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Adrenoleukodystrophy

OVERVIEW:

A sphingolipidosis which combines the features of leukodystrophy and Addison's disease. A rare, sex-linked recessive metabolic disorder that occurs in boys. Characteristics include - adrenal atrophy and widespread cerebral demyelinization. Usual course - progressive.

CAUSES:

• unknown biochemical enzyme defect

TREATMENT

• hormone replacement therapy
• dietary therapy

MISCELLANEOUS

SYNONYMS:

• sudanophilic leukodystrophy with adrenal atrophy
• Addison-Schilder disease
• Siemerling-Creutzfeldt disease
• diffuse cerebral sclerosis with adrenocortical atrophy
• Addison disease with cerebral sclerosis
• Sex-linked metachromatic leukodystrophy

ICD-9-CM:

330.0 leukodystrophy

(see images)

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Total Medical Terms: 109

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