demyelinating degenerative diseases Flashcards

1
Q

demylinating diseases are _ conditions that preferentially damage _ with relative presevation of _

the CNS has a limited capacity to regenerate _

A

acquired conditions

myelin

axons

myelin

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2
Q

Multiple Sclerosis has distinct episodes of neurologic deficits sperated in _ attributable to white matter lesions that are seperated in _

A

time

space

(there are new, old, and in between lesions that occur in different parts of the brain)

AUTOIMMUNE

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3
Q

in MS there are _ and _ episodes of variable duration

A

relapsing and remmitting

(neurologic def followed by gradual partial recovery)

the freq of relapses tend to decrease with the longer youve had the disease but there is steady neurologic deterioration**

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4
Q

common signs of MS

inital symptom:

brainstem:

spinal cord:

A

inital symptom: unilateral visual impairment** (optic neuritis)**

brainstem: ataxia and nystagmus (CN signs)

spinal cord: Motor and sensory impairment of the trunk and limbs with a loss of bladder control

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5
Q

who is more likely to get MS

genetic MS is 15 fold higher in first degree relatives what are the receptor genes and HLA genetic links to MS?

Environmental risk factors:

Inflammation risk factors:

A

women are more likely

genetic: receptors; IL-2 and IL-17, HLA DR2

Environmental: away from equator, low vitamin D

inflammation: chronic

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6
Q

inflammation causing MS is initiated by _ and _ cells that react against self _ antigens and secrete cytokines.

A

CD4+/TH1 and TH17 cells

myelin

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7
Q

TH1 cells secrete _ which activated _

A

IFN gamma which activates macrophages

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8
Q

TH17 cells promote recruitment of _

A

leukocytes

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9
Q

MS plaques are usually infiltrated by?

A

T cells (CD4+)

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10
Q

MS has damage to the _ matter (can extend though)

grossly the finding of MS is?

A

white matter

multiple well circumscribed slightly depressed plaques that is firmer than the surrounding white matter (sclerosis)

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11
Q

where is the pathology in MS located in the brain

A

ADJACENT TO THE VENTRICLES and optic nerves (optic neuritis)

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12
Q

what is the stain you can do in MS to show the presence or absence of myelin

A

Luxol Fast Blue stain

  • blue= myelin
  • pink= no myelin
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13
Q

what cells are present in MS and what is preserved in MS- what stain can you use to see this preservation?

A

lipid laden macrophages ( as we breakdown myelin lipids are released and macrohpages comes to clean it up)

axons are preserved- can see with a neurofilamtent IHC stain

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14
Q

what is a stain for macrophages we can see in MS with the increase in lipid laden macrophages

on histology of MS you can also see _ cuffing

A

CD68+ (stain for macrophages-will be increased)

perivascualar cuffing

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15
Q

there are 3 stages to a MS plaque

active, inactive and shadow

active plaque has _ myelin breakdown with abundant _ that contain lipid rich _ positive debris. There is perivascular and inflammatory infiltrate at the _ edge of the plaque. There is relative preservation of _ within the plaque and depletion of _

Inactive plaque is _ and inflmmation _. There is no _, there is a decrease in _ and a slight decrease in _. In this stage there is _ proliferation and gliosis.

Shadow plaque in an in between stage and the white matter is _ sharply circumscribed. There is abnormally thinned out _ sheaths and partial/incomplete _ by the surviving oligodentrocytes

A

ongoing, macrophages ,PAS, outer, axons, oligodendrocytes

silent, disappears, myelin, oligodetrocyte nuclei, axons, astrocyte

NOT, myelin sheaths, remyelinization

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16
Q

CSF examination findins for MS

protein
cells
immunoglobulin

A

protein: elevated
cells: pleocytosis
immunoglobulin: IGG increased/ oligoclonal iGG bands in the gamma region!

17
Q

what is neuromyelitis optica (NMO)

A

this is a disease related to MS that develops 10-50% of the time

this is bilateral neuritis with spinal cord demylination

devic disease

18
Q

CSF of neuromyelitis NMO

A

neutrophils, bacterial/turbid, increased opening pressure

19
Q

antibodies in NMO

A

Aquaporins

20
Q

TX of NMO

A

plasmapharesis (decrease antibody burden)

  • glucocorticoids for acute attacks
21
Q

Acute disseminate encephalomyelitis (ADEM) is a _ encephalomyelitis that is a diffuse _ demylinating disease that follows a _ infection of a _ immunization.

symptoms includes headache, lethargy, and _ after the antecedent event

morphology: _ discoloration around the white matter, _ loss with relative presevation of _ , accumulatio of _, lesions are _ (biphasic/monophasic)

A

perivascular

monophasic

viral; viral

come

graysih

axons

lipid laden macrophages

monophasic

22
Q

Acute necrotizing hemorrhagic encephalomyelitis (ANHS) is a fulminnate syndrome of CNS _ with similar histology to ADEM. It occurs in _ (children/elderly) and is caused by a recent _ _ infeciton. It is fatal in most.

A

demyelination

children

upper respiratory

23
Q

central pontine myelinolysis is also known as?

A

osmotic demyleination syndrome

24
Q

central pontine myelinolysis is a loss of _ with a symmetric pattern involving the _ _ and the portions of the _ _. There is _ loss without the evidence of inflammation

symptoms include: (the d’s)

it occurs 2-6 days after overly rapid correction of _

there is a severe electrolye or osmolar imbalance

**liver transplant can help

A

myelin

basal pontis

pointine tegementum

myelin loss without inflammation

dysphagia, dysarthria, diplopia, and LOC

hyponatremia