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RITE (Dr. Cheng, Berkowitz tables and path) > Leukodystrophy > Flashcards

Leukodystrophy Flashcards

1
Q

4 Major categories of leukodystrophy

A

Lysosomal storage disease, Peroxisomal
Mitochondrial
Other (canavan,PMD,alexander etc)

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2
Q

Three types of lysosomal storage disease

A

Metachromatic leukodystrophy
Krabbe
Mucopolysaccharidosis

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3
Q

Two types of peroxismal disorders

A

ALD

Zellweger

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4
Q

Three types of Mitochondrial dysfunction

A

Leigh
MELAS
Kearn-sayre

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5
Q

Metachromatic enzyme def

A

Arylsulfatase A

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6
Q

Krabbe enzyme def

A

Galactocerebroside-aspartoacylase

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7
Q

ALD enzyme def

A

acyl-CoA synthetase

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8
Q

two disoders that do NOT enhance on MRI

A

Metachromatic and PMD

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9
Q

Trigoid pattern (2) but what is the difference in locavtion

A

metachromatic and alexander (frontal involvement)

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10
Q

High CT attenuation

A

Krabbe

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11
Q

Migrating infarct

A

MELAS

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12
Q

Two disorders that include macrocephaly

A

MELAS and alexaNDER

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13
Q

Two x-linked disorder

A

ALD and PMD (all others are autosomal recessive)

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14
Q

ALD stands for

A

adrenaleukodystrophy

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15
Q

PMD stands for

A

Pelizaeus-Merzbacher disease

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16
Q

All leukodystrophies

A

McL, Krabbe, NPd, Fabry, Gm1/2, Mucopolysac, Fuco,Mucolipid, Wolmann,Ceroid lipofucinosis

17
Q

What does metachromatic spare early ?

A

U fibers

RITE (Dr. Cheng, Berkowitz tables and path) (17 decks)

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