Neuro: MS

Question 1

What is multiple sclerosis?

Answer 1

Chronic and progressive condition that involves demylination of the myelinated neurones in the central nervous system.
his is caused by an inflammatory process inolving the acitvation of immune cells against the myelin.

Question 2

Which patients are typicall effected by MS

Answer 2

Women under 50

Question 3

When might women specifically see an improvement in MS symptoms?

Answer 3

During pregnancy and the post partum period

Question 4

What is myelin?

Answer 4

Myelin covers the axons of neurones in the central nervous system.
This myelin helps the electrical impulse move faster along the axon.
Myelin is provided by cells that wrap themselves around the axons.

Question 5

Which cells provide myelin?

Answer 5

Schwann cells - PNS

Oligodendrocytes - CNS

Question 6

Which system does MS effect and therefore which cells?

Answer 6

CNS - the oligodendrocytes

Question 7

Pathophysiology of MS

Answer 7

There is inflammation around myelin and infiltration of immune cells that cause damage to the myelin. This affects the way electrical signals travel along the nerve leading to the symptoms of multiple sclerosis.

Question 8

What is the pathophysiology behind MS remission?

Answer 8

In early disease, re-myelination can occur and symptoms can resolve. In the later stages of the disease, re-myelination is incomplete and symptoms gradually become more permanent.

Question 9

What happens during an MS ‘‘attack’’?

Answer 9

Lesions of demyelination occuring throughout the CNS

Specific lesions may cause symptoms (ie. optic neuritis) whilst others do not

Question 10

Characteristic feature of MS lesions?

Answer 10

Variation in location over time, meaning that different nerves are affected and symptoms change over time
- ‘disseminated in time and space’

Question 11

Factors that may influence the cause of dyemilination in MS?

Answer 11

Multiple genes
EBV
Low vitamin D
Smoking
Obesity

Question 12

Over what time period do MS symptoms usually progress?

Answer 12

More than 24 hours

Question 13

Signs and symptoms in MS?

Answer 13

Optic neuritis
Eye movement abnormalities
Focal weakness (bells palsy, horners syndrome, limb paralysis, incontinence)
Focal sensory symptoms (trigeminal neuralgia, numbness, paaesthesia, Lhermitte’s sign)
Ataxia (sensory, cerbellar)

Question 14

Lesions in the sixth cranial nerve may cause which two disorders?

Answer 14

Internuclear ophtalmoplegia - unilateral lesions

Conjugate lateral gaze disorder - bilateral lesions

Question 15

What is internuclear opthalmoplegia?

Answer 15

Unilateral lesions in the sixth nerve causes a condition called internuclear ophthalmoplegia.

Internuclear refers to the nerve fibres that connect between the cranial nerve nuclei that control eye movements (3rd, 4th and 6th cranial nerve nuclei).

The internuclear nerve fibres are responsible for coordinating the eye movements to ensure the eyes move together.

Ophthalmoplegia means a problem with the muscles around the eye.

Question 16

What is conjugate lateral gaze disorder?

Answer 16

Lesions in the sixth cranial nerve cause a conjugate lateral gaze disorder.

Conjugate means connected. Lateral gaze is where both eyes move together to look laterally to the left or right. It is disordered in a sixth cranial nerve palsy.

When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion affecting the left eye, when looking to the left, the right eye will adduct (move towards the nose) and the left eye will remain in the middle as the muscle responsible for making it move laterally is not functioning.

Question 17

What is Lhermitte’s sign?

Answer 17

An electrical shock sensation that travels down the spinal and into the limbs when flexing the neck

It indicates disease in the cervical spinal cord in the dorsal column.

It is caused by stretching the demyelinated dorsal column.

Question 18

What is ataxia?

Answer 18

Discoordinated movement

Question 19

What ins sensory ataxia?

Answer 19

Sensory ataxia is the result of loss of the proprioceptive sense, which is the ability to sense the position of the joint (e.g. is the joint flexed or extended).

This results in a positive Romberg’s test and can cause pseudoathetosis.

Question 20

What is cerebellar ataxia?

Answer 20

Cerebellar ataxia is the result of problems with the cerebellum coordinating movement. This suggests cerebellar lesions.

Question 21

What are the possible disease patterns in multiple sclerosis?

Answer 21

Clinically isolated syndrome
Relapsing-remitting
Secondary progressive
Primary progressive

Question 22

What is clinically isolated syndrome in terms of MS?

Answer 22

First episode of demyelination and neurological signs and symptoms
Not diagnostic of MS as lesions have not ben disseminated in time and spcae
These patients may never have another episode or develop MS
Pts with lesions seen on MRI are more likely to go on and develop MS

Question 23

What is relapsing-remiting MS?

Answer 23

Most common pattern at initial diagnosis
Characterised by episodes of disease and neurological symptoms followed by recovery
Different areas, episodes
Can be further classified as active, not active, worsening and nor worsening

Question 24

What is ‘active MS’?

Answer 24

New symptoms are developing or new lesions are appearing on MRI

Question 25

What is 'not active' MS?

