Demyelinating disorders Flashcards

1
Q

Nodes of ranvier precipitate what type of conduction?

A

Saltatory conduction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Name 3 primary demyelinating disorders

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the most common phenotype of multiple sclerosis?

A

Relapsing and remitting

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Risk factors for MS?

A
Higher latitudes
Previous EBV exposure (viral trigger)
1st degree MS relative
HLA DRB1
Young females
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the prevalence of MS?

A

1/1000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What do plaques look like?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are common areas of demyelination?

A

Corpus callous, optic nerve and spinal cord

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the two types of plaques?

A

Active

Inactive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A

Gliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

In inactive cells what predominates?

A

Gliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

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
What is included in charcot's neurological triad?
Dysarthria Nystagmus Intention tremor
26
For a diagnosis of MS what must be present?
Two distinct neurological deficits occurring at different times implicating lesions of white matter
27
For MS what will be present on CSF analysis?
IgG oligoclonal bands
28
In areas of white mater what does demyelination show up as on T2 weighted MRI scans?
Hyperintense regions
29
Aside from MRI and CSF analysis what else can be used to help diagnose MS?
``` Conduction studies (slowed conduction) Histology (lymphocytic infiltration) ```
30
What are important DDx to consider with MS?
``` Vasculitis Granulomatous disorders Vascular disease Structural lesions Infection Metabolic disorders ```
31
MS is incurable. What do they tend to die of?
Pneumonia
32
How do you manage an acute relapse of MS?
Mild--> Symptomatic Moderate-->Oral prednisolone Severe--> Hospital admission & IV methylprednisolone
33
What disease modifying therapy is 1st line in RR MS?
Tecfidera (oral)
34
What disease modifying therapy is 1st line in early primary progressive MS?
Ocrelizumab
35
What is used to alleviate spasticity?
Physiotherapy + occupational therapy + oral baclofen/gabapentin
36
What is used if there if fatigue?
Amantadine +- modafinil