not been shown to be beneficial
- FVC regularly to monitor respiratory function
Prognosis
- 20% suffer permanent disability, 5% die
31
Subdural haemorrhage
Basics
- most commonly secondary to trauma e.g. old person/alcohol falling over
- initial injury may be minor and is often forgotten
- caused by bleeding from damaged bridging veins between cortex and venous sinuses
Features
- headache
- classically fluctuating conscious level
- raised ICP
Treatment
- needs neurosurgical review ? burr hole
32
Epilepsy medication:
first-line
- generalised seizure: sodium valproate
- partial seizure: carbamazepine
33
Tuberous sclerosis (TS)
- is a genetic condition of autosomal dominant inheritance.
| - Like neurofibromatosis, the majority of features seen in TS are neuro-cutaneous
34
Dopamine receptor agonists
- e.g. Bromocriptine, ropinirole, cabergoline, apomorphine
35
Progressive supranuclear palsy
- parkinsonism
- impairment of vertical gaze
Management
- poor response to L-dopa
36
Idiopathic intracranial hypertension
Classically
- Obese
- young female with headaches / blurred vision
37
cholinesterase inhibitors
- Only role is to improve some cognitive function and improvement in activities of daily living.
- There is no role for cholinesterase inhibitors in advanced Alzheimer's disease.
38
Tremor
- Difficulty in initiating movement (bradykinesia), postural instability and unilateral symptoms (initially) are typical of Parkinson's.
- Essential tremor symptoms are usually eased by alcohol. - Mx by propranalol
39
acoustic neuroma
Classically
- Loss of corneal reflex
- Hearing loss
40
Motor neurone disease
| amyotrophic lateral sclerosis
- mixture of upper and lower motor neurone signs
- normal sensation
C9ORF72 is associated with an autosomal dominant inheritance of motor neurone disease and frontotemporal dementia
Mx (Riluzole)
- prevents stimulation of glutamate receptors
- prolongs life by about 3 months
FXN is the gene for Friedreich Ataxia
41
Vertigo
- Viral labyrinthitis typically causes constant symptoms of a shorter duration.
- Meniere disease usually have associated hearing loss and tinnitus. Also, the vertigo associated with Meniere disease typically lasts much longer
42
Normal pressure hydrocephalus
Classically
| - Urinary incontinence + gait abnormality + dementia
43
Von Hippel-Lindau syndrome
- Retinal and cerebellar haemangiomas are key features
| - Retinal haemangiomas are bilateral in 25% of patients and may lead to vitreous haemorrhage
44
Normal pressure hydrocephalus
Classical triad
- urinary incontinence
- dementia and bradyphrenia
- gait abnormality (may be similar to Parkinson's disease)
45
Neglect in Stroke
often contralateral stroke of parietal lobe
46
Medication to improve severe motor deficits post stroke
FLAME Trial - The Lancet Neurology. 2011. 10(2):123-130
- fluoxetine
- initiated 5 to 10 days after stroke onset
- in addition to conventional physiotherapy
- improves motor recovery in patients with moderate to severe motor deficits due to ischemic stroke.
47
Meniere disease
Triad
- episodic vertigo
- sensisorineural hearing loss
- tinnitus
RACP
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