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Semester 3 > Case 6 - Multiple Sclerosis > Flashcards

Case 6 - Multiple Sclerosis Flashcards

1
Q

what is MS

A

chronic inflammatory condition of the CNS

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2
Q

what does MS result from

A

demyelination and axonal loss

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3
Q

which nerves are not affected

A

peripheral

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4
Q

what is the typical onset age of MS

A

20-40

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5
Q

how much more common is it in females rather than males

A

3 times more in women

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6
Q

what is the prevalence directly correlated to

A

the distance from the equator at which the patient has lived in pre adult years

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7
Q

what is pregnancy thought to be

A

preventative against replaces

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8
Q

what is a single episode known as

A

clinically isolated syndrome

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9
Q

what is clinically isolated syndrome

A

this refers to a set of neurological symptoms that last more than 24 hours before resolution, caused by inflammation or demyelination in the brain, optic nerves or spinal cord.

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10
Q

how many episodes must a patient have before being diagnosed with MS

A

needs to have 2 or more episodes

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11
Q

what disease Is common in those diagnosed with MS

A

more common in those who contract glandular fever at a later stage in life

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12
Q

who is MS rare in

A

those who are seronegative for EBV

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13
Q

what gene is associated with increase risk in MS

A

HLA-DRB1B

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14
Q

what is the lifetime risk for developing MS in the uK

A

1 in 400

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15
Q

percentage for siblings

A

5%

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16
Q

percentage for dizygotic twins

A

5%

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17
Q

percentage for monozygotic twins

A

30%.

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18
Q

what cells mediate an attack on the white matter

A

CD4 T cells mediate an attack on the white matter, specifically the oligodendrocytes which produce myelin and on myelin itself

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19
Q

which cells produce myelin

A

oligodendrocytes

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20
Q

how can macrophages cross the BBB in MS

A

they exhibit glycoprotein alpha 4 beta 1 allowing them to adhere and cross the endothelium

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21
Q

what are the active lesions called

A

plaques

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22
Q

what do macrophages essentially do

A

digest the myelin sheath

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23
Q

what do macrophages produce

A

nitric oxide which directly damages the axon

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24
Q

where are plaques particularly found

A

optic nerves
periventricular region
corpus callosum
brainstem and cerebellar connections
cervical spinal cord
posterior columns
corticospinal traces

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25
Q

how do plaques show up on an MRI

A

white areas

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26
Q

why are they white

A

essentially due to conduction block / axonal loss at the site of the plaques

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27
Q

why can steroid treatment rapidly reverse the symptoms

A

because it rapidly reduces inflammation and allows the oligodendrocytes time to recover

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28
Q

when does axonal loss occur

A

in the latter stages of the disease, and results progressive and persistent disability

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29
Q

what Is benign MS

A

some people only have 1 attack of the disease. essentially this does not progressively deteriorate although there is now evidence that there is actually a gradual decline in function but not on the same scale as other varieties

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30
Q

what is progressive relapsing MS

A

steady decline since onset with super-imposed attacks

<10%

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31
Q

what is secondary progressive MS

A

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

follows on from the relapsing/remitting

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32
Q

what is primary progressive MS

A

steady increase in disability without attacks

10-20% of cases

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33
Q

what is relapsing remitting MS

A

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

80-90%

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34
Q

graphs showing different types of MS

A
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35
Q

what is a relapse

A

there is focal inflammation, damage to myelin and oligodendrocytes and conduction block

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36
Q

what is remission

A

the inflammation subsides and there is re myelination of damaged areas and much of the damaged function may return to normal

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37
Q

what kind of motor function signs are there

A

UMN and LMN signs.

