Lec 67 MS

Question 1

What is a dysmyelinating disease? what 2 types?

Answer 1

dysmyelinating = myelin sheeth is abnormally formed = usually inherited metabolic cause
2 types
- those affecting primarily white matter [adrenoleukodystrophy]
- those primarily accumulate myelin byproducts in neurons [tay sachs, nieman pick]

Question 2

What is a demyelinating disease?

Answer 2

myelin sheeth normally formed but is primary target for destruction; usually myelin lost but central axon relatively intact

Question 3

What matter particularly vulnerable in MS?

Answer 3

white matter tracks = myelinated axon pathways like the corpus callosum

Question 4

What is clinical presentation of MS?

Answer 4

autoimmune inflammation and demyelination of CNS
optic neuritis [sudden loss of vision], internuclear opthalmoplegia, hemiparesis, hemisensory symptoms, bladder/bowel incontinence, brainstem symptoms, imbalance and cerebellar signs

relapsing and remitting course

most often women in 20s an 30s

Question 5

Classic charcot triad of MS mnemonic?

Answer 5

ms is a SIN

• Scanning speech
• Intention tremor [incontinence, internuclear opthalmoplegia]
• Nystagmus

generally represent relatively late signW

Question 6

What are common early features of MS?

Answer 6

motor weakness, parasthesias, impaired vision, double vision, intention tremor, ataxia

Question 7

What is presentation of optic neuritis?

Answer 7

over period of days partial or complete loss of vision usually in one eye
unilateral eye pain, classically accentuate by eye movement

Question 8

What are the different types of clinical patterns of MS?

Answer 8

• relapsing-remitting
• secondary progressive
• primary progressive
• progressive relapsing

Question 9

What is presentation of relapsing-remitting multiple sclerosis [RRMS]?

Answer 9

symptoms as manifestations of acute flares = can lead to more and more disability as pts do not always completely recover from attacks

usually have clinically isolated syndrome at first presentation

Question 10

What are signs of secondary-progressive MS?

Answer 10

occurs within 10 yrs of relapsing/remitting

associated with significant accumulating disability and degenerative phase

Question 11

What is epidemiology of MS?

Answer 11

age of onset 20-40
women outnumber men
pregnancy is protective
genetics –> 1st, 2nd, and 3rd degree relatives at risk = associated with HLA-DR2

latitudinal effect = increase prevalence with norther exposure

Question 12

1. All of the following are common initial manifestations of Multiple Sclerosis EXCEPT:
   a. Optic Neuritis
   b. Ataxia
   c. Aphasia
   d. Sensory loss
   e. Diplopia

Answer 12

C aphasia

Question 13

What are early features of MS?

Answer 13

motor weakness, paresthesias, impaired vision, brainstem/CN deficits, double vision, intention tremor, ataxia
optic neuritis

Question 14

What are late features of MS?

Answer 14

cerebellar symptoms = intention tremor, nystagmus, scanning speech

Question 15

2. Secondary Progressive MS (SPMS) is characterized by:
   a. A history of steady progressive neurologic decline without a history of relapses
   b. Attacks of neurologic dysfunction with complete recovery from each
   c. Attacks of neurologic dysfunction without complete recovery from each
   d. A history of relapses followed by gradual decline in neurologic function with or without relapses
   e. A rare and unusual form of MS usually found only in east Asia

Answer 15

D

Question 16

What are characteristic MRI findings in MS?

Answer 16

disseminated, sclerosing lesions in white matter; most are clinically silent

MRI lesions predict development of MS after first attack

Question 17

What are characteristic CSF findings of MS

Answer 17

normal protein/glucose/cells
high IgG synthetic rate
oligoclonal bands present
normal myelin basic protein = elevated in acute relapse

Question 18

All of the following are common locations for MS lesions on MRI EXCEPT:

a. periventricular
b. brainstem/infratentorial
c. optic nerve
d. basal ganglia	
e. spinal cord

Answer 18

d. basal ganglia

Question 19

What is pathophysiology of MS?

Answer 19

• distributed across many regions time/space
• T helper 1 [TH1] cells activated by unknown antigen [virus or other]
• molecular mimicry mistake myelin protein for antigen
• TH2 play regulatory role = decreased relative to TH1

TH1 = pro-inflammatory –> increase inflammation, cause tissue edema, damage to myelin and nerves

Question 20

WHat are 4 main treatment goals for MS?

Answer 20

1. treat MS relapse/exacerbations = short courses of high IV steroids
2. modify disease course = meds prevent relapses, achieve clinical stability, decrease new lesions on MRI [b interferon, immunosuppressants]
3. reduce development of disability
4. treat symptoms = spasticity, pain, fatigue, etc

Question 21

What is treatment of acute MS?

Answer 21

treat with IV methylpredisolone for 3-5 days

Question 22

4. When considering a possible relapse in an MS patient, you should:
   a. Stop the patient’s disease-modifying therapy
   b. Treat with oral prednisone for 1 week
   c. Treat with IV steroids (methyl-prenisolone) for 5 days
   d. Get an MRI every time
   e. Examine for new neurologic signs, rule out infection, treat with high-dose IV steroids for 5 days.

Answer 22

c. methyl-prenisolone

don’t give IV!!