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Yr 4 - CCC > Neurology: Multiple Sclerosis & MND > Flashcards

Neurology: Multiple Sclerosis & MND Flashcards

1
Q

What is multiple sclerosis (MS)?

A

A chronic and progressive autoimmune condition involving demyelination in the central nervous system.

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2
Q

Pathophysiology of MS?

A

The immune system attacks the myelin sheath of the myelinated neurones.

Inflammation and immune cell infiltration cause damage to the myelin, affecting the electrical signals moving along the neurones.

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3
Q

What is myelin?

A

Myelin covers the axons of neurones and helps electrical impulses travel faster.

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4
Q

What produces myelin in the:
a) CNS
b) PNS

A

a) oligodendrocytes
b) Schwann cells

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5
Q

Does MS typically affect men or women more?

A

Women (3x)

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6
Q

What age does MS typically affect?

A

Multiple sclerosis typically presents in young adults (under 50 years)

most commonly diagnosed in people aged 20-40 years

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7
Q

Does MS affect the CNS or the PNS?

A

The CNS (i.e. the oligodendrocytes)

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8
Q

In which locations is MS more common?

A

much more common at higher latitudes (5 times more common than in tropics)

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9
Q

What are the 3 subtypes of MS?

A

1) Relapsing-remitting disease

2) Secondary progressive disease

3) Primary progressive disease

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10
Q

What is the most common type of MS?

A

Relapsing remitting disease

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11
Q

What happens in relapsing remitting MS?

A

acute attacks (e.g. last 1-2 months) followed by periods of remission

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12
Q

In early MS, remyelination can occur. What happens here?

A

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

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13
Q

What is 2ary progressive MS?

A

Describes relapsing-remitting patients who have DETERIORATED and have developed neurological signs and symptoms between relapses.

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14
Q

Does relapsing remitting MS always progress to 2ary progressive MS?

A

Around 65% of patients with relapsing-remitting disease go on to develop secondary progressive disease within 15 years of diagnosis

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15
Q

What is 1ary progressive MS?

A
  • accounts for 10% of patients
  • progressive deterioration from onset
  • more common in older people
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16
Q

Causes of MS?

A

The cause of the multiple sclerosis is unclear, but there is growing evidence that it may be influenced by:

  • Multiple genes
  • Epstein–Barr virus (EBV)
  • Low vitamin D
  • Smoking
  • Obesity
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17
Q

Onset of MS symptoms?

A

Symptoms usually progress over more than 24 hours. Symptoms tend to last days to weeks at the first presentation and then improve.

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18
Q

How does MS present?

A

There are many ways MS can present, depending on the location of the lesions.

A characteristic feature of MS is that lesions vary in location, meaning that the affected sites and symptoms change over time. The lesions are described as “disseminated in time and space”.

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19
Q

Potential features of MS?

A

Non-specific e.g. lethargy (75%)
Visual:
- optic neuritis
- optic atrophy
- Uhthoff’s phenomenon: worsening of vision following rise in body temp
- internuclear ophthalmoplegia

Sensory:
- pins/needles
- numbness
- trigeminal neuralgia
- Lhermitte’s syndrome: paraesthesiae in limbs on neck flexion

Motor:
- spastic weakness: most commonly seen in the legs

Cerebellar:
- ataxia: more often seen during an acute - relapse than as a presenting symptom
tremor

Others:
- urinary incontinence
- sexual dysfunction
- intellectual deterioration

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20
Q

What is Uhthoff’s phenomenon?

A

Worsening of vision following rise in body temperature

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21
Q

What is the most common presentation of MS?

A

Optic neuritis (demyelination of the optic nerve)

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22
Q

How does optic neuritis typically present? Features?

A

Unilateral reduced vision:
- Central scotoma (an enlarged central blind spot)
- Pain with eye movement
- Impaired colour vision
- Relative afferent pupillary defect

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23
Q

Is optic neuritis unilateral or bilateral?

A

Unilateral

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24
Q

What is a relative afferent pupillary defect?

A

Where the pupil in the affected eye constricts more when shining a light in the contralateral eye than when shining it in the affected eye.

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25
Q

Describe the:
a) direct pupillary reflex
b) consensual pupillary reflex

in optic neuritis

A

a) reduced pupil response to shining light in the eye affected by optic neuritis

b) affected eye has a normal pupil response when testing the consensual pupillary reflex

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26
Q

Give some causes of optic neuritis

A

1) MS

2) Sarcoidosis

3) SLE

4) Syphilis

5) Measles or mumps

6) Neuromyelitis optica

7) Lyme disease

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27
Q

Management of optic neuritis?

A

1) Urgent ophthalmology input.

2) High dose steroids.

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28
Q

What imaging can help to predict which patients with optic neuritis will go on to develop MS?

A

MRI

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29
Q

MS can cause eye movement abnormalities.

