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Adreno

-

leuko ' dystrophy TW tissue Adrenal

Gland degradation .

.

Rare genetic condition caused by

Mutation in ABC Dt Gene in X - c hmm

-

O some

H,

o

Progressive loss of white matter in Nervous

system °

Degradation of Adrenal Gland

㱺 ABC Dt

gene

in located at [Reduced white matter]

Xqzs and show x- linked Recessive Inheritance

°

In more detail , it in a disorder , peroxisomal fatly acid

p -oxidation which results in accumulation g Very long fatty Acids .

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°

These

very long fatty acids accumulate in tissue throughout the

body and affect :

•

Myelin in CNS

°

Adrenal Cortese

•

Leydig Cells in Testes .

•

Immune Cells

• Males are more prone

to ALD as they are hemiaygous and ,

females are often symptomatic later in life .

Type :

• 4 types -

( A MN )

in Childhood Cerebral Type (cc ALD ) (3) Adreno

my

elon

europathy

←, Adult Cerebral Type ( ac AID ) ( 4 , Adrenal Insufficiency

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Symptoms :

Adnezoemyelopathy (most common)

°

Adrenal Problems

•

Difficulty controlling Urination

•

Possible worsening muscle

weakness or leg stiffness .

°

Problems with thinking speed a

Visual

memory -

other symptoms ( Addison type)

° Coma . Inc skin Pigmentation • Decreased Appetite . loss g wtf muscle mass

° Vomiting