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
what are the target proteins in MS
myelin basic protein (MBP) Proteolipid proetin (PPL) Myelin associated gycoprotein (MAG)
26
what paraclinical tests provide support in the diagnosis of MS
MRI spinal fluid evoked potentials
27
what are the diagnostic criteria in order to diagnose MS
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
what are the disease types in MS
clinically isolated syndrome relapsing remitting MS 85% primary progressive MS 15% secondary progressive --> most people diagnosed with RRMS will eventually transition to SPMS
29
a first neurological event suggestive of demyelination in ms
clinically isolated syndrome
30
individuals with Clinically isolated syndrome are at high risk of __________
developing definite MS if the neurological event is accompanies by multiple clinically silent lesions on MRI typical of MS
31
inflammatory, demyelinating conduction that causes acute, usually mononuclear visual loss. highly associate with MS
optic neuritis
32
optic neuritis is a presenting feature of MS in ___% of patients and occurs in ___% during some course of disease
15-20 | 50
33
in optic neuritis due to MS _______ is identified at symptom onset and precedes changes in the cerebrospinal fluid
systemic T cell activation
34
B cell activation against myelin in MS is not seen in ________ but can be seen in ________
peripheral blood cerebrospinal fluid
35
previously known as devic disase
neuromyelitis optica
36
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
neuromyelitis optica
37
traditionally considered a variant of MS, it is now recognized as a distinct clinical entity based on unique immunologic features
neuromyelitis optics
38
a water channel protein highly concentrated in spinal cord gray matter, periaqueductal and periventriculat regions, and astrocytic foot processes at the BBB
neuromyelitis optica :AQP4 target antigen of NMO-IgG
39
AQP4 in MS vs AQP4 in neuromyelitis optic (NMO)
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
what are inital symtpoms of MS
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
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
internuclear ophtalmoparesis