Demyelinating disorders

Question 1

Characteristics of MS

Answer 1

Chronic inflammation
Demyelination
Gliosis
Neuronal Loss

Question 2

Characteristic finding on CSF analysis in MS

Answer 2

Oligoclonal bands

Question 3

Gene associated with predisposition for MS

Answer 3

HLA-DRB1 gene

Question 4

General immunologic pathogenesis of MS

Answer 4

Autoreactive T lymphocytes (type IV hypersensitivity)

B cell activation and antibody response

Question 5

Helper T cell types associated with MS

Answer 5

Th1
Th17

Question 6

CSF abnormalities in MS

Answer 6

Mononuclear cell pleocytosis
Oligoclonal bands (increased IgG)

Question 7

Histology shows perivenular cuffing by inflammatory mononuclear cells predominantly with T cells and macrophages

Answer 7

Active MS plaque

Question 8

Histology shows loss of oligodendrocytes with astrocyte proliferation and gliosis

Answer 8

Inactive MS plaque

Question 9

How areas of demyelination in MS appear on MRI

Answer 9

High-signal on T2 and FLAIR

Question 10

Treatment for acute attack of MS

Answer 10

Corticosteroids

Question 11

Humanized monoclonal antibody against alpha-4 integrin used in treatment of remitting relapsing MS

Answer 11

Natalizumab

Question 12

Reduces the transmission of immune cells into the CNS by interfering with the alpha-4-beta-1 integrin receptor molecules

Answer 12

Natalizumab

Question 13

Risk associated with Natalizumab

Answer 13

Multifocal leukoencephalopathy

Question 14

Acts by suppressing the activity of T cells, B cells, and macrophages on the myelin sheath

Answer 14

Mitoxantrone

Question 15

General mechanism of cladribine

Answer 15

Depletes B cells

Question 16

Mimics the structure of myelin basic protein to act as a decoy for T cells

Answer 16

Glatiramer

Question 17

Shifts population of T cells from proinflammatory Th1 T cells to regulatory Th2 T cells to suppress inflammatory response

Answer 17

Glatiramer

Question 18

Increases cytotoxicity of NK cells and increases phagocytic activity of macrophages to treat MS

Answer 18

Beta interferons (beta-1a)

Question 19

Selective sphingosine-1-phosphate receptor modulators

Answer 19

Fingolimod
Siponimod

Question 20

Drugs that sequester lymphocytes in lymph nodes to prevent them from contributing to an autoimmune reaction, used to treat MS

Answer 20

Fingolimod
Siponimod

Question 21

Multifocal areas of demyelination of varying size in the brain, sparing the spinal cord and optic nerves

Answer 21

Progressive multifocal leukoencephalopathy

Question 22

Cause of progressive multifocal leukoencephalopathy

Answer 22

Reactivation of latent JC virus

Question 23

Demyelination without inflammation in the base of the pons with relative sparing of axons and nerve cells

Answer 23

Osmotic demyelination syndrome/central pontine myelinolysis

Question 24

Tracts affected in osmotic demyelination syndrome

Answer 24

Bilateral corticospinal and corticobulbar tracts

Question 25

Caused by rapid correction of hyponatremia

Answer 25

Osmotic demyelination syndrome

Question 26

Characterized by massive astrocyte death

Answer 26

Osmotic demyelination syndrome

Question 27

Clinical features of osmotic demyelination syndrome

Answer 27

Quadriparesis
Pseudobulbar palsy –> dysarthria, dysphagia, and dysphonia
Locked-in syndrome

Question 28

First manifestation of MS

Answer 28

Impaired vision associated with optic neuritis and internuclear ophthalmoplegia

Question 29

Name for MRI finding in MS

Answer 29

Dawson’s fingers