N 38

Front 1

Define leukencephalopathies

Back 1

- white matter disorders b/c toxin, nutrition

Front 2

Define leukodistrophy

Back 2

- genetic, progressive cause of myelin/myelin cell disorder

Front 3

Demyelinating process

Back 3

- loss/damage to myeline either primary (instrinsic) or secondary (damage to neuron/axon)

Front 4

Dysmyelinating process

Back 4

- abnormal myelin from onset

Front 5

What are the 4 CNS demyelinating disorders?

Back 5

1. Multiple sclerosis
2. acute disseminated encephalomyelitis
3. progressive multifocal leukoencephalopathy
4. central pontine myelinolysis

Front 6

Multiple Sclerosis

Back 6

- neuro deficits separated by time and white matter lesions separated in space
- macros and CD8 do most of damage to myeline
**Autoimmune
- Morph: gray spots in white matter with active or inactive plaques

Front 7

Acute Disseminated Encephalopmyeitis (ADEM)

Back 7

***hypersensitive
- Clinical: monophasic demyelinating, 1-2 weeks after infection/vaccine, most recover
-Path: multiple aras w/o myeline

Front 8

Acute hemorrhagic leukoencephalitis

Back 8

- sub category of ADEM
- more severe, possible immune problem, thrombosis, more often fatal

Front 9

Progressive Multifocal Leukoencephalopathy (PML)

Back 9

- JC Virus
- path- oligos are targeted and have intranuclear enlargement

Front 10

Central Pontine myelinolysis

Back 10

- clinical: quadriparesis after hyponatremia
- path: demylination of basil pons

Front 11

What are the 5 CNS Dysmyelinating disorders?

Back 11

1. Metachromatic Leukodystrophy
2. Adrenoleukodystrophy
3. Krabbe's/Globoid cell Leukodystrophy
4. Canavan's
5. Alexander's

Front 12

What do all CNS Dysmyelinating disorders have in commo?

Back 12

- widespread white matter demylination
- Gross: atrophic white with texture change and discoloration

Front 13

Metachromatic Leukodystrophy

Back 13

- Defect: decrease in aryl-sulfatase
- Path: metachromatic material

Front 14

Adrenoleukodystrophy

Back 14

Defect: abnormal peroxisome
Path: inflammatory leukodystrophy and adrenal atrophy

Front 15

Krabbe's/Globoid cell Leukodystrophy

Back 15

Defect: decerase in Galactocerebrosidase
Path: perviascular enlarged histocytes with lipids

Front 16

Canavan's

Back 16

Defect: decrease aspartoacylase
Path: vacuoles in white

Front 17

Alexander's

Back 17

Defect: GFAP gene mutation
Path: accumulation Rosenthal fibers in subcortical/periventricular around vessels

Front 18

What are the4 non-inflammatory demyelination

Back 18

1. diptheria
2. hereditary motor and sensory neuropathies type I
3. herditary motor and sensory neuropathies type III
4. Refsum's

Front 19

Diptheria

Back 19

Define: rare in US
Path: toxin demyelination of Schwann

Front 20

Hereditary motor and sensory neuropathies (HMSN Type I)

Back 20

define: AD, distal weak an dsensory impaired
Path: onion bulbs

Front 21

Heredidtary motor and sensory Neuropathies (HMSN type III)

Back 21

Define: AR, aggressive sensorimotor neuropathy, may present with arthogryposis multiplex congenita
Path: onion bulbs

Front 22

Refsum's

Back 22

Clinical: chronic distal symmetrical sensorimotor neuropathy, increase in phytanic acid
Path: onion bulbs

Front 23

What are teh 2 inflammatory demyelination disorders?

Back 23

1. Guillain-Barre
2. Chronic Idiopathic Demyelinating POlyneuropathy (CIDP)

Front 24

Guillain-Barre

Back 24

- most common acute paralytic, rapid onset, incidting event, cell mediated and huoral attack of myelin
Path: demylinationg and chronic inflammation

Front 25

Chronic Idiopathic Demyelinating POlyneuropathy (CIDP)

Back 25

Clinical: motor manifestations, longer than 8 weeks
Path: onion bulbs and chronic inflam

Front 26

What is the characteristic of PNS myelin disorders that are acute?

Back 26

naked axons

Front 27

What is the characteristic of chronic PNS myelin disorders morphology?

Back 27

onion bulbs