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CNS Diseases > Myelin disorders > Flashcards

Myelin disorders Flashcards

1
Q

Oligodendrocytes are found in…and myelinate

A

CNS

several internodes

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2
Q

Schwann cells are found in …and myelinate

A

PNS

a single internode

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3
Q

What type of conduction occurs with myelination?

A

Saltatory conduction

- continuous conduction from one node of Ranvier to the next

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4
Q

What causes stress to demyelinated neurons

A

The use of large calibre Na channels to mediate conduction

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5
Q

Re-myelination will not occur if

A

the damage is due to intrinsic myelin defect

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6
Q

A cause of secondary myelin loss is

A

axonal loss

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7
Q

Function of astrocytes

A

Control permeability of BBB

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8
Q

Function of microglia

A

Macrophages of CNS

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9
Q

Destruction of myelin sheathes with preservation of the axons =

A

Demyelination

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10
Q

in demyelination there is the axon is

A

initially preserved

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11
Q

Dysmelination is

A

a genetic disorder of the formation or maintenance of myelin

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12
Q 
MS
transverse myelitis
Neuromyelitis optics 
PML 
are all examples of
A

Dysmyelinating disorders

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13
Q

dysemelinaÒon in the pons causes

A

Locked in syndrome

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14
Q

MS is an inherited or acquired autoimmune disorder

A

acquired

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15
Q

MS is attributable to white or grey matter lesions

A

white

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16
Q

Radiology

fluffy spots

A

MS

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17
Q

Gross appearance of MS
acute
chronic

A

sharply circumscribed plaques in white matter
acute - pink or tan ( pink Is cute)
chronic - grey or glassy
- old ppl have grey hair + glasses

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18
Q

Acute microscopic features of MS

A

The things driving the myelin loss

  • lymphocytes + macrophages
  • loss of myelin + debris
19
Q

Chronic microscopic features of MS

A
  • less oligodendrocytes
  • gliosis - astrocyte scar tissue
  • macrophages filled w debris
  • secondary axonal loss
    might see remyelination
20
Q

Does MS affect the CNS or PNS

A

CNS

21
Q

What stain is used for myelin

A

Lugol fast blue

22
Q

is MS more prevalent at poles or equator?

A

poles

23
Q

Is MS more common in men or women?

A

women 2:1

24
Q

TH ? are involved in MS

A

TH1 and 17

25
Q

TH17 active …via…to become …

A

th17 activate microglia via IFNy to become macrophages

26
Q

What is the best predictor of long term outcome of MS

A

Axonal loss detected in optic disc
swing flashlight to bad eye - pupil dilates
colour desaturation - pale optic disc
NO RED EYE

27
Q

RED EYE =

A

Uveitis

28
Q

Uhthoff’s phenomenon

A

Vision problems with MS

symptoms worsen with heat

29
Q

What indicates active MS on MRI?

A

Gadolium enhancement

30
Q

What can be detected in the CSF of a patient with MS?

Are these found anywhere else

A

Oligobands in CSF and plasma

should not be in both

31
Q

What can be used to suppress trafficking and Th17 activity in MS?

A

IFN B1

32
Q

Natalizumab targets… to frustrate….

A

targets vessels to frustrate lymphocyte trafficking

33
Q

What is the function of mitoxantrone

A

Suppress lymphocyte proliferation

34
Q

Once off inflammation of the spinal cord, bilateral / symmetrical

A

Transverse myelitis

35
Q

Devic’s neuromyelitis Optics is caused by

A

Anti-aquaporin antibodies

AQP4 in brain

36
Q

risk of recurrence with Acute disseminated encephalomyelitis?

A

0 -once off

20% risk of mortality though

37
Q

Is actute necrotising haemorrhagic encephalomyelitis due to an autoimmune conditon?

A

No

due to immune complexes + complement activation

38
Q

PML ?

A

JC virus latent infection in B cells, kidney and CNS

reactivation occurs in immnocompromised

39
Q

Possible consequence of a vigorous correction of low serum sodium?

A

Central pontine myelinolysis aka osmotic demyelination syndrome
- locked in syndrome

40
Q

Galactocerebroside B galactosidase deficiency
manifestation?
prognosis?
treat?

A

Krabbe disease

  • auto recessive
  • Rapid motor difficulties from 3-6 months
  • die before age 2
  • bone marrow transplant to replace microglia
41
Q

Build up of sulfatides

A

deficient lysosomal arylsulfatase A - > metachromatic leukodystrophy

42
Q

Galactosyl sphingosine and globoid cells

4 months old baby with motor difficulties

A

Krabbe’s disease

43
Q

adrenoleukodystrophy is associated with an accumulation of ….

A

Very Long Chain Fatty Acids

44
Q

What is the target of the MS antigen?

A

Mylein basic protein

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