N: Demyelination & Csf

Front 1

How is MS defined?

What cells are postulated to be the attackers?

Back 1

progressive AI dz of the DNS. defined clinically by the occurence of multiple discrete episodes of neurologic dysfunction separated in time and the presence of demyelinating lesions separated by anatomic space.

CD4+ T cells targeted against the myelin sheath.

Front 2

What is the most common demyelinating disorder? M/F predom?

Back 2

MS.
2:1 female:male

Front 3

What is seen in pathological specimens w/ MS?

What are Dawson fingers?

CSF samples?

Back 3

irregularly shaped plaques w/i white matter of brain & spinal cord, especially in periventricular regions.

Periventricular lesions (finger like) --> think MS!

mildly elevated protein & lymphocytic pleocytosis

Front 4

If CSF is examined by electrophoresis in MS pts, what is often seen? What produces then and where are they produced?

Back 4

oligoclonal bands: antibodies produced by B cells in the nervous system.

Front 5

What is often the 1st reported sx of MS? Commonly unilateral or bilateral?

Back 5

optic neuritis
- unilateral

Front 6

In a pt w/ suspected MS, CN palsies, ataxia, and nystagmus are often seen due to MS plaque formation where?

Back 6

brainstem.

Front 7

What is the most common pattern of dz progression i/ MS pts?

How do pts ultimately die?

Back 7

relapsing-remitting MS: pt has discrete episodes of neurologic dysfunction owing to new plaque formation. Each episode is followed by slow, partial recovery.
- This can occur over months/years

--> dz can then switch to 2ndary progressive form: disease relentlessly progresses w/o the benefit of remission.

illnesses secondary to neurological dysfunction (pneumonia 2ndary to aspiration).

Front 8

What is Guillain-Barre syndrome, and how can it lead to pt death?

Back 8

immune-mediated peripheral neuropathy that can lead to death owing to respiratory muscle paralysis

Front 9

What is the pathogenesis of Guillain-Barre syndrome?

Postulated dz mechanism?

Back 9

large proportion of cases are preceded by a flu-like syndrome. Most cases are epidem assoc w/ prior CMV infec, EBV, campylobacter jejuni, or mycoplasma pneumoniae.

T cell response w/ subsequent peripheral demyelination as a result of macrophages actv.

Front 10

What does the CSF often look like in pts with Guillain-Barre syndrome?

Clinical picture of the dz?

Back 10

albumin-cytologic dissociation: elevated pro w/o pleocytosis.

rapidly progressive ascending paralysis... can last from days to weeks, followed by gradual, full recovery.

Front 11

Oligodendrocytes myelinate one segment of ____ axons. Schwann cells?

What are the lipids used in myelin? Proteins?

Back 11

one seg. of many axons. Schwann: one seg of ONE axon.

- cholesterol, sphingomyelin, galactocerebroside, sulfatide
- MBP, MAG, PLP (CNS), PO (PNS)

Front 12

In demyelinating dz, are the axon's typically preserved?

What are the "chronic Lesions" seen in MS pts? "Acute lesions"?

Does the BBB stay intact?

Back 12

yes, as a rule.

reactive astrocytes --> fibrillary astrocytic gliosis.

have more to do w/ just he absence of myelin

no, there is often breakdown of the BBB

Front 13

Label the following demyelinating dz as either immune mediated, toxic, viral, or vascular:
1) MS
2) Acute disseminated encephalomyelitis
3) Acute hemorrhagic leukocephalitis
4) osmotic myelinolysis
5) subcortical arteriosclerotic leukoencephalopathy
- alternate name for this?
6) Progressive multifocal leukoencephalopathy (PML)
7) AIDS

Back 13

1) immune
2) immune
3) immune
4) toxic
5) vascular
- Binswanger Dz
6) Viral
7) Viral

Front 14

What is PML? Etiologic agent?

Population?

Early vs late stage?

Back 14

progressive multifocal leukoencephalopathy; caused by papova virus (JC strain) infection.

occurs as a complication for immune-compromised pts w/ a relatively short clinical course.

Early: mult. foci of demyelination in subcortical white matter
Late: large necrotic lesion involving cortex and white matter

Front 15

What are some signs of AIDS leukoencephalopathy?
- seen on histology?
- perivascularly?
- inflammatory cell response?
- associated with AIDS dementia?

