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Flashcards in Neuro - MS Deck (35):
1

Demographic of MS:

Age of onset? 

Gender?

Demographic of MS:

15-45 years old

70% female

2

What is the most common form of ms?

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

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.

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?

Q image thumb

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

4

EDDS:

1. What does this stand for?

2. Level 0 = ?

3. Level 5 = ?

4. Level 10 = ?

EDDS:

1. Expanded disability status scale

2. Level 0 = normal

3. Level 5 = reduced ambulation

4. Level 10 = death

5

What type of MS does the graph represent?

Q image thumb

The graph represents relapsing-remitting MS (RRMS)

6

What type of MS does the graph represent?

Q image thumb

This is secondary progressive MS

7

What type of MS does the graph represent?

Q image thumb

This is primary progressive MS

8

What type of MS does the graph represent?

Q image thumb

This is progressive relapsing MS

9

Relapsing-Remitting MS (RRMS)

1. What is it defined by?

2. How does remission occur?

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

10

Progressive MS

1. What is primary progressive MS (PPMS)?

2. What is secondary progressive MS (SPMS)?

3. What is progressive relapsing MS (PRMS)?

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

11

Benign/Mild MS

1. Considered a subset of what MS?

2. Definition?

3. 10 year follow up?

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.

12

Benign MS

Favorable: 

1. Age of onset?

2. Area of involvement?

3. Area/nature of first attack?

4. Remission duration? Gender?

Benign MS

Favorable

1. Before the age of 40

2. Isolated involvement

3. Optic neuritis, sensory, brainstem

4. First remission duration > 1 year; female

13

Benign MS

Unfavorable:

1. Age?

2. Areas of involvement?

3. Area/nature of first attack?

4. Gender?

Benign MS

Unfavorable:

1. Older than 40

2. Multiple areas of involvement

3. Motor, cerebellar, sphincter

4. Male

14

Pathology of MS:

1. Immune mediated in  ____ susceptible individuals

2. ______ leads to slower nerve conduction

3. Where do lesions occur?

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.

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 ____.

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.

16

What are the red arrows pointing at?

Q image thumb

The red arrows indicate MS plaques

17

Lesions of MS

1. What are active lesions characterized by?

2. What are chronic active lesions characterized by?

 

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

18

Diagnosis:

Ultimately what type of diagnosis?

Diagnosis:

Ultimately a clinical diagnosis - there is no definitive lab test

19

Most common presenting symtpoms?

a. Sensory symptoms in arms/legs - 33%

b. Unilateral vision loss - 16%

c. Multiple symptoms at onset - 14%

20

Diagnoses that mimic MS:

1. Infections?

2. Inflammatory?

3. Metabolic?

4. CNS ____

5. 3 others?

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

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?

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

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?

 

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

23

Symptoms:

1. Cerebral and corpus callosum lesions, as well as generalized brain atrophy,  may cause?

2. Common symptoms in diagnosed MS can include?

 

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

24

McDonald diagnostic criteria:

1. What is traditional diagnostic criteria?

2. Outcome of the diagnostic workup should yield  3 of the following?

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

25

Atrophy in MS:

1. Atrophy in the brain and SC correlates with what 2 things?

2. When does it correlate with poor prognosis?

Atrophy in MS:

1. Correlates with the loss of axons and the occurance of demyelination

2. Early atrophy correlates with poor prognosis

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?

 

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

27

What are the two classes of disease modifying medications?

a. immunomodulators

b. immunosuppressants

28

MS immunotherapy:

Nonspecific immunomodulation drugs?

Selective immunomodulation drug?

Nonspecific immunosuppresion drug?

MS immunotherapy:

Nonspecific immunomodulation: Interferon beta-1b and Interferon beta-1a

Selective immunomodulation: Glatiramer acetate

Nonspecific immunosuppression: Corticosteroids and mitoxantrone

29

1. What drugs slow accumulation of disability?

2. What drug has the lowest relapse rate?

3. What drugs reduce lesions?

 

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)

30

How should actue MS relapses be managed?

Why should it be treated early?

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

31

Neuromyelitis Optica:

1. What is this?

2. Course?

3. First example of?

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

32

Neuromyelitis Optica:

1. Pathology shows?

2. Absolute criteria?

3. Supportive criteria?

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

33

Neuromyelitis Optica:

1. Minor criteria?

2. Treatment?

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

34

Acute Transverse Myelitis:

1. Common in?

2. Treated with?

Acute transverse myelitis:

1. Common in: MS, mycoplasma infection

2. Treatment: high dose steroids, ventilator support, or surgery if indicated

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?

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)