Give some features of MS causing eye movement abnormalities

A

1) Diplopia & nystagmus (can lead to oscillopsia): caused by lesions to the oculomotor (CN III), trochlear (CN IV) or abducens (CN VI)

2) Internuclear ophthalmoplegia (characterised by impaired adduction of ipsilateral eye with nystagmus of contralateral abducting eye): caused by lesion to medial longitudinal fasciculus

3) Conjugate lateral gaze disorder: caused by lesion in the abducens (CN VI)

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30
Q

Define oscillopsia

A

Refers to the visual sensation of the environment moving and being unable to create a stable image.

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31
Q

Lesions affecting what nerves in MS cause diplopia & nystagmus?

A

Lesions affecting the oculomotor (CN III), trochlear (CN IV) or abducens (CN VI)

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32
Q

What is internuclear ophthalmoplegia in MS caused by?

A

Caused by a lesion in the medial longitudinal fasciculus

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33
Q

What characterises internuclear ophthalmoplegia?

A

1) impaired adduction on ipsilateral eye
2) nystagmus in contralateral abducting eye

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34
Q

What does the medial longitudinal fasciculus connect?

A

The nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei (“internuclear”) that control eye movements (the 3rd, 4th and 6th cranial nerve nuclei).

These fibres are responsible for coordinating the eye movements to ensure the eyes move together.

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35
Q

What is a conjugate lateral gaze disorder?

A

Conjugate means connected.

Lateral gaze is where both eyes move to look laterally to the left or right.

When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion involving 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.

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36
Q

What causes a conjugate lateral gaze disorder in MS?

A

A lesion in the abducens (CN VI)

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37
Q

Multiple sclerosis may present with focal weakness.

Give some examples of this

A

1) Incontinence

2) Horner syndrome

3) Facial nerve palsy

4) Limb paralysis

5) Ataxia

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38
Q

Multiple sclerosis may present with focal sensory symptoms.

Give some examples of this

A

1) Trigeminal neuralgia

2) Numbness

3) Paraesthesia (pins and needles)

4) Lhermitte’s sign

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39
Q

what is Lhermitte’s sign?

A

Describes an electric shock sensation that travels down the spine and into the limbs when flexing the neck.

It is caused by stretching the demyelinated dorsal column.

40
Q

What does Lhermitte’s sign indicate in MS?

A

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

41
Q

What is transverse myelitis in MS?

A

Transverse myelitis refers to a site of inflammation in the spinal cord, which results in sensory and motor symptoms depending on the location of the lesion.

42
Q

What is ataxia?

A

Ataxia is a problem with coordinated movement. It can be sensory or cerebellar.

43
Q

What is sensory ataxia?

A

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

44
Q

What is Romberg’s test?

A

A positive test is an inability to maintain an erect posture over 60 seconds with eyes closed.

45
Q

Describe Romberg’s test in sensory ataxia

A

Positive Romberg’s test (they lose balance when standing with their eyes closed)

46
Q

A lesion where in MS can cause sensory ataxia?

A

In the dorsal columns of the spine

47
Q

What can sensory ataxia result in?

A
  • Positive Romberg’s
  • Pseudoathetosis
48
Q

What is pseudoathetosis?

A

Involuntary writhing movements

49
Q

What is cerebellar ataxia?

A

Cerebellar ataxia results from problems with the cerebellum coordinating movement, indicating a cerebellar lesion.

50
Q

What is ‘clinically isolated syndrome’ in MS?

A

Describes the first episode of demyelination and neurological signs and symptoms.

51
Q

Prognosis of ‘clinically isolated syndrome’ in MS?

A

Patients with clinically isolated syndrome may never have another episode or may go on to develop MS.

Lesions on an MRI scan suggest they are more likely to progress to MS.

52
Q

Relapsing-remitting MS can be further classified based on whether the disease is active or worsening.

What do the following mean:
a) active
b) not active
c) worsening
d) not worsening

A

a) new symptoms are developing, or new lesions are appearing on the MRI

b) no new symptoms or MRI lesions are developing

c) there is an overall worsening of disability over time

d) there is no worsening of disability over time

53
Q

2 important investigations in MS?

A

1) MRI scans
2) Lumbar puncture

54
Q

Purpose of MRI scans in MS?

A

can demonstrate typical lesions

55
Q

Purpose of a lumbar puncture in MS?

A

can detect oligoclonal bands in the cerebrospinal fluid (CSF)

56
Q

What can be detected in the CSF in MS?

A

oligoclonal bands

57
Q

What does treatment in MS focus on?

A

Treatment in multiple sclerosis is focused on reducing the frequency and duration of relapses. There is no cure.

58
Q

Management of an acute relapse?

A

High-dose steroids (e.g. oral or IV methylprednisolone): may be given for 5 days to shorten the length of an acute relapse.

Options:

1) 500mg orally daily for 5 days

2) 1g intravenously daily for 3–5 days (where oral treatment has previously failed or where relapses are severe)

59
Q

What drugs are used in reducing relapse in patients with MS?

