Myelin disorders

Question 1

Oligodendrocytes are found in…and myelinate

Answer 1

CNS

several internodes

Question 2

Schwann cells are found in …and myelinate

Answer 2

PNS

a single internode

Question 3

What type of conduction occurs with myelination?

Answer 3

Saltatory conduction

- continuous conduction from one node of Ranvier to the next

Question 4

What causes stress to demyelinated neurons

Answer 4

The use of large calibre Na channels to mediate conduction

Question 5

Re-myelination will not occur if

Answer 5

the damage is due to intrinsic myelin defect

Question 6

A cause of secondary myelin loss is

Answer 6

axonal loss

Question 7

Function of astrocytes

Answer 7

Control permeability of BBB

Question 8

Function of microglia

Answer 8

Macrophages of CNS

Question 9

Destruction of myelin sheathes with preservation of the axons =

Answer 9

Demyelination

Question 10

in demyelination there is the axon is

Answer 10

initially preserved

Question 11

Dysmelination is

Answer 11

a genetic disorder of the formation or maintenance of myelin

Question 12

MS
transverse myelitis
Neuromyelitis optics 
PML 
are all examples of

Answer 12

Dysmyelinating disorders

Question 13

dysemelinaÒon in the pons causes

Answer 13

Locked in syndrome

Question 14

MS is an inherited or acquired autoimmune disorder

Answer 14

acquired

Question 15

MS is attributable to white or grey matter lesions

Answer 15

white

Question 16

Radiology

fluffy spots

Answer 16

MS

Question 17

Gross appearance of MS
acute
chronic

Answer 17

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

Question 18

Acute microscopic features of MS

Answer 18

The things driving the myelin loss

• lymphocytes + macrophages
• loss of myelin + debris

Question 19

Chronic microscopic features of MS

Answer 19

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

Question 20

Does MS affect the CNS or PNS

Answer 20

CNS

Question 21

What stain is used for myelin

Answer 21

Lugol fast blue

Question 22

is MS more prevalent at poles or equator?

Answer 22

poles

Question 23

Is MS more common in men or women?

Answer 23

women 2:1

Question 24

TH ? are involved in MS

Answer 24

TH1 and 17

Question 25

TH17 active ...via...to become ...

Answer 25

th17 activate microglia via IFNy to become macrophages

Question 26

What is the best predictor of long term outcome of MS

Answer 26

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

Question 27

RED EYE =

Answer 27

Uveitis

Question 28

Uhthoff's phenomenon

Answer 28

Vision problems with MS | symptoms worsen with heat

Question 29

What indicates active MS on MRI?

Answer 29

Gadolium enhancement

Question 30

What can be detected in the CSF of a patient with MS? | Are these found anywhere else

Answer 30

Oligobands in CSF and plasma | should not be in both

Question 31

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

Answer 31

IFN B1

Question 32

Natalizumab targets... to frustrate....

Answer 32

targets vessels to frustrate lymphocyte trafficking

Question 33

What is the function of mitoxantrone

Answer 33

Suppress lymphocyte proliferation

Question 34

Once off inflammation of the spinal cord, bilateral / symmetrical

Answer 34

Transverse myelitis

Question 35

Devic's neuromyelitis Optics is caused by

Answer 35

Anti-aquaporin antibodies | AQP4 in brain

Question 36

risk of recurrence with Acute disseminated encephalomyelitis?

Answer 36

0 -once off | 20% risk of mortality though

Question 37

Is actute necrotising haemorrhagic encephalomyelitis due to an autoimmune conditon?

Answer 37

No | due to immune complexes + complement activation

Question 38

PML ?

Answer 38

JC virus latent infection in B cells, kidney and CNS | reactivation occurs in immnocompromised

Question 39

Possible consequence of a vigorous correction of low serum sodium?

Answer 39

Central pontine myelinolysis aka osmotic demyelination syndrome - locked in syndrome

Question 40

Galactocerebroside B galactosidase deficiency manifestation? prognosis? treat?

Answer 40

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

Question 41

Build up of sulfatides

Answer 41

deficient lysosomal arylsulfatase A - > metachromatic leukodystrophy

Question 42

Galactosyl sphingosine and globoid cells | 4 months old baby with motor difficulties

Answer 42

Krabbe's disease

Question 43

adrenoleukodystrophy is associated with an accumulation of ....

Answer 43

Very Long Chain Fatty Acids

Question 44

What is the target of the MS antigen?

Answer 44

Mylein basic protein