Demyelinating Diseases

Question 1

In general, Demyelinating diseases damage ____ and preserve _____

Answer 1

Damage myelin

Preserve axons

Question 2

What is an autoimmune disease that involves neuro deficits separated in time due to lesions separated in space?

Answer 2

Multiple Sclerosis (MS)

Question 3

Multiple Sclerosis involves relapsing and remitting episodes of variable duration. What is commonly the initial symptom?

Answer 3

Optic Neuritis = UNILATERAL visual impairment

Question 4

What are 3 genetic links that have been identified for Multiple Sclerosis?

Answer 4

DR2
IL-2
IL-7

Question 5

What vitamin deficiency is linked to Multiple Sclerosis?

Answer 5

Vitamin D Deficiency

– more cases farther away from the equator

Question 6

What cells initiate the chronic inflammation and react against self myelin antigens with Multiple Sclerosis?

Answer 6

CD4+ Th1 and Th17 = T cells

Question 7

With Multiple Sclerosis, how to the plaques look and where are they usually located?

Answer 7

Depressed, glassy, gray-tan plaques

= Adjacent to lateral ventricles and Optic N.

Question 8

What are the 3 plaque types that can be seen with Multiple Sclerosis?

Answer 8

1. Active - ongoing myelin breakdown
2. Inactive
3. Shadow

Question 9

Active plaques with Multiple Sclerosis are sites of ongoing myelin breakdown. What is preserved with these plaques that is NOT with Inactive plaques?

Answer 9

Axons are preserved with Active plaques

– Inactive plaques = Decreased axons

Question 10

Shadow plaques with Multiple Sclerosis

Answer 10

NOT sharply circumscribed

Question 11

What are the CSF findings with Multiple Sclerosis?

Answer 11

• INCREASED protein and IgG

- Pleocytosis (increased cells)

Question 12

Oligoclonal IgG bands in the gamma region may indicated with demyelinating disease?

Answer 12

Multiple Sclerosis

Question 13

Neuromyelitis Optica

Answer 13

Synchronous BILATERAL optic neuritis and spinal cord demyelination

Question 14

Those with Neuromyelitis Optica may develop?

Answer 14

Multiple Sclerosis

Question 15

With Neuromyelitis Optica, there are antibodies to?

Answer 15

Aquaporins

Question 16

This demyelinating disease has antibodies to Aquaporins and it can be treated with Plasmapheresis

Answer 16

Neuromyelitis Optica

= Synchronous BILATERAL optic neuritis and spinal cord demyelination

Question 17

What is found in the CSF with Neuromyelitis Optica?

Answer 17

Neutrophils – turbid appearance

Question 18

What does Acute Disseminated Encephalomyelitis follow?

Answer 18

Viral infection or rarely, Viral immunization

Question 19

Unlike Multiple Sclerosis, the lesions causing myelin loss with Acute Disseminated Encephalomyelitis are ____

Answer 19

Monophasic

Question 20

This disease involves myelin loss with preservation of axons following a viral infection

Answer 20

Acute Disseminated Encephalomyelitis

Question 21

Acute Necrotizing Hemorrhagic Encephalomyelitis is likely fatal. What does it follow?

Answer 21

An Upper Respiratory Infection

Question 22

In what patient population is Acute Necrotizing Hemorrhagic Encephalomyelitis usually seen?

Answer 22

Children and young adults

Question 23

This disease involves demyelination in the CNS following an Upper respiratory tract infection

Answer 23

Acute Necrotizing Hemorrhagic Encephalomyelitis

Question 24

Where is the myelin lost from with Central Pontine Myelinolysis?

Answer 24

• Basis Pontis

- Portions of Pontine Tegmentum

Question 25

Where is the myelin lost from with Central Pontine Myelinolysis?

Answer 25

• Basis Pontis

- Portions of Pontine Tegmentum

Question 26

What causes Central Pontine Myelinolysis?

Answer 26

Overly rapid correction of Hyponatremia

Question 27

What causes Central Pontine Myelinolysis?

Answer 27

Overly rapid correction of Hyponatremia

Question 28

What are the symptoms of Central Pontine Myelinolysis?

Answer 28

Paralysis
Dysphagia, Dysarthria (slurred speech), Diplopia (double vision)
Loss of consciousness

Question 29

If a patient presents a few days after treatment for Hyponatremia with paralysis, dysphagia, diplopia and dysarthria, what is the likely disease?

Answer 29

Central Pontine Myelinolysis