MS and other Inflammatory Diseases

Question 1

[MS Types]

S/sx improve partially or completely
Followed after a variable interval by recurrence or new abnormality in other parts

A) Clinically isolated syndrome
B) Relapsing-remitting pattern
C) Secondary progressive MS
D) Primary progressive MS

Answer 1

B) Relapsing-remitting pattern

Question 2

[MS Types]

Initially relapsing profile becomes steadily progressive in later stages of the disease

A) Clinically isolated syndrome
B) Relapsing-remitting pattern
C) Secondary progressive MS
D) Primary progressive MS

Answer 2

C) Secondary progressive MS

Question 3

[MS Types]

Disease has a steadily progressive course from its initial presentation, esp those more than 40 yo.

A) Clinically isolated syndrome
B) Relapsing-remitting pattern
C) Secondary progressive MS
D) Primary progressive MS

Answer 3

D) Primary progressive MS

Question 4

What are destroyed in acute lesions of MS?

Answer 4

Myelin sheaths

Question 5

What are destroyed in chronic lesions of MS?

Answer 5

Axons and neurons

Question 6

Histologic appearance of long-standing MS lesions

Answer 6

Thickly matted, relatively acellular glial tissue

Question 7

Pathologic appearance of most severe older MS lesions

Answer 7

Cavitation

Question 8

Dominant mechanism of demyelination in MS

Answer 8

Antibody and complement-mediated myelin phagocytosis

Question 9

[MS Histologic Subgroups]

Inflammatory lesions w/ T cells and macrophages

Answer 9

Pattern I

Question 10

[MS Histologic Subgroups]

Apoptosis of oligodendrocytes and absence of Ig or complement.

Partial remyelination

Answer 10

Pattern III

Question 11

[MS Histologic Subgroups]

Autoantibody lesion, Ig and complement

Answer 11

Pattern II

Question 12

[MS Histologic Subgroups]

Oligodendrocyte dystrophy

No remyelination

Answer 12

Pattern IV

Question 13

Peak age at presentation of MS

Answer 13

30 years

Question 14

Strongest genetic association for MS

Answer 14

DR locus on chromosome 6

HLA-DR6

Question 15

Infectious agents associated with MS

Answer 15

B. burgdorferi
C. pneumoniae
Herpesvirus type 6

*EBV

Question 16

In MS, antibodies are directed against (2)?

Answer 16

Myelin oligodendrocyte glycoprotein (MOG)

Myelin basic protein (MBP)

Question 17

MOG and MBP activate what kind of T cells?

Answer 17

T cell subset CD41 Th2 cells

Question 18

What is the Uhthoff phenomenon?

Answer 18

Temporary induction by HEAT or EXERCISE of symptoms of MS

Question 19

What is the Lhermitte sign?

Answer 19

Tingling, electric-like feeling down the shoulders and back

2/2 inc sensitivity of demyelinated axons to stretch or pressure

Question 20

Initial symptom in 1/2 of MS patients

Answer 20

Weakness or numbness in one or more limbs

Question 21

What is the Pulfrich effect

Answer 21

Pendulum swinging perpendicularly appears to move in a 3d, circular path

Question 22

What is the Charcot triad?

Answer 22

Nystagmus
Scanning speech
Intention tremor

Question 23

What is the most common manifestation of progressive MS?

Answer 23

Asymmetric spastic paraparesis

Impaired joint position & vibration sense in legs

Question 24

What is La Belle Indifference?

Answer 24

Pathologic cheerfulness in the face of an obvious neurologic deficit

Question 25

In terms of bladder dysfunction in MS patients, which is more common? A) Hesitancy, urgency, frequency, incontinence B) Urinary retention

Answer 25

A) Hesitancy, urgency, frequency, incontinence

Question 26

Other diseases that will present with CSF oligoclonal bands

Answer 26

MS Syphilis Lyme disease SSPE

Question 27

CSF IgG is considered positive for MS if __% of total protein

Answer 27

>12%

Question 28

4 typical locations of lesions in MS

Answer 28

Periventricular Juxtacortical Infratentorial Spinal cord

Question 29

What is the feature most predictive of long-term disability in MS?

