Neuro - MS

Question 1

Demographic of MS:

Age of onset?

Gender?

Answer 1

Demographic of MS:

15-45 years old

70% female

Question 2

What is the most common form of ms?

50% of these patients convert to ____ ____ MS within 10 years, if ___ modifying therapy is given.

Answer 2

Relapsing-remitting MS (RRMS) is the most common form at initial diagnosis: 85%

50% of RRMS patients convert to secondary progressive MS (SPMS) within 10 years, if NO modifying therapy is given.

Question 3

Natural history of MS:

1. What does the green line indicate?
2. What does the purple line indicate?
3. Red line and yellow aarows?

Answer 3

Natural hisotry of MS

1. The green line measures brain volume
2. The purple line shows relapses and impairment
3. The red line and yellow aarows indicate activity of MRI

Question 4

EDDS:

1. What does this stand for?
2. Level 0 = ?
3. Level 5 = ?
4. Level 10 = ?

Answer 4

EDDS:

1. Expanded disability status scale
2. Level 0 = normal
3. Level 5 = reduced ambulation
4. Level 10 = death

Question 5

What type of MS does the graph represent?

Answer 5

The graph represents relapsing-remitting MS (RRMS)

Question 6

What type of MS does the graph represent?

Answer 6

This is secondary progressive MS

Question 7

What type of MS does the graph represent?

Answer 7

This is primary progressive MS

Question 8

What type of MS does the graph represent?

Answer 8

This is progressive relapsing MS

Question 9

Relapsing-Remitting MS (RRMS)

1. What is it defined by?
2. How does remission occur?

Answer 9

Relapsing-Remitting MS (RRMS)

1. It is defined by “attacks” (flare ups/exacerbations/relapses) that last greater than or equal to 24 hours. Symptoms may take up to a month to resolve
2. Remission occurs either spontaneously or with treatment

Question 10

Progressive MS

1. What is primary progressive MS (PPMS)?
2. What is secondary progressive MS (SPMS)?
3. What is progressive relapsing MS (PRMS)?

Answer 10

Progressive MS

1. PPMS: (10%) Progressive deterioration without remission
2. SPMS: develpos in approx. 50% of untreated people with RRMS; slow progressive neurological deterioration with or without occasional acute flare-ups/remissions/plateaus
3. PRMS: (5%) progressive from the start (with acute flare-ups) and continued deterioration between relapses

Question 11

Benign/Mild MS

1. Considered a subset of what MS?
2. Definition?
3. 10 year follow up?

Answer 11

Benign/Mild MS

1. Considered a subset of RRMS
2. No accepted definition (no restriction of activity at >10 years, able to work after 20 years.. etc etc)
3. Only 10-20% maintain a true mild course. Benign MS does not always remain benign.

Question 12

Benign MS

Favorable:

1. Age of onset?
2. Area of involvement?
3. Area/nature of first attack?
4. Remission duration? Gender?

Answer 12

Benign MS

Favorable

1. Before the age of 40
2. Isolated involvement
3. Optic neuritis, sensory, brainstem
4. First remission duration > 1 year; female

Question 13

Benign MS

Unfavorable:

1. Age?
2. Areas of involvement?
3. Area/nature of first attack?
4. Gender?

Answer 13

Benign MS

Unfavorable:

1. Older than 40
2. Multiple areas of involvement
3. Motor, cerebellar, sphincter
4. Male

Question 14

Pathology of MS:

1. Immune mediated in ____ susceptible individuals
2. ______ leads to slower nerve conduction
3. Where do lesions occur?

Answer 14

Pathology of MS:

1. Immune mediated in genetically susceptible individuals
2. Demyelination leads to slower nerve conduction
3. Lesions occur in optic nerves, cerebellum, cerebral cortex, periventricular white matter, spinal cord, and the brain stem.

Question 15

Cause of Demyelination and Axonal Loss:

Activation of autoreactive ____ ___ cells in the peripheral immune system. There is then migration of autoreactive ___ cells into the ____. This is followed by in situ reactivation by myelin autoantigens and activation of _____ and ___ cells. There is secretion of _____ ____. The terminal result is inflammation, _____, ____ transection, and ____.