38
Q

what are the different optic nerve signs

A

optic neuropathy
optic disc swelling
Uthoff’s phenomena
relative afferent pupillary defet

39
Q

what is optic neuropathy

A

there is typically blurring of vision in one eye that develops over a period of days. it can vary from frosted glass sensation to severe visual loss, but there is rarely blindness. there might also be some slight eye pain. it is almost always unilateral and full recovery within 2 months

40
Q

what iso optic disc swelling

A

can occur if this area of the nerve is affected, but if the lesion is higher up the nerve than this, there is unlikely to be opthalmosopic abnormalities when the patient sees nothing and neither does the doctor

41
Q

what is this type of lesion known as when neither the patient or doctor can see it

A

retrobulbar neuritis

42
Q

what does disc swelling in optic neuritis cause

A

early and gradual vision loss which can help you differentiate it from disc swelling due to raised intracranial pressure - which causes sudden, late visual loss

43
Q

what is Uthoff’s Phenomenon

A

worsening of the neurological symptoms after a period of exercise and/or increase body heat. this is because conduction is slowed by an increased temperature

44
Q

what are the late optic nerve signs

A

scotoma - dark spot in the centre of vision
colour vision problems
disc pallor
diploma
nystagmus
red may appear grey

45
Q

what are the motor spinal cord signs

A

UMN: weakness, spasticity, brisk reflexes. generally develops over a period of days or weeks, gradually getting worse. usually the result of demyelination in the spinal cord

46
Q

what is L’Hermitte sign

A

on voluntary flexing of the head there is an electric shock sensation travelling down the spine and into the limbs. sometimes called the barber chair phenomenon

47
Q

what is L’Hermitte sign due to

A

a lesion in the posterior columns

48
Q

what are the sensory spinal cord features

A

numbness, parathesia, typically in an ascending pattern over a number of days

49
Q

what are the autonomic spinal cord features

A

incontinence, sexual dysfunction

50
Q

what are the brain stem clinical features

A

opthalmaplegia (INO) neuralgia
hearing loss
vertigo
dysphagia
dysphonia
facial numbness

51
Q

what is internuclear opthalmoplegia

A

a condition affecting the movement of the eyes that is characterised of MS. when covering one eye, unilateral movements will be normal

but when together, the adducting eye will not move past the midline

it may affect one or both eyes, and the patient may complain of horizontal diplopia

it is due to a lesion In the medial longitudinal fasiculus - which is a heavily myelinated tract

thus conjugate movement, but not unilateral movement is affected

52
Q

features of the medial longitudinal fasiculus

A

heavily myelinated tract, which connect optic brainstem nuclei on either side of the brain stem

53
Q

what are the cerebellum features

A

ataxia

54
Q

what are the cerebral hemisphere features

A

anything
epilepsy
trigeminal neuralgia
tonic spasm - brief spasms of the limbs

55
Q

what are the more common neurological signs

A

UMN

56
Q

how does death result in MS

A

from bronchopneumonia or renal failure

57
Q

what is Devic’s disease

A

neuromyelitis optica ( considered a subtype of MS)

58
Q

Features of Devic’s disease

A

optic neuritis
acute myelitis

59
Q

what is the definitive investigation for MS

A

MRI

60
Q

what will MRI show

A

plaques in 85% of clinical presentation

typically flare ups will have about 10 plaques

61
Q

what is the visual evoked potentials test

A

can detect lesions in the visual pathway.
The patient has EEG probes placed on the skull to measure brain response to visual stimuli
They are then given a visual stimulus, and the time between the visual stimulus and the brain response (on EEG) is measured. If the response is delayed this is evidence of some sort of optic nerve lesion

62
Q

what can be seen in a lumbar puncture

A

oligoclonal bands in CSF
Similar to monoclonal bands seen in lymphoma
However, where monoclonal bands represent massive proliferation of one type of plasma cell, oligoclonal bands represent massive proliferation of a small number of type of plasma cell.
Usually only necessary when MRI cannot confirm the diagnosis
Actually not that useful! In 80% of cases there will be oligoconal bands, but these just show that there is lot of antibody to something in the CSF.

