MS Flashcards

1
Q

what are the 4 demyelination mechanisms

A
  1. oligodentrocyte or schwann cell death
  2. interference with myelin synthesis
  3. interference with myelin replacement
  4. myelin destruction immune mediated
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2
Q

what is the composition of myelin?

A

70% lipids

30% proteins (myelin basic protein, myelin associated glycoprotein, oligodendrocyte glycoprotein)

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3
Q

what two things contain myelin?

A
schwann cells (PNS)
oligodendrocytes (CNS) 

both from neural crest

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4
Q

shwann cells and oligodendrocytes are components of what systems

A
schwann cells (PNS)
oligodendrocytes (CNS)
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5
Q

how much does each schwann cell myelinate the axon?

A

1mm

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6
Q

how many axonal segments do oligodendrocytes myelinate

A

each oligo. myelinates 60 axonal segments

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7
Q

conduction velocity of an axon is proportional to its _________ and the ditance between________

A

diameter

distance between ranviers nodes

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8
Q

what is the speed of an action potential

A

120 m/s

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9
Q

perivascular and perivenulat immune infiltration/ plaques in white matter

A

demyelination

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10
Q

most common immune mediated inflammatory demyelinating disease of the CNS

A

MS

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11
Q

MS
age group affected
sex affected

A

most common neurologic disease among young adults
incidence is highest from ages 20-40 but can start in childhood or after 50

female to male 7:3 (2.3:1)

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12
Q

prevelance for MS decreases with?

A

proximity to equator

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13
Q

Genetic risk for MS for
general population?
one parent or 1st degree relative affected?
both parents?

A

0.1%
4%
20%

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14
Q

MS is associated with major __________

A

histocompatibility complex II (MHC II) and certain DR2 haplotypes

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15
Q

most frequent cause of permanent disability in young adults aside from trauma (among CNS disorders)

A

MS

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16
Q

mean age of ms

A

28-31

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17
Q

what is the best theory explaining the demyelination in MS

A

begins as an inflammatory immune mediated disorder characterized by autoreactive lymphocytes –> later the disease is dominated by microglial activation and chronic neurodegeneration

18
Q

immunopath of ms/ inflammation in conjunction with BBB disruption –> inflammatory T cells, B cells, and macrophages are typically seen–> presence of IgG and IgM oligoclonal bands in ___________ but not in the ___________

A

Cerebrospinal fluid

serum

19
Q

immunopath of ms/ myelin reactive ______ are found in MS _______ and in the ______ and the peripheral circulation of patients with MS

A

T cells

MS plaques

CSF

20
Q

no direct proof that an autoimmune causes MS as ……

A

no specific autoantibodies or autorecessive T cells directed against self antigen in the CNS can passively transfer MS to experimental animals

21
Q

how does MS appear or look like

A

multiple discrete pink or gray areas that have a hard or rubbery texture are id’d within the white matter of the brain and spinal cord

22
Q

in MS lesions can occur at any site within the CNS, they have a predilection for involvement of the _______, _______, ______, and______

A
juxtacortical and periventricular white matter
the corpus callosum
optic nerves and the dorsal spinal cord 
brain stem
cerebellum
23
Q

the characteristic neuropathologic feature of MS is the presence of focal ____________ within the CNS accompanied by variable degrees of _______ and ______ with partial preservation of axons

A

focal demyelinated plaques

degrees of inflammation and gliosis

24
Q

On MRI we can see that MS can cause diffuse damage of _____________ both of which are associated with a progressive loss of _______

A

normal appearing white matter (NAWM)
normal appearing gray matter (NAGM)

Loss of brain volume

25
Q

what are the target proteins in MS

A

myelin basic protein (MBP)

Proteolipid proetin (PPL)

Myelin associated gycoprotein (MAG)

26
Q

what paraclinical tests provide support in the diagnosis of MS

A

MRI
spinal fluid
evoked potentials

27
Q

what are the diagnostic criteria in order to diagnose MS

A

dissemination in time and space: evidence that damage has occurred in at least two separate areas of the CNS at different points in time
There must be no other explination

28
Q

what are the disease types in MS

A

clinically isolated syndrome
relapsing remitting MS 85%
primary progressive MS 15%
secondary progressive –> most people diagnosed with RRMS will eventually transition to SPMS

29
Q

a first neurological event suggestive of demyelination in ms

A

clinically isolated syndrome

30
Q

individuals with Clinically isolated syndrome are at high risk of __________

A

developing definite MS if the neurological event is accompanies by multiple clinically silent lesions on MRI typical of MS

31
Q

inflammatory, demyelinating conduction that causes acute, usually mononuclear visual loss. highly associate with MS

A

optic neuritis

32
Q

optic neuritis is a presenting feature of MS in ___% of patients and occurs in ___% during some course of disease

A

15-20

50

33
Q

in optic neuritis due to MS _______ is identified at symptom onset and precedes changes in the cerebrospinal fluid

A

systemic T cell activation

34
Q

B cell activation against myelin in MS is not seen in ________ but can be seen in ________

A

peripheral blood

cerebrospinal fluid

35
Q

previously known as devic disase

A

neuromyelitis optica

36
Q

inflammatory disorders of the CNS and axonal damage predominantly targeting optic nerves and spinal cord/ disease specific serum NMO-IgG antibody selectively binds aquaporin -4

A

neuromyelitis optica

37
Q

traditionally considered a variant of MS, it is now recognized as a distinct clinical entity based on unique immunologic features

A

neuromyelitis optics

38
Q

a water channel protein highly concentrated in spinal cord gray matter, periaqueductal and periventriculat regions, and astrocytic foot processes at the BBB

A

neuromyelitis optica :AQP4

target antigen of NMO-IgG

39
Q

AQP4 in MS
vs
AQP4 in neuromyelitis optic (NMO)

A

in MS lesions the distribution of AQP4 protein expression depends upon the stage of demyelination

while in NMO lesions there is a loss of AQP4 expression that is unrelated to the stage of demyelation

40
Q

what are inital symtpoms of MS

A

sensory disturbances, parenthesis, dysesthesias (burning, gritty, sandy, electrical, or wet sensations) , weakness, visial loss, abnormal gait, diminished dexterity, diplopia, ataxia, vertigo, or spinchter disturbances

Motor disturbances: weakness, spasticity, hyperreflexia babinski

non specific fatigue headache

41
Q

a specific gaze abnormality characterized by impaired horizontal eye movements with weak adduction of the affected eye, and abduction nystagmus of the contralateral eye

IN MS

A

internuclear ophtalmoparesis