Back 15

diffuse pallor of cerebral white matter on myelin stain
- very little histological evidence of myelin degeneration

perivascular presence of multinucleated giant cells expressing HIV viral antigens

very little inflammatory response (acute)

associated w/ AIDS dementia (loss of neurons)

Toxic cytokines may play a role.

Front 16

A rapid correction of serum electrolyte imbalance that results in rapid rise in serum Na can cause what demyleinating dz?
- area of effect?
- effect on oligodendrocytes?

Back 16

central pontine myelinolysis (osmotic myelinolysis)
- center of pontine base (also LGN and IC/EC in some cases)
- decreased oligodendrocytes w/i demyelinating lesion

Front 17

What are the leukodystrophies? Causes?
- demyelination affects CNS or PNS?

Back 17

inherited metabolic dz's primarily affecting white matter
- mut of the gene for the myelin protein or specific enzyme for myeline lipid metabolism.
--> perturbation in myelin sheath formation.

- both

Front 18

What is a good dx marker for ALD and AMN? (adrenoleukodystrophy & adrenomyeloneuropathy)
- relevant genetic inheritence? Particular defect?
- Difference between ALD and AMN?
- CNS or PNS affected?

Back 18

- v. long chain fatty acids in tissue (and in macrophages, adrenal cortical cells, and leydig cells in testis)
- X-linked; ATP-b/ transport protein ALDP
- occipital white matter (ALD); brainstem/spinal cord (AMN)
- Both

Front 19

What is the name of the dz characterized by a defect in Arylfulfatase A?
- inheritance pattern?
- what accumulates in CNS cells and visceral organs (and in macrophages) in this dz?
- CNS or PNS affected?

Back 19

Metachromatic leukodystrophy
- autosomal recessive
- sulfatide
- both

Front 20

What is Krabbe Dz?
- what is seen in cells (and macrophages) in this dz?
- correspondingly, where is the defect?
- inheritance?
- CNS or PNS affected?
- what unique cells are seen in the demyelinating white matter?

Back 20

Globoid cell leukodystrophy (GLD)
- galactocerebroside
- galactosylceramidase (galactocerebrosidase)
- autosomal recessive
- both
- globoid cells

Front 21

What dz is more common in Jewish infants, in which myelin vacuolation is followed by demyelination?
- CNS or PNS affected?
- inheritance?
- deficiency in what?
- can is cause macrocephaly?

Back 21

Canavan Dz (Spongy degeneration of the CNS)
- just CNS
- autosomal recessive
- aspartoacylase
- yes.

Front 22

Define and differentiate normal pressure hydrocephalus, noncommunicating, and communicating.

Back 22

noncomm: obstruction w/i the ventricular system

Comm: blockage w/i the subarachnoid space (eg adhesions post-meningitis)

Norm: CSF is not being absorbed by arachnoid villi

Front 23

What can occur secondary to postraumatic meningeal hemorrage that can make the pt have progressive dementia, ataxic gait, and urinary incontinence?

Back 23

normal-pressure hydrocephalus
"Wacky, wobbly, and wet"

Front 24

Approximately ____ mL of CSF are in the ventricles and subarachnoid space?

Tight junctions cell walls where form part of the BBB?

What are the 3 components of the BBB in brain vessels?

Back 24

~130mL

choroid plexus

Astrocytes via their foot processes that adhere to the outer surface of the capillary walls.

Basement membrane

Endothelial tight junctions

Front 25

What is the CSF profile for:
- normal
- bacterial meningitis
- viral meningitis
- viral encephalopathy
- subarachnoid hemorrhage

answer in: color, cellularity, protein, and glucose

Back 25

- normal: clear/colorless, <5 cell/uL (lymphocytes), <45mg/dL protein, ~66% of Blood glucose (50-75mg/dL)

- cloudy, >1000/uL PMN predom, elevated >500, Reduced <45mg/dL

- clear/cloudy; <250 lympho, slightly elevated <150, normal range

- clear/cloudy, 25-500 lymphocytes, slightly elevated <100, normal

- frankly bloody, xanthochromic; RBCs, normal or slightly elevated; normal range.