Multiple sclerosis

Question 1

What is MS?

Answer 1

Presence of multiple CNS lesions causing symptoms lasting longer than 24 hours with lesions being disseminated in time (> 1 month apart) and space (clinically on MRI)

Question 2

What is a clinically isolated syndrome (CIS)?

Answer 2

First attack of demyelination

Question 3

How common is MS?

Answer 3

120/100,000 prevalence
Approx 80,000 people have it in UK
Lifetime risk 1 in 400
Incidence 7 per 100,000
Women 2x more likely to have it than med
Typically presents between 20 and 40
Much more common in white populations with increasing frequency with increasing distance from equator
Other autoimmune diseases occur with increased frequency in patients and their relatives

Question 4

What are the 4 different types of MS?

Answer 4

Progressive-relapsing MS
Secondary progressive MS
Primary progressive MS
Relapsing-remitting MS

Question 5

What happens in progressive-relapsing MS?

Answer 5

Steady decline since onset with superimposed attacks

Question 6

What happens in secondary progressive MS?

Answer 6

Initially relapsing-remitting MS that suddenly begins to have decline without periods of remission

Question 7

What happens in primary progressive MS?

Answer 7

Steady decrease in disability without attacks

Question 8

What happens in relapsing-remitting MS?

Answer 8

Unpredictable attacks which may or may not leave permanent deficits followed by periods of remission

Question 9

What are the possible causes of MS?

Answer 9

Not fully understood
Polygenic inheritance pattern but increased risk for MS in 1st degree relatives of those affected
Environmental factors - viral infections (EBV), HHV-6, low levels of vit D and lack of sunlight exposure

Question 10

What is the pathology of MS?

Answer 10

Inflammatory - focal myelin loss with relative preservation of axons
Neurodegenerative - axonal loss may contribute to fixed and progressive deficits
Chronic autoimmune, T-cell mediated, inflammatory disorder of CNS
Multiple plaques of demyelination (2-10mm) found throughout brain and spinal cord occurring sporadically over years
Plaques can occur anywhere but most likely to occur in optic nerves, periventricular region, corpus callosum, brainstem, cerebellar connection, cervical cord (corticospinal tracts and posterior columns)

Question 11

How does MS present?

Answer 11

Visual loss (optic neuritis)
Pyramidal weakness, spastic paraparesis
Sensory disturbance - often unusual eg sensation of water trickling down skin, reduced vibration sense and proprioception
Clumsiness
Cerebellar symptoms – nystagmus, vertigo, tremor, ataxia, dysarthria/dysphasia
Bladder involvement/sexual dysfunction
Lhermitte’s and Uhthoff’s phenomenon
o Lhermitte’s – sudden sensation resembling an electric shock passing down back of neck into spine and then radiating out to arms and legs, triggered by bending head towards chest
o Uhthoff’s – temporary worsening of MS symptoms caused by increase in temperature eg hot bath/shower
Fatigue
Cognitive impairment
UMN signs – spastic paraparesis, brisk reflexes
Optic atrophy
Relative afferent pupillary defect – pupils respond differently to light stimuli shone in one eye at a time due to unilateral or asymmetrical disease of retina or optic nerve, on good eye, both pupils will react, on bad eye, neither pupil will respond
Internuclear ophthalmoplegia (decreased adduction of ipsilateral eye, nystagmus of abduction of contralateral eye)
Diplopia
Urinary symptoms – bladder hyper-reflexia causing urinary urgency/frequency
Neuropathic pain
Depression
Sexual dysfunction
Temperature sensitivity

Question 12

What investigations should you do in MS?

Answer 12

MRI - lesions of high T2 signal intensity in white matter of brain, brain stem/cerebellum or spinal cord, typically periventriuclar
LP - oligoclonal bands
Evoked potentials eg visual evoked responses
Bloods to exclude other inflammatory disorder such as sarcoidosis or SLE

Question 13

How do you treat an acute relapse of MS?

Answer 13

Steroids - first check no infection before commencing as infections can cause relapse
Don’t alter trajectory but hasten recovery
Methylprednisolone 1g/day for 3 day IV

Question 14

How do you prevent relapses?

Answer 14

Disease modifying therapies
SC - beta interferon, glatimar acetate
IV - ocrelizumab, natalizumab
Oral - fingolimod

Question 15

What else should you do in MS treatment?

Answer 15

Education
Support from MDT including MS nurses
PT and OT
Symptomatic treatments

Question 16

What immune cells are specific to MS?

Answer 16

Th17 cells

Question 17

What is the definition of an MS relapse?

Answer 17

Relapse has to be experienced by patient
May be able to be confirmed by examination
> 48 hrs

Question 18

What is the McDonald criteria?

Answer 18

Definition of MS for diagnosis
An inflammatory demyelination disease of CNS where there is
- Dissemination in time
- Dissemination in space
- No alternative neurological disease that may explain S&S
Clinical diagnosis
Need at least 1 episode of typical relapse

Question 19

What supportive evidence can help with diagnosis?

Answer 19

Periventricular lesions on MRI
Juxtacortical lesions
Callosal lesions
Dawson’s fingers
Posterior fossa and brainstem (ipsilateral hemiataxia)
Temporal lobe (often not affected in other conditions)
Optic nerve