Answer 25

No new symptoms or MRI lesions are developing

Question 26

What is worsening MS?

Answer 26

Overall worsening of disease over time (regardless of relapses)

Question 27

What is not progressing MS?

Answer 27

No worsening of disease over time

Question 28

What is secondary progressive MS?

Answer 28

Secondary progressive MS is where there was relapsing-remitting disease at first, but now there is a progressive worsening of symptoms with incomplete remissions. Symptoms become more and more permanent. Secondary progressive MS can be further classified based on whether the disease is active and/or progressing.

Question 29

What is primary progressive MS?

Answer 29

Primary progressive MS is where there is a worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions. This can be further classified in a similar way to secondary progressive based on whether it is active and/or progressing.

Question 30

How is MS diagnosed?

Answer 30

Diagnosis is made by a neurologist based on the clinical picture and symptoms suggesting lesions that change location over time. Symptoms have to be progressive over a period of 1 year to diagnose primary progressive MS. Other causes for the symptoms need to be excluded. Supportive investigations: MRI scans can demonstrate typical lesions Lumbar puncture can detect ''oligoclonal bands'' in the cerborospinal fluid (CSF)

Question 31

How does optic neuritis present and what are the key features?

Answer 31

Unilateral reduced vision developing over hours to days: - Central scotoma - this is an enlarged blind spot - Pain of eye movement - Impaired colour vision - Relative afferent pupillary defect

Question 32

Causes of optic neuritis?

Answer 32

``` MS Sarcoidosis SLE Diabetes Syphilis Measles Mumps Lyme disease ```

Question 33

How should optic neuritis?

Answer 33

Patients presenting with acute loss of vision should be seen urgently by an ophthalmologist for assessment. It is treated with steroids and recovery takes 2-6 weeks. Around 50% of patients with a single episode of optic neuritis will go on to develop MS over the next 15 years. Changes on an MRI scan help to predict which patients will go on to develop MS.

Question 34

How is MS managed?

Answer 34

MDT: physio, neurologists, CNS, OT Disease modifying drugs Relapse management - steroids Symptomatic treatments: Spacisity: baclofen, gabaPentin, physio Neuropathic pain: amitriptyline, gabapentin Depression: SSRIs, CBT, TCA Incontinence: oxybutynin,, indwelling catheter, pelvic floor exercises, percutaneous tibial nerve stimulation Fatigue: CBT, CVS exercise, management of other symptoms to improve sleep Paroxysmal symtpoms: carbamazepine, gabapentin, levetiracetam, amitriptyline

Question 35

Treatment of MS - disease modifying drugs?

Answer 35

Aim of treatment is to induce remission with no evidence of disease activity Target various mechanisms: interlukins, inflammatory cytokines and various immune cells

Question 36

Treating relapses of MS?

Answer 36

Methyprednisolone: 500mg orally daily for 5 days or 1g IV daily for 3-5 days where oral treatment has failed previously or where relapses are severe

Question 37

Symptomatic treatments of MS?

Answer 37

Exercise to maintain activity and strength Neuropathic pain - amitriptyline or gabapentin Depression - SSRIs, CBT Urge incontinence - oxybutynin (caution as my worse cognitive impairment) Spacisity: baclofen, gabapentin and physiotherapy

Question 38

Management of trigeminal neuralgia?

Answer 38

``` Baclofen (Lioresal) Carbamazepine (Tegretol) - FIRST LINE Gabapentin (Neurontin) Lamotrigine (Lamictal) Oxcarbazepine (Trileptal) Pregabalin (Lyrica) ```

Question 39

DMTs that may be used to manage relapsing remmiting MS?

Answer 39

First-line injectables such as beta-interferon and glatiramer New oral agents such as dimethyl fumarate, teriflunomide and fingolimod Biologics such as natalizumab and alemtuzumab.

Question 40

Pregnancy in MS?

Answer 40

Risk of relapse increases 6 months post partum due to hormonal changes Many DMTs not compatible with pregnancy/breastfeeding and may require a washout periord Some DMTs safter than others DMTs should not be delayed due to family planning as this could lead to irreversable disability later in life

Question 41

MS clinical modes of presentation?

Answer 41

1. Optic neuritis 2. Double vision 3. Sensory disturbance in face or limbs 4. Weakness due to upper motor neurone involvement 5. Impaired balance or clumsiness of the limbs 6. Neuropathic pain (e.g. trigeminal neuralgia) 7. Bladder symptoms 8. Cognitive impairment (although this usually occurs late)

Question 42

McDonald criteria for diagnosing MS.

Answer 42

1. Lesions consistent with an inflammatory process 2. No alternative diagnosis 3. Multiple lesions in time and space (Relapsing remitting MS) 4. Progressive neurological deterioration for 1 year (Primary progressive MS)

Question 43

Investigations to consider in ?MS

Answer 43

1. MRI of brain or spinal cord - will demonstrate demyelination plaques 2. Immunoelectrophoresis of CSF - will show oligoclonal bands of IgG