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ADELE-Noodles of Neurology > Demyelinating disorders > Flashcards

Demyelinating disorders Flashcards

1
Q

Nodes of ranvier precipitate what type of conduction?

A

Saltatory conduction

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2
Q

Schwann Cells have a limited capacity to remyelinate after damage TRUE/FALSE

A

FALSE

Oligodendrocytes have a limited capacity to demyelinate after damage

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3
Q

Many neurological disorders cause damage to the myelin. Therefore what classifies a demyelinating disorder?

A

Demyelinating disorder is where there is preferential damage to the myelin

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4
Q

Name 3 primary demyelinating disorders

A

Multiple sclerosis
Acute disseminated encephalitis (ADEM)
Acute haemorrhage leukoencephalitis (AHL)

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5
Q

Acute disseminated encephalitis is rapidly fatal TRUE/FALSE

A

FALSE

Acute disseminated encephalitis is mild, self-limiting and low mortality (mainly in children)

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6
Q

What is multiple sclerosis?

A

Autoimmune demyelinating disorder characterised by distinct episodes of neurological deficits, separated in time and which correspond to spatially separated foci of neurological injury

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7
Q

What is the most common phenotype of multiple sclerosis?

A

Relapsing and remitting

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8
Q

Risk factors for MS?

A 
Higher latitudes
Previous EBV exposure (viral trigger)
1st degree MS relative
HLA DRB1
Young females
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9
Q

What is the prevalence of MS?

A

1/1000

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10
Q

What is the pathophysiology of the plaques in MS?

A

T cells cause inflammation and damage to oligodendrocytes in CNS which leaves behind scarred areas of demyelinated neurones called plaques

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11
Q

What do plaques look like?

A

Well circumscribed and demarcated
Glassy translucent quality
vary from small to large lesions

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12
Q

What are common areas of demyelination?

A

Corpus callous, optic nerve and spinal cord

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13
Q

What are the two types of plaques?

A

Active

Inactive

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14
Q

As active plaques age, astrocytes undergo reactive change and inflammatory cells reduce in number. Inflammation dampens down and ——- occurs

A

Gliosis

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15
Q

In inactive cells what predominates?

A

Gliosis

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16
Q

Macroscopically what is the difference between active and inactive plaques?

A

Active- yellow/brown with an ill defined edge which blends into surrounding white matter

Inactive- Well-demarcated grey/brown lesions in white matter, classically situated around lateral ventricles

17
Q

What are shadow plaques?

A

Inactive plaques that appear less distinct and less well circumscribed due to a degree of peripheral demyelination or progressively thinned out myelin sheaths

18
Q

TRUE/FALSE symptoms of MS can worsen with fever or high temperatures

A

TRUE dat

19
Q

What is presentation of MS typically?

A

Focal neurological deficit –> often resolves in early disease
Followed by emergence of another different deficit which worsens over weeks and linger for months

20
Q

What is pathognomic of MS?

A

Bilateral INO (Internuclear opthalmoplegia)

21
Q

What is Lhermitte’s sign?

A

Electric shock like sensations down the upper limbs and trunk on neck flexion

22
Q

What in Uhthoff’s phenomenon?

A

Exacerbation of current symptoms in hot environment i.e. after a hot shower

23
Q

What are the categories that the symptoms of MS fall into?

A

Pyramidal dysfunction
Ocular symptoms
Sensory symptoms
Cerebellar dysfunction

24
Q

What does DANISH stand for?

A 
DANISH is the acronym for the cerebellar symptoms of MS
D- Dysdiadochokinesia
A-Ataxia
N-Nystagmus
I-Intention tremor (and past pointing)
S- Slurred speech
H- Hypotonia
25
Q

What is included in charcot’s neurological triad?

A

Dysarthria
Nystagmus
Intention tremor

26
Q

For a diagnosis of MS what must be present?

A

Two distinct neurological deficits occurring at different times implicating lesions of white matter

27
Q

For MS what will be present on CSF analysis?

A

IgG oligoclonal bands

28
Q

In areas of white mater what does demyelination show up as on T2 weighted MRI scans?

A

Hyperintense regions

29
Q

Aside from MRI and CSF analysis what else can be used to help diagnose MS?

A 
Conduction studies (slowed conduction)
Histology (lymphocytic infiltration)
30
Q

What are important DDx to consider with MS?

A 
Vasculitis
Granulomatous disorders
Vascular disease
Structural lesions
Infection
Metabolic disorders
31
Q

MS is incurable. What do they tend to die of?

A

Pneumonia

32
Q

How do you manage an acute relapse of MS?

A

Mild–> Symptomatic
Moderate–>Oral prednisolone
Severe–> Hospital admission & IV methylprednisolone

33
Q

What disease modifying therapy is 1st line in RR MS?

A

Tecfidera (oral)

34
Q

What disease modifying therapy is 1st line in early primary progressive MS?

A

Ocrelizumab

35
Q

What is used to alleviate spasticity?

A

Physiotherapy + occupational therapy + oral baclofen/gabapentin

36
Q

What is used if there if fatigue?

A

Amantadine +- modafinil

ADELE-Noodles of Neurology (38 decks)

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