Multiple Sclerosis Flashcards
Typical onset of an inflammatory cause of weakness
Monosymptomatic (20% of patients present with unilateral optic neuritis)
Symptoms may worsen with heat
Slow, but progressive muscle weakness
Fatigue after walking or standing
Frequent episodes of tripping or falling
Difficulty in swallowing or breathing
Some people develop muscle pain - tender to touch
Aetiology of inflammatory-induced weakness
MS Polymyositis Dermatomyositis Inclusion body myositis Necrotising autoimmune myopathy
What is MS?
Inflammatory plaques of demyelination in the CNS, disseminated in space and time
- i.e. occurring at multiple sites with more than 30 days between attacks
Demyelination heals poorly, causing axonal loss
Incidence increases with latitude (risk is fixed at the place you went through puberty)
Clinical presentations of MS
Monosymptomatic
Optic nerve
- optic neuritis
- optic disc swelling
- RAPD
Spinal cord
- UMN lesions
- numbness, paraesthesia and decreased vibration sense
- incontinence/constipation and sexual dysfunction
Brainstem
- opthalmaplegia
- neuralgia, hearing loss, vertigo, dysphagia and dysphonia
Cerebellum - rare
- epilepsy, trigeminal neuralgia and tonic spasms
Others
- cognitive and visuospatial decline, depression and decreased executive functioning
How is MS diagnosed? - McDonalds criteria
2 or more attacks with 2 or more objective clinical lesions
- no additional tests required
2 or more attacks with 1 objective clinical lesion
- MRI: spatially disseminated lesions or
- positive CSF and 2 MRI lesions or
- second attack at a new site
1 attack with 2 or more objective clinical lesions
- dissemination in time by a new lesion on repeat MRI after 3 months, or a second attack
1 attack with 1 objective clinical lesion
- dissemination in space (MRI, or positive CSF + 2 lesions consistent with MS) - dissemination in time (MRI or second attack)
Insidious neurological progression suggestive of primary progressive MS
- positive CSF AND dissemination in space on MRI OR
- continued progression for one year
Investigations in ?MS
If presentation with 2 or more attacks and 2 or more objective clinical lesions, then no further imaging is required
MRI - can detect lesions disseminated in space and time (2 at different times and different sites required)
CSF
- oligoclonal bands of IgG on electrophoresis that are not present in the serum
- suggests CNS inflammation
Visual evoked potentials (VERS)
- if there has been demyelination at any time along the optic nerve (symptomatic or asymptomatic), the conduction of visual images to the occipital cortex will be delayed
- >100ms
What are the clinical subtypes of MS
Relapsing remitting - most common
Secondary progressive MS
Primary progressive MS
Progressive remitting MS
Describe relapsing remitting MS
Unpredictable attacks that may or may not leave permanent deficits
Followed by periods of remission
Describe secondary progressive MS
Initially relapsing remitting illness that suddenly begins to decline, without periods of remission
Describe primary progressive MS
Steady increase in disability without relapses, from onset
Describe progressive remitting MS
Steady decline since onset, with super-imposed attacks
Define relapse and remission
Relapse - focal inflammation, damage to myelin and oligodendrocites and conduction block
Remission - inflammation subsides and there is remyelination of damaged area
- some can return to normal function
Acute management of MS relapse
IV methyprednisolone for 3-5 days (and PPI) shortens acute relapses
- no change in prognosis
- give no more than twice a year
Physiotherapy and occupational therapy
Long-term management of MS
Lifestyle advice - regular exercise, smoking cessation and avoidance of stress
Disease modifying drugs
Symptom management
Describe the role of disease modifying drugs in MS
Dimethyl fumerate - mild/moderate relapsing remitting MS
Monoclonal antibodies
- alemtuzimab (acts against T-cells)
- netalizumab (acts against VLA-4 receptors that allow immune cells to cross the BBB)
1st line
- beta-interferon and glatiramer (reduce relapse frequency)
- dimethyl fumerate
- teriflunomide suppresses immune system
2nd line
- fingolimol
- natalizumab
3rd line
- alemtuzumab (resets immune system and can eliminate relapses)