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Demyelinating disease Flashcards

(46 cards)

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1
Q

What is the function of myelin

A

required for saltatory AP conduction

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2
Q

What is the consequences of myelin loss

A

renders an axon electrically non-functional

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3
Q

What is myelin formed by in the CNS

A

Oligodendrocytes

*multiple axons invested

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4
Q

What is myelin formed by in the PNS

A

Schwann cells

*single axon invested

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5
Q

What is primary demelination

A

disorders which damage the oligodendroglia or schwann cells with their myelin sheaths directly

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6
Q

What is secondary demyelination

A

disorders which damage the axons that lead to myelin sheath destruction

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7
Q

Name two central myelin disorders

A 
Multiple sclerosis (MS)
Post-infectious encephalomyelitis
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8
Q

Name two peripheral myelin disorders

A

Guillain-Barre syndrome

Charcot-Marie-Tooth disease

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9
Q

Name a set of disorders characterized by both central and peripheral loss of myelin

A

Leukodystrophies

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10
Q

MS is an inflammatory disease predominantly afflicting

A

females between 20-40 years old

  • best regarded as an autoimmune disease in which teh oligodendroglia are targetted
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11
Q

MS is clinically denoted by lesions disseminated in

A

time and space

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12
Q

What is dissemination in time mean

A

episodes of demylelination are separated by periods of remmission

*attack-remission-relapse-remission

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13
Q

What is dissemination in space mean

A

demyelination occuring in multiple sites in the CNS

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14
Q

What is essential for a confident diagnsosis of MS

A

lesions disseminated in space and time

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15
Q

What are the areas in the CNS affected by MS

A

Long myelinated pathways

  • motor/sensory tracts
    cerebellar tracts
    optic nerve tracts/radiations
    medial longitudinal fasciculus
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16
Q

What are signs of long myelinated pathway injuries

A

weakness-motor
paresthesias, numbness-sensory
incoordination-cerebellar
central scotomata/diplopia-optic tract/radiations

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17
Q

What are gross signs of MS in the CNS

A

Irregular light pink-grey areas of demyelination called plaques

*especially around the ventricles
grey- d/t loss of lipid in myelin
pink- seen in acute loss of myelin

*No definitive lab studies, must have dissemination of time/space

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18
Q

What are 5 advantages of MRI

A

allows detection of asymptomatic plaques

Aids in diagnosis

Allows disease activity to be followed through time

Aids in therapeutic trials/management

*Can demonstrate dissemination in space and time

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19
Q

Demyelination in nerve root entry zone leads to what 3 paroxysmal symtoms

Study These Flashcards
A

Trigeminal neuralgia
Hemifacial spasm
Radicular pain

  • zone is transition b/t CNS and PNS myelin
20
Q

What are 6 microscopic signs of MS

Study These Flashcards
A 
perivascualar lymphocytic infiltrates
loss of oligodendroglia
myelin sheath stripping
macrophage infliltration
astrogliosis
sparing of axons
21
Q

What are the two tx strategies for MS

Study These Flashcards
A

immonosuppression with corticosteroids during the ACUTE phase

immunomodulation with Beta interferon

22
Q

Post-infectious demyelination (encephalomyelitis) is characterized by what 5 features

Study These Flashcards
A 
T-cell (immune) mediated hypersensitivity 
Monophasic
perivenular lymphocytes
marked swelling 
scattered foci of hemorrhage
23
Q

Post-infectious encephalomyelitis follows exposure to

Study These Flashcards
A

antigen (viral or vaccinaton)

24
Q

What are symptoms associated with PI encephalomyelitis

Study These Flashcards
A 
h/a
neck stiffness
lethargy
coma 
DEATH

*symptoms develop within days

25
If focal hemorrhage and necrosis is seen with PI encephalomyelitis, the term is changed to
Acute hemorrhagic leukoencephalopathy
26
Guillain-Barre syndrome is similar to PI encephalomyelitis in what sense
it tends to follow viral infections or vaccinations
27
Gullian-Barre syndrome is different than PI encephalomyelitis in what two ways
the immune-mediated demyelination is confinded to the PNS Multiphasic- relapses are possible
28
Guillian-Barre syndrome preferentially targets
peripheral motor systems with no sensory involvement *mild weakness with loss of DTR from lower extremity >>trunk and arms>>respiratory muscle involvement>>respiratory failure
29
What is the pathological features of Guillian-Barre syndrome
focal accumulations of lymphocytes and macrophages throught the peripheral nerves. Myelin lamellae are split and digested by macrophages, leaving the schwann cells and axons intact for remyelination and recovery
30
With repeat bouts of demyelination and remyelination of peripheral nerves in Gullian-Barre syndrome, what is seen
Onion-bulbing *thicknening of the myelin sheath
31
Name the three GENETIC disorders of central and peripheral myelin
Metachromatic leukodystrophy Adrenoleukodystrophy Krabbe's disease
32
Leuko/dystrophies means
disorders of the white matter of brain and/or spinal cord
33
Metachromatic leukodystrophy by a deficiency of what enzyme
arylslufatase A
34
What is the characteristic appearance under the micropscope of metachromatic leukodystrophy
Metachromasia *samples stain BROWN
35
Where is the brown stain seen in the CNS/PNS
``` neurons macrophages schwann cells (PNS) free tissue space extraneural tissues ```
36
What does the brain on coronal sections reveal in metachromatic leukodystrophy
shrunken, yellow, white matter with sparing of the subcortical myelin
37
When is the onset of metachromatic leukodystrophy
first 4 years of life * progressive motor impairment, mental deterioration, and peripheral neuropathy
38
How is metachromatic leukodystrophy diagnosed
measuring urinary arylsulfatase A
39
What is the genetype of Metachromatic leukodystrophy
Autosomal recessive
40
What deficiency is seen with Krabbe's disease
Galactoceramidase
41
What is the gross and microscopic similarities and differences b/t Krabbe's disease and metachromatic leukodystrophy
grossly similar in shrunken, yellow, white matter demylination with sparing of subcortical myelin microscopically different in absence of metachromasia and presence of LARGE, MULTINUCLEATED globoid cells
42
When do symptoms begin with Krabbe's disease
early infancy *death within a year
43
What tx is available that will prevent the onset of CNS problems or reverse CNS manifestations of Krabbe's disease
Bone marrow transplantation
44
What genotype is seen with Krabbe's disease
Autosomal recessive
45
What deficiency is seen with adrenoleukodystrophy
transporter of lignoceroyl-CoA ligase *peroxisomal membrane protein (pmp)
46
What are the 3 clinical features of adrenoleukodystrophy
inflammation demyelination hypoadrenalism

Nervous system (7 decks)

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