A

Disease-modifying drugs:

1) natalizumab
2) ocrelizumab
3) fingolimod
4) beta-interferon
5) glatiramer acetate

60
Q

What is often used 1st line for preventing MS relapse?

A

natalizumab

61
Q

Give some symptomatic treatments in MS:

A

1) Exercise to maintain activity and strength

2) Fatigue may be managed with amantadine, modafinil or SSRIs

3) Neuropathic pain may be managed with medication (e.g., amitriptyline or gabapentin)

4) Depression may be managed with antidepressants, such as SSRIs

5) Urge incontinence may be managed with antimuscarinic medications (e.g., solifenacin)

6) Spasticity may be managed with baclofen or gabapentin

7) Oscillopsia may be managed with gabapentin or memantine

62
Q

Management of oscillopsia in MS?

A

Gabapentin (or memantine)

63
Q

Management of fatigue in MS?

A

amantadine, modafinil or SSRIs

64
Q

Management of neuropathic pain in MS?

A

may be managed with medication (e.g., amitriptyline or gabapentin)

65
Q

Management of urge incontinence in MS?

A

antimuscarinic medications (e.g., solifenacin)

66
Q

Management of spasticity in MS?

A

baclofen or gabapentin

67
Q

Before prescribing anticholinergics for bladder dysfunction in MS, what should you do?

A

Get an US of bladder first to assess bladder emptying - anticholinergics may worsen symptoms in some patients

68
Q

What is motor neurone disease (MND)?

A

A term that encompasses a variety of specific diseases affecting the motor nerves. Motor neurone disease is a progressive, eventually fatal condition where the motor neurones stop functioning.

69
Q

Does MND affect sensory nerves?

A

NO - sensory symptoms suggest an alternate diagnosis.

70
Q

What is the most common type of MND?

A

Amyotrophic lateral sclerosis (ALS)

71
Q

What are 4 types of MND?

A

1) Amyotrophic lateral sclerosis (ALS)

2) Progressive bulbar palsy

3) Progressive muscular atrophy

4) Primary lateral sclerosis

72
Q

What does progressive bulbar palsy primarily affect?

A

The muscles of talking and swallowing (the bulbar muscles).

73
Q

Pathophysiology of MND?

A

Motor neurone disease involves a progressive degeneration of both the upper and lower motor neurones. The sensory neurones are spared.

74
Q

Risk factors for MND?

A

1) FH
2) Smoking
3) Heavy metals exposure
4) Pesticides exposure

75
Q

Typical patient with MND?

A

Late middle aged (e.g. 60 y/o) man, possibly with an affected relative.

76
Q

Presentation of MND?

A
  • Insidious, progressive weakness of the muscles throughout the body, affecting the limbs, trunk, face and speech
  • Weakness is often first noticed in the upper limbs.
  • Increased fatigue when exercising
  • Clumsiness, dropping things more often or tripping over.
  • Clurred speech (dysarthria).
77
Q

Give some LMN signs seen in MND

A

1) Muscle wasting

2) Reduced tone

3) Reduced reflexes

4) Fasciculations (twitches in the muscles)

78
Q

Give some UMN signs seen in MND

A

1) Increased tone or spasticity

2) Brisk reflexes

3) Upgoing plantar reflex

79
Q

What is the most common presenting symptom in ALS?

A

asymmetric limb weakness

80
Q

Does MND affect affect external ocular muscles?

A

No

81
Q

Are there cerebellar signs in MND?

A

No

82
Q

How is a diagnosis of MND made?

A

The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy.

83
Q

Investigations in MND?

A

1) nerve conduction studies: will show normal motor conduction and can help exclude a neuropathy

2) electromyography: shows a reduced number of action potentials with increased amplitude

3) MRI: usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy

84
Q

What may an electromyography show in MND?

A

a reduced number of action potentials with increased amplitude

85
Q

Presentation of progressive bulbar palsy?

A

palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

86
Q

Which MND carries the worst prognosis?

A

Progressive bulbar palsy

87
Q

Which MND has UMN signs only?

A

Primary lateral sclerosis

88
Q

Which MND has LMN signs only?

A

Progressive muscular atrophy

89
Q

Which MND carries the best prognosis?

A

Progressive muscular atrophy

90
Q

Describe LMN signs in progressive muscular atrophy

A

affects distal muscles before proximal

91
Q

Focus of MND management?

A

There are no effective treatments for halting or reversing the progression of the disease.

Focus on symptomatic control and slowing progression.

92
Q

1st line management of slowing MND progression?

A

Riluzole

93
Q

What can be used to support breathing when the respiratory muscles weaken in MND?

A

Non-invasive ventilation (NIV) - usually BIPAP

94
Q

What is preferred way to support nutrition and has been associated with prolonged survival in MND?

A

percutaneous gastrostomy tube (PEG)

95
Q

MND prognosis?

A

poor: 50% of patients die within 3 years

Yr 4 - CCC (54 decks)

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