Multiple Sclerosis

Question 1

What is Multiple Sclerosis?

Answer 1

A degenerative condition characterised by multiple episodes of CNS lesions, disseminated in time and space, not explained by anything else

Question 2

What are the risk factors for developing MS?

Answer 2

• Lack of Vitamin D3
• Living in the northern hemisphere
• Genetic link
• Viral link (EBV)
• Smoking

Question 3

How does the epidemiology of MS vary between males and females?

Answer 3

Females have a higher incidence (2:1) but males have more destructive lesions

Question 4

What are the common patterns of disease?

Answer 4

• Relapsing-remitting (85%) leading to secondary progressive

- Primary progressive (15%)

Question 5

Name some differential diagnoses of monophasic MS

Answer 5

Isolated optic neuritis, transverse myelitis or ADEM (acute disseminated encephalomyelitis)

Question 6

Name some differential diagnoses of relapsing remitting MS

Answer 6

SLE, sarcoid, multiple strokes, Hughe’s syndrome

Question 7

Name some differential diagnoses of chronic progressive MS

Answer 7

Metabolic (B12, thyroid, mitochondrial disease)
Structural (cervical spondylosis)
Infections (HIV, Lyme, syphilis)

Question 8

What is the pathophysiology of MS?

Answer 8

An initial trigger starts an autoimmune reaction –> inflammation leads to defective BBB –> activated T cells enter CNS –> clones and proliferates, releasing gamma and toxic cytokines –> destruction of myelin sheath

In RR MS remyelination can occur

Question 9

What is the difference in the early presentation of RR and PP MS?

Answer 9

PP - more lesions on spinal cord, preserved cognition

RR - more lesions on brain, cognition deteriorates

Question 10

How is suspected MS investigated?

Answer 10

Bedside: examination
Bloods
Imaging: MRI
Special Tests: LP

Question 11

What can be seen on MRI?

Answer 11

Periventricular and juxtacortical lesions

Persistent black holes = irreperable axonal loss

Brain atrophy

Question 12

What can be seen on LP?

Answer 12

Oligoclonal bands - these represent the clumping of antibodies together (during electrophoresis)
Abnormal protein (left over from inflammation)

Question 13

What constitutes an MS attack?

Answer 13

Previous diagnosis of MS, could present with any symptoms as long as they last over 24 hours in the absence of a fever or other explanation

Question 14

What is optic neuritis?

Answer 14

Unilateral visual loss, desaturation and pain on movement, due to swelling
Onset: hours-days
Recovery: days-weeks

Question 15

What is transverse myelitis?

Answer 15

Inflammation of the spinal cord causing upper motor neurone weakness (below level of attack, with numbness and bowel and bladder disturbance
Onset: days
Recovery: weeks-months (if not recovered over 6 months, likely to be permanent)

Question 16

What brainstem attacks can occur in an MS flare up?

Answer 16

Double vision, facial palsy, facial numbness, vertigo, dysarthria, ataxia, tremor

Question 17

How should MS relapses be treated?

Answer 17

Anti-emetics and steroids

NB - steroids are only good for fresh inflammatory activity; they dont affect disease progression or outcome

Question 18

What paroxysmal symptoms can occur in an MS flare up?

Answer 18

Lhermittes phenomenon 
Trigeminal neuralgia
Tonic spasms
Epilepsy
Uthoffs phenomenon

Question 19

What us Lhermittes phenomenon?

Answer 19

When putting your chin to your chest evokes an electrical shock
- seen in MS attack

Question 20

What is Uthoffes phenomenon?

Answer 20

Inability to function in heat due to axon exhaustion (e.g. can’t get out of a hot bath)
- seen in MS attack

Question 21

What members of the MDT are involved in the care of an MS patient?

Answer 21

Neurologist, specialist nurse, GP, voluntary, therapists, rehab/disability services, OT, PT, social services

Question 22

What is the lifetime prevalence of depression with MS?

Answer 22

50%

Question 23

What are the main symptoms of chronic MS?

Answer 23

Fatigue, mood changes, memory changes, visual changes, swallowing/speech, arms, arm and leg weakness, bowel and bladder disturbance, erectile dysfunction, numbness/tingling/pain, spasticity, ambulation limitations

Question 24

What conditions can lead to death in MS patients?

Answer 24

• Aspiration pneumonia from swallowing difficulties
• UTIs from bladder problems
• LRTI/URTI from weak rest muscles
• Falls from instability

Question 25

How can stem cells be used in the treatment of MS?

Answer 25

Umbilical cord progenitors stimulate the presence of Tregs and thus regulate T-cell activity

Question 26

Name some disease modifying therapies

Answer 26

Current:
- Teriflunomide
- Dimethyl fumarate
- Natalizumab
- Alemtuzumab
Going out of use:
- Beta-interferons
- Glatiramer Acetate

Question 27

What are the guidelines for starting DMT?

Answer 27

Active RR MS (2 or more relapses in previos 2 years) or new lesions on scan

Question 28

What are the pros and cons of Alemtuzamab (monoclonal antibody)?

Answer 28

Pros: stays in system for 5 years, unrivalled efficacy
Cons: expensive, high infection risk, thrombocytopenic purpura, thyroid problems, requires monthly monitoring of bloods

Question 29

What are the pros and cons of Natalizumab (Tysabri)?

Answer 29

Pros: safe in pregnancy
Cons: lifelong monitoring, progressive multifocal leukoencephalopathy (fatal)

Question 30

What is progressive multifocal leukoencephalopathy?

Answer 30

A rare viral brain infection causing inflammation of the white matter (secondary to severe immunosuppresion) .

It is caused by the JC virus, which is harmless except in the case of weakened immune systems

Question 31

What are the symptoms of PML?

Answer 31

Clumsiness, progressive weakness, personality changes and ‘alien hand syndrome’