Answer 29

Degree of disability at 5 years from the first symptom

Question 30

MOA of natalizumab

Answer 30

Directed against alpha-integrin

Question 31

Which MS medication is known to be associated the most with PML?

Answer 31

Natalizumab

Question 32

Rituximab targets CD__ lymphocytes

Answer 32

CD20

Question 33

Alemtuzumab targets CD__

Answer 33

CD52

Question 34

Which MS drug is known to cause autoimmune thyroiditis and idiopathic thrombocytopenic purpura?

Answer 34

Alemtuzumab

Question 35

MOA of Fingolimod

Answer 35

S1P1 receptor analogue

Question 36

Side effects of Fingolimod

Answer 36

``` Bradycardia, AV block Macular edema Melanoma Herpes infections Elevated LFT ```

Question 37

MOA of Teriflunomide

Answer 37

Inhibits dihtdro-orotage dehydrogenase | Suppresses synthesis of DNA pyrimidine bases

Question 38

SE of teriflunomide

Answer 38

Thinning hair Teratogenic Transaminitis Nasopharyngitis Diarrhea

Question 39

SE of Glatiramer

Answer 39

Flushing Chest tightness, palpitations Anxiety

Question 40

Which class of MS drugs is known to induce development of antibodies against it?

Answer 40

Interferon beta

Question 41

Which MS drug can cause systemic capillary leak syndrome?

Answer 41

Interferon-beta

Question 42

True or false. Treatment with oral prednisone alone slightly increased the risk of new episodes of Optic Neuritis

Answer 42

True

Question 43

Use of Amantadine in MS

Answer 43

Fatigue tx

Question 44

Antibodies in NMO

Answer 44

Aquaporin-4 water channel protein

Question 45

Criteria for NMO

Answer 45

2 of the ff: Longitudinally extensive myelopathy Positive antibodies Initial MRI not characteristic of MS

Question 46

NMO affects A) astrocytes B) oligodendrocytes

Answer 46

A) Astrocytes

Question 47

MS affects A) astrocytes B) oligodendrocytes

Answer 47

B) oligodendrocytes

Question 48

NMO immunologic process A) humoral B) cellular

Answer 48

A) humoral

Question 49

MS immunologic process A) humoral B) cellular

Answer 49

B) cellular

Question 50

Areas of predilection for NMO:

Answer 50

Optic nerves Spinal cord Brain (area postrema, hypothalamus)

Question 51

Oligoclonal bands are more commonly seen in A) MS B) NMO

Answer 51

A) MS

Question 52

CSF pleocytosis more commonly seen in A) MS B) NMO

Answer 52

B) NMO

Question 53

True or false. NMO clinical effects less likely to be permanent

Answer 53

False

Question 54

True or fase. Disease modifying agents used in MS may have a deleterious effect on NMO’s relapse rate

Answer 54

True IFN-B, natalizumab, fingolimod

Question 55

Associated infections in ADEM

Answer 55

Older: Measles*, rubella, smallpox, chickenpox More recent: EBV, CMV, M. Pneumoniae, HIV

Question 56

ADEM syndrome associated with chickenpox

Answer 56

Pure cerebellar syndrome

Question 57

Most fulminant form of demyelinating disease

Answer 57

Acute Necrotizing Hemorrhagic Encephalomyelitis / Acute Hemorrhagic Leukoencephalitis of Weston Hurst

Question 58

Focal demyelination of central area of corpus callosum seen in middle-aged chronic alcoholics

Answer 58

Marchiafava-Bignami Disease

Question 59

Granular mitosis in reactive astrocytes are called __ cells. How do you differentiate from gliomas?

Answer 59

Creutzfeldt-Peters Cells Distributed evenly, do not clump together

Question 60

Sharply circumscribed hypocellular plaque. Fibrillary gliosis. A) Active MS lesion B) Chronic inactive MS lesion

Answer 60

B) Chronic inactive MS lesion

Question 61

Most common form of MS

Answer 61

Relapsing-remitting MS