Answer 15

Cause of Demyelination and Axonal Loss:

Activation of autoreactive CD4+ T cells in the peripheral immune system. There is then migration of autoreactive TH1 cells into the CNS. This is followed by in situe reactivation by myelin autoantigens and activation of macrophages and B cells. There is secretion of proinflammatory cytokines. The terminal result is inflammation, demyelination, axonal transection, and degeneration.

Question 16

What are the red arrows pointing at?

Answer 16

The red arrows indicate MS plaques

Question 17

Lesions of MS

1. What are active lesions characterized by?
2. What are chronic active lesions characterized by?

Answer 17

Lesions of MS

1. Active lesions are characterized by abundant and evenly distributed MHC II cells and myelin-protein positive inclusions within macrophages
2. Chronic active lesions are characterized by MHC II that are abundant at the edges of lesions and less frequent in the center

Question 18

Diagnosis:

Ultimately what type of diagnosis?

Answer 18

Diagnosis:

Ultimately a clinical diagnosis - there is no definitive lab test

Question 19

Most common presenting symtpoms?

Answer 19

a. Sensory symptoms in arms/legs - 33%
b. Unilateral vision loss - 16%
c. Multiple symptoms at onset - 14%

Question 20

Diagnoses that mimic MS:

1. Infections?
2. Inflammatory?
3. Metabolic?
4. CNS ____
5. 3 others?

Answer 20

Diagnoses that mimic MS:

1. Lyme disease, neurosyphilis, HIV, HTLV-1
2. SLE, Sjorgren’s, Sarcoidosis
3. Vitamin B12 and E deficiencies
4. CNS Lymphoma
5. Cervical spondylosis, motor neuron disease, Myasthenia gravis

Question 21

CSF in MS:

1. How many cells per ml? Type of cell?
2. If the cell count is greater than 50/ul consider other ____
3. CSF protein? IgG? Oligoclonal bands?
4. What is possible to note with Oligoclonal bands?

Answer 21

CSF in MS:

1. 5-15 cells/ul; mostly T- lymphocytes
2. etiology
3. Protein = normal; IgG = elevated; OCB = often detected
4. Number of bands may increase with disease duration

Question 22

Symptoms:

1. Inflammation of the optic nerve can cause?
2. Brain stem lesions may cause changes in?
3. Cerebellar lesions may cause?
4. Spinal cord lesions may cause?

Answer 22

Symptoms:

1. Inflammation of the optic nerve can cause retrobulbar pain, color desaturation, and visual loss.
2. Brain stem lesions may cause changes in eye movement, vertigo, dysarthria, dysphagia, and facial weakness.
3. Cerebellar lesions may cause ataxia and tremor.
4. Spinal cord lesions may cause bowel, bladder, and sexual dysfunction, and spastic gait difficulties

Question 23

Symptoms:

1. Cerebral and corpus callosum lesions, as well as generalized brain atrophy, may cause?
2. Common symptoms in diagnosed MS can include?

Answer 23

Symptoms:

1. Cerebral and corpus callosum lesions, as well as generalized brain atrophy, may cause cognitive dysfunction.
2. Fatigue, Pain, Spasticity, Elimination dysfunctions, Cognitive impairment, and Sexual dysfunction

Question 24

McDonald diagnostic criteria:

1. What is traditional diagnostic criteria?
2. Outcome of the diagnostic workup should yield 3 of the following?

Answer 24

McDonald diagnostic criteria:

1. Preserve traditional diagnostic criteria of 2 attacks of disease separated in space and time.
2. 1 Gd-enhancing lesion or 9 hyperintense lesions, 1 or more infratentorial lesions, 1 or more juxtacortical lesions, or 3+ periventricular lesions

Question 25

Atrophy in MS: 1. Atrophy in the brain and SC correlates with what 2 things? 2. When does it correlate with poor prognosis?