63
Q

how is MS diagnosed

A
  • at least 1 attack - several months apart
  • multiple plaques on MRI

OR

  • a single attack
  • multiple plaques on MRI
  • +oligoclonal bands in CSF
  • visual evoked potentials
64
Q

what is a pseudo relapse

A

typically less than 24 hours, systems may fluctuate and then completely resolve, symptoms may be identical to previous relapses. may be triggered by heat, stress, infection.

65
Q

what are pseudo relapses not caused by

A

new lesions

66
Q

what is a true repulse

A

systems typically last more than 24 hours duration and are present in the absence of obvious triggers and often involve different symptoms to previous relapses

67
Q

what are good prognostic factors

A

optic neuritis or isolated sensory symptoms only at diagnosis
Relapsing remitting initial course
Long-interval to second relapse
No disability at 5 years
Normal initial MRI
Younger age at onset
Complete recovery from first episode

68
Q

what are poor prognostic factors

A

multi-focal initial presentation
High lesion load on MRI at initial presentation
Substantial disability at 5 years
High replace rate in first 5 years

69
Q

what percent have significant disability at 5 years

A

<5%

70
Q

without prognosis altering treatment, median time:

A

To requiring walking assistance - 15 years
To bed bound - 26 years
Death - 41 years

71
Q

what is the life expectancy in relation to general population

A

typically 5-10 years less

72
Q

if it is a true release, what is the treatment plan

A

Short course of steroids e.g methylprednisolone, 1g/day for 3-5 days IV or PO
Can induce remission, and reduce the severity of the relapse - do not alter long term outcome
Urgent neurology referral - if acutely disabling symptoms consider same-day neurology review
Avoid using steroids in patients whose symptoms involve only fatigue or sensory loss, or induce only very minimal disability as the benefit is very limited

73
Q

when may patients start on a disease modifying drug

A

when a second episode has been confirmed

74
Q

what is the usual DMD

A

glatiramer

75
Q

what is glatiramer acetate

A

a synthetic polypeptide, similar in structure to myelin basic protein. it is thought that because of this similar structure, it can bind to T-lymphocytes and block the presentation of myelin antigens to the T cells

76
Q

how is GA administered

A

SC 20mg daily
given IV

77
Q

what is mitoxantrone

A

a cytotoxic antibody
may improve long term outcome when given at 3 monthly interval

78
Q

what is natalizumab

A

monoclonal antibody that inhibits adhesion, thus reduces the number of inflammatory cells that are able to cross the BBB

79
Q

how is natilzumab given

A

Reduce the relapse rate
Give IV 300mg once a month
It has been shown to be more effective than both glatirmaer and B interferon however 1 in 600 patients will suffer from progressive multifocal leukocephelopathy as a result of taking natalizumab - which is caused by infection with the JC virus.
The risk is greater the longer that natalizumab is used.

80
Q

what is cyclophosphamide

A

cyclonic effect on lymphocytes

81
Q

what is fingolidmod

A

spinhosine 1 phosphate receptor modulator

82
Q

what does B-interferon do

A

causes the down regulation of interferon y and increases the activity of suppressor T cells

83
Q

what is baclofen used for

A

GABA agonist - reduces spasticity

84
Q

what does diazepam do

A

reduces tremor

85
Q

what does carbamazepine do

A

reduce fatigue, weakness as well as tremor

86
Q

what does pronanolol do

A

reduce rtremor

87
Q

what does oxybutynin or amitriptyline do

A

treats urinary urgency or incontinence

88
Q

what does sildenafil do

A

treats erectile dysfunction

89
Q

pregnancy and MS

A

most drugs used are not safe in pregnancy except glatiramer and natalizumab. Relapse rate during pregnancy is typically much lower than the year preceding pregnancy but high in the postpartum period
Decision on when to cease and when to re start treatments should be discussed with parents on an individual basis

90
Q

what can exacerbate symptoms

A

UTI’s

Semester 3 (94 decks)

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