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08 Neuro > Demyelinating Diseases > Flashcards

Demyelinating Diseases Flashcards

1
Q

major proteins of cns myelin

A

plp

mpb

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2
Q

what is dysmyelination

A

production of abnormal and unstable myelin sheath (hypomyelination)

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3
Q

what is demyelination

A

destruction of normal myelin

primary: oligodendroglia and schwann cells with myelin sheath, axons preserved
secondary: axon loses myelin sheath, axons damaged

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4
Q

what is multiple sclerosis

A

episodes of focal disorders of the optic nerves, spinal cord, and brain

diagnosis requires: history of remission and relapse and evidence of examination of more than one discrete lesion of the cns

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5
Q

most common symptoms

A

pyramidal weakness or numbness, visual loss, sensory

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6
Q

clinically isolated syndrome

A

optic neuritis, transverse myelitis, cerebellar ataxia, brainstem syndromes

clinically isolated syndrome = syndrome + other features of ms

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7
Q

characteristics of optic neuritis

A

sudden loss of monocular partial or complete vision
pain with movement of affected eye
loss of color vision, particularly the color red

uhtoff’s phenomenon: worsening neurologic symptoms with increased body temp

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8
Q

mri of optic neuritis

A

hyperintense optic nerve = active inflammation

funduscopy: edema or swelling of the optic head

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9
Q

natural history of optic neuritis

A

worsens over several days to 2 weeks and then improves
visual acuity complete in 5 weeks
50% of patients develop other signs of ms

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10
Q

presentation of transverse myelitis

A 
acute/subacute sx/sy of neurologic dysfunction in motor, sensory, or autonomic nerves and spinal cord
symmetrical or asymmetrical paraparesis or paraplegia
ascending paresthesia
loss of deep sensibility of feet
sensory level on trunk
sphincter dysfunction
bilateral babinski
prior infectious illness
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11
Q

csf and mri findings in transverse myelitis

A

csf: modest number of lymphocytes and increased total protein
mri: focal demyelination of spinal cord + hyperintense areas

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12
Q

incidence of ms

A 
21-40 ys
f > m
white populations
western areas
lack of vitamin d
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13
Q

etiology of ms

A

viral infection is trigger
ebv and hhv6
chlamydia and borrelia burgdorferi

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14
Q

three types of ms

A

acute and tumefactive ms
diffuse cerebral sclerosis of schilder
concentric sclerosis of balo

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15
Q

presentation of acute and tumefactive ms (marburg’s)

A

rapidly progressive and highly malignant
cerebral, brainstem, and spinal manifestations
stupor, coma, decerebrate with prominent cn and corticospinal abnormalities

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16
Q

what is diffuse cerebral sclerosis of schilder

A

large, sharply outline asymmetrical foci demyelination in cerebral hemispheres
causes steady or unremitting or punctuated by a series of episodes

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17
Q

diagnostic findings for acute and tumefactive ms

A

table 5

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18
Q

diagnostic findings for schilder ms

A

table 6 – mbp

19
Q

what is concentric sclerosis of balo

A

similar to schilder ms

have alternating bands of destruction and preservation of myelin in a series of concentric rings

20
Q

most helpful ancillary exam in ms diagnosis

A

mri to se ms plaques

21
Q

mri findings for ms in brain and spinal cord

A

brain: ms plaques are hyperintense
dawson’s fingers - multifocal, well demarcated, oval, or linear radially oriented lesions that denote ms

spinal cord: motor weakness

22
Q

csf findings for ms

A

oligoclonal bands for confirmation of ms (but not specific and sensitive)
mononuclear pleocytosis
compute igg index

23
Q

evoked potentials in ms

A

visual evoked responses may show prolonged latency
brainstem auditory responses can show which part of auditory response is affected
somatosensory response is delayed

24
Q

common locations of cerebral plaque

A

lateral ventricle
optic nerve
spinal cord
cerebellum

25
Q

____ signifies the demyelination process

A

hypopigmentation

26
Q

what is an ms attack

A

neurological disturbance
at least 24 hour duration in absence of fever or infection
more acceptable if it’s multiple episodes over 24 h

27
Q

mcdonald criteria for ms

A

table 7

28
Q

what is progressive relapsing ms

A

initial manifestations improve partially or completely but recur or form new ones in different parts of the nervous system
steady decline or disease progression

29
Q

primary progressive ms

A

> 40 yo, disease steadily progresses

no relapses or remissions

30
Q

relapsing/remitting ms

A

unpredictable attacks followed by period of remission

may or may not leave permanent deficits

31
Q

secondary progressive ms

A

rrms that develops into progressive type after a certain time
suddenly begins to decline and loses period of remission and relapses

32
Q

treatment for acute demyelinating optic neuritis

A

currently no treatment

short course iv methylprednisolone + 2 weeks oral prednisone to improve recovery (but visual function decline is same)

33
Q

treatment for acute exacerbations of ms

A

3-5d pulsated high dose methylprednisolone
followed by oral prednisone over 11-14 d

improvement: maintain steroids
no improvement: plasma exchange

34
Q

available immuno-modulators

A 
beta-interferon 1a/1b
glatirament acetate (mimis myelin basic protein)
monoclonal antibodies
immunosuppressants
plasma exchange
35
Q

what is neuromyelitis optica

A

simultaneous or successive and severe involvement of optic nerves and contiguous segments of spinal cord
no cerebral demyelination

36
Q

clinical presentation of devic’s disease

A

subacute to acute onset of blindness in 1 or both eyes, followed within days or weeks by severe transverse or ascending myelitis
necrotizing and cavitary spinal cord lesions

37
Q

lab findings in devic’s disease

A

(+) antibody towards aquaporin 4 water channel protein

(-) oligoclonal bands in the csf

38
Q

distinguishing factor of adem to acute ms

A

multifocal meningeal infiltration

39
Q

manifestations of adem

A

more common in children
previous viral infection or vaccination or tetanus antitoxin
encephalitis: acute fever, headache, confusion, somnolence, convulsion, stiff neck
cerebritis: acute ataxia
acute transverse myelitis: paraparesis or quadriparesis, areflexia, sensory, bowel and bladder problems

40
Q

most fulminant form of demyelinating disease

A

acute necrotizing hemorrhagic encephalomyelitis

41
Q

manifestations of anhe

A

previous respiratory infection (mycoplasma pneumoniae)
headache, fever, stiff neck, confusion
cerebral and brainstem signs: focal seizures, hemiplegia, quadriplegia, pseudobulbar paralysis, progressively deepening coma

42
Q

diagnosis of anhe

A

peripheral leukocytosis
increased icp
ct/mri showing edema and hemorrhage

43
Q

what is graft vs host disease

A

brain inflammation after bone marrow transplant

s/sx appear months or years later: subacute hemiparesis, seizures, behavioral changes, atazia

08 Neuro (24 decks)

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