Answer 25

Atrophy in MS: 1. Correlates with the loss of axons and the occurance of demyelination 2. Early atrophy correlates with poor prognosis

Question 26

Treat: 1. When is the best time to treat? 2. Increased relapse rate early is associated with? 3. Where does axonal damage occur? What does it correlate with?

Answer 26

Treat: 1. The best time to treat is early, as soon as possible 2. Associated with increased long-term disability 3. Axonal damage occurs in areas of demyelination and correlates with permanent disability

Question 27

What are the two classes of disease modifying medications?

Answer 27

a. immunomodulators b. immunosuppressants

Question 28

MS immunotherapy: Nonspecific immunomodulation drugs? Selective immunomodulation drug? Nonspecific immunosuppresion drug?

Answer 28

MS immunotherapy: _Nonspecific immunomodulation_: Interferon beta-1b and Interferon beta-1a _Selective immunomodulation_: Glatiramer acetate _Nonspecific immunosuppression_: Corticosteroids and mitoxantrone

Question 29

1. What drugs slow accumulation of disability? 2. What drug has the lowest relapse rate? 3. What drugs reduce lesions?

Answer 29

1. _Slows accumulation of disability_: IFN B-1a (avonex and rebif) 2. _Lowest relapse rate_: IFN B-1a (avonex) 3. _Reduces lesions_: IFN B-1a and Glatiramer: _Betaseron_ every other day reduced lesions the most compared to baseline for RRMS (relapsing-remiting MS)

Question 30

How should actue MS relapses be managed? Why should it be treated early?

Answer 30

_Relapses_: high-dose steroids _Treat early_: permanent clinical disability is related to axonal damage which occurs early in the disease course (thus treat to minimize damage); not based solely on severity of clinical disease but on preventing cumulative axonal damage/cerebral atrophy/and eventual irreversible disability

Question 31

Neuromyelitis Optica: 1. What is this? 2. Course? 3. First example of?

Answer 31

Neuromyelitis Optica: 1. This is a demyelinating disorder characterized by optic neuritis or transverse myelitis 2. It may follow a monophasic, chronic, or relapsing-remitting course. Attacks may be separated by several decades 3. This may be the first example of an _autoimmune channelopathy_

Question 32

Neuromyelitis Optica: 1. Pathology shows? 2. Absolute criteria? 3. Supportive criteria?

Answer 32

Neuromyelitis Optica: 1. _Pathology_: inflammatory or necrotic lesions, infiltration by neutrophils/eosinophils, vascular hyalinization, perivascular IgM deposition and compliment activation, & B cell mediated 2. _Absolute criteria_: bilateral optic neuritis, acute transverse myelitis, and no evidence of clinical disease outside SC or optic nerve 3. _Supportive criteria_: NMO-Ig Ab in sera, Negative brain MRI at onset, SC MRI with signal abnormality extending over 3 vertebrae

Question 33

Neuromyelitis Optica: 1. Minor criteria? 2. Treatment?

Answer 33

Neuromyelitis Optica: 1. _Minor criteria_: CSF pleocytosis \>50WBC/mm3; _Absence of oligoclonal bands_; and visual acuity of 20/200 in at least one eye 2. _Treatment_: high dose IV steroids, plasma exchange, and/or immunosuppressives

Question 34

Acute Transverse Myelitis: 1. Common in? 2. Treated with?

Answer 34

Acute transverse myelitis: 1. _Common in_: MS, mycoplasma infection 2. _Treatment_: high dose steroids, ventilator support, or surgery if indicated

Question 35

Optic neuritis: 1. What is this? 2. What is retrobulbar neuritis? 3. What is papillitis? Associated with? 4. This can lead to? 5. Treatment?

Answer 35

Optic neuritis: 1. Partial/total loss of vision in one or both eyes 2. _Retrobulbar neuritis_: lesion in posterior 2/3rds of optic N. 3. _Papillitis_: lesion in anterior portion of nerve, assiciated with pain and decrease in visual acuity 4. Optic Neuritis leads to MS in 85% 5. _Treatment_: steroids (and use of steroids delays conversion to MS)