Lec 67 MS Flashcards
What is a dysmyelinating disease? what 2 types?
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]
What is a demyelinating disease?
myelin sheeth normally formed but is primary target for destruction; usually myelin lost but central axon relatively intact
What matter particularly vulnerable in MS?
white matter tracks = myelinated axon pathways like the corpus callosum
What is clinical presentation of MS?
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
Classic charcot triad of MS mnemonic?
ms is a SIN
- Scanning speech
- Intention tremor [incontinence, internuclear opthalmoplegia]
- Nystagmus
generally represent relatively late signW
What are common early features of MS?
motor weakness, parasthesias, impaired vision, double vision, intention tremor, ataxia
What is presentation of optic neuritis?
over period of days partial or complete loss of vision usually in one eye
unilateral eye pain, classically accentuate by eye movement
What are the different types of clinical patterns of MS?
- relapsing-remitting
- secondary progressive
- primary progressive
- progressive relapsing
What is presentation of relapsing-remitting multiple sclerosis [RRMS]?
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
What are signs of secondary-progressive MS?
occurs within 10 yrs of relapsing/remitting
associated with significant accumulating disability and degenerative phase
What is epidemiology of MS?
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
- All of the following are common initial manifestations of Multiple Sclerosis EXCEPT:
a. Optic Neuritis
b. Ataxia
c. Aphasia
d. Sensory loss
e. Diplopia
C aphasia
What are early features of MS?
motor weakness, paresthesias, impaired vision, brainstem/CN deficits, double vision, intention tremor, ataxia
optic neuritis
What are late features of MS?
cerebellar symptoms = intention tremor, nystagmus, scanning speech
- 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
D
What are characteristic MRI findings in MS?
disseminated, sclerosing lesions in white matter; most are clinically silent
MRI lesions predict development of MS after first attack
What are characteristic CSF findings of MS
normal protein/glucose/cells
high IgG synthetic rate
oligoclonal bands present
normal myelin basic protein = elevated in acute relapse
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
d. basal ganglia
What is pathophysiology of MS?
- 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
WHat are 4 main treatment goals for MS?
- treat MS relapse/exacerbations = short courses of high IV steroids
- modify disease course = meds prevent relapses, achieve clinical stability, decrease new lesions on MRI [b interferon, immunosuppressants]
- reduce development of disability
- treat symptoms = spasticity, pain, fatigue, etc
What is treatment of acute MS?
treat with IV methylpredisolone for 3-5 days
- 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.
c. methyl-prenisolone
don’t give IV!!