Neurology - Multiple sclerosis Flashcards

1
Q

What is multiple sclerosis

A

Chronic progressive demyelination of the neurones in the central nervous system

Caused by an inflammatory process involving the activation of immune cells against the myelin.

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2
Q

Pathophysiology of MS in more detail

A

Typically only the central nervous system (oligodendrocytes)

There is inflammation around the myelin and infiltration of immune cells that damage the myelin

This affects electrical signal transmission and results in symptoms

Lesions vary in their location over time - dissemination in time and space

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3
Q

What is the function of oligodendrocytes?

A

Produce myelin to wrap around the axons

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4
Q

Causes of MS

A

Cause of demyelination is unclear but it may be influenced by a combination of:

  • Multiple genes
  • EBV
  • Low vitamin D
  • Smoking
  • Obesity
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5
Q

Is demyelination in MS permanent?

A

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.

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6
Q

Signs and symptoms of MS - broad

A

Optic neuritis - most common

Eye movement abnormalities

Focal weakness

Focal sensory symptoms

Ataxia

Depression/labile mood

Speech/swallowing difficulties

Bowel/bladder dysfunction

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7
Q

What is the presentation of optic neuritis?

A

Unilateral reduced vision over hours to days

Central scotoma (enlarged blind spot)
Pain on eye movement
Impaired colour vision
RAPD

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8
Q

What things other than MS can cause optic neuritis?

A
Sarcoidosis
SLE
Diabetes
Syphilis
Measles
Mumps
Lyme disease
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9
Q

How is optic neuritis treated?

A

Steroids

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10
Q

What eye movement abnormalities can occur in MS?

A

6th nerve lesions can occur - Can cause double vision

Unilateral lesions can cause intranuclear ophthalmoplegia

Lesions can also cause conjugate lateral gaze disorders

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11
Q

What is intranuclear ophthalmoplegia

A

Internuclear refers to the nerve fibres that connect between the cranial nerve nuclei that control eye movements (3rd, 4th and 6th cranial nerve nuclei). This is an interneuron called the medial longitudinal fasciculus (MLF).

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.

INO is a conjugate lateral gaze palsy

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12
Q

What is conjugate lateral gaze palsy?

A

Inability of both the eyes to look together to the side

In the affected eye the eye will not abduct, whereas the unaffected eye will adduct to the nose

Nystagmus is usually observed with the abduction of the contralateral eye, as it tries to normalise the two discordant images being sent to the brain simultaneously

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13
Q

What focal weakness problems can occur?

A

Bells Palsy

Horners syndrome

Limb paralysis

Incontinence

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14
Q

What focal sensory symptoms can occur?

A

Trigeminal neuralgia

Numbness

Paraesthesia (pins and needles)

Lhermitte’s sign

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15
Q

What is Lhermitte’s sign?

A

An electric shock sensation that travels down the spine 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.

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16
Q

What are the types of ataxia?

A

Sensory - loss of proprioceptive sense, resulting in positive Romberg’s test

Cerebellar - problems with the cerebellum coordinating movement

17
Q

Most common symptoms on initial presentation

A

Limb numbness/tingling
Limb weakness (subacute onset)
Cerebellar symptoms
Optic neuritis

18
Q

Central symptoms of MS

A
  • Motor - loss of power/spacticity
  • Sensory e.g. tingling, parasthesia
  • Cerebellar - tremor, balance issues
  • Fatigue
  • Depression/labile mood
19
Q

Ophthalmic symptoms of MS

A

Nystagmus

Optic neuritis

Diplopia - internuclear ophthalmoplegia

20
Q

Otolaryngeal symptoms of MS

A

Dysphagia

Slurring/stuttering speech

21
Q

Urinary and GI symptoms of MS

A

Urinary frequency, incontinence, retention

Constipation, diarrhoea

22
Q

Important things to ask in the history of MS - other than HPC?

A

Past medical history (such as any history of focal neurologic deficit, or other autoimmune diseases)

Family history of MS/other autoimmune diseases

Smoking status
Diet
Impact on activities of daily living
Driving status
Falls risk assessment
23
Q

Examination in suspected MS

A

Full neurological examination including CN exam and cerebellar examination

24
Q

Lesions in these cranial nerves may cause

A

CN2 - optic neuritis, RAPD

CN3,4,6 - related eye movement abnormalities or INO

CN5 - facial paraesthesia

CN7 - weakness in facial muscles - can mimic stroke

CN8 - balance loss, sensorineural hearing loss

CN9,10,12 - loss of motor function to tongue/pharynx resulting in speech and swallowing problems

CN11 - Loss of motor function to sternocleidomastoid and trapezius resulting in neck weakness and hypertonia.

25
Q

Cerebella signs of MS

A

Nystagmus - towards the side of the lesion

Intention tremor

Scanning dysarthria - sentences or even words are broken up into a number of separate syllables that can be expressed at varying volume. It may appear as though the patient is searching for (or scanning for) the correct next word/sound.

26
Q

Risk factors for MS

A

Family history

Sex (F>M)

Age between 25-35

Other co-existing autoimmune diseases, such as type 1 diabetes mellitus

Smoking

Previous EBV infection; infectious mononucleosis

Vitamin D deficiency

27
Q

What are some phenomena that can occur with MS?

A

Uhtoff’s - worsening of neurological symptoms on exercise/in warm environments (e.g. in a bath) e.g. worsening vision

Lhermitte’s phenomenon: lightning-shock pain down the spine on flexion of the neck secondary to cervical cord plaque formation.

28
Q

Differential diagnosis

A

Broad due to the varied MS presentations

Spinal cord/brainstem compression e.g. cervical spondylosis, Chiari malformation, disc herniation

Peripheral neuropathy e.g. B12 deficiency, diabetic, MND

Guillain-Barre syndrome

Stroke

Space occupying lesion

29
Q

Investigations in suspected MS

A

Bloods and lab investigations to rule out other pathology:

  • FBC, CRP, LFTs, U&Es (to rule out infection, electrolyte imbalance)
  • TFTs - to rule out hypothyroid
  • Calcium/ACE - to rule out sarcoidosis
  • Vitamin B12
  • HIV serology?

MRI brain and spine with gadolinium contrast is the main investigation - will show high signal T2 white matter plaques

LP - high protein content and oligoclonal bands of immunoglobulin aggregates on CSF electrophoresis

30
Q

Types of MS and description of each

A

Relapsing-remitting - episodes of disease followed by recovery

Secondary progressive -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

Primary progressive - worsening of disease and neurological symptoms from the point of diagnosis without initial relapses and remissions

31
Q

Management of MS - relapse treatment

A

Acute relapse - high dose methylprednisolone can be given to shorten relapse for 5 days

  • 500mg orally daily for 5 days
  • 1g intravenously daily for 3–5 days where oral treatment has failed previously or where relapses are severe
32
Q

Management of MS generally

A

MDT approach :

  • Neurologists, specialist nurses
  • SALT for speech/swallow problems
  • OT - any home modifications/aids
  • Physio - rehabilitation of balance, strength, mobility difficulties

Disease modifying drugs and biologic therapy to aim for long term remission with no evidence of disease activity

  • Disease modifiers e.g. interferon-beta, glatiramer
  • Biologics e.g. monoclonal antibodies e.g. alemtuzumab and natalizumab

For problems with spasticity:

  • Baclofen or gabapentin are first line
  • Dantrolene in severe renal failure

Treat:

  • neuropathic pain e.g. amitriptyline/gabapentin,
  • depression e.g. CBT, SSRIs
  • Urge incontinence - ACh medications e.g. oxybutynin

Also exercise to maintain activity and strength