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What is Adrenomyeloneuropathy, a variant of adrenoleukodystrophy which manifests as adrenal insufficiency beginning in childhood later develop to a progressive spastic paraparesis is transmitted as: and explain in detail?
This X-linked disorder is a variant of adrenoleukodystrophy. Affected males usually have a history of adrenal insufficiency beginning in childhood and then develop a progressive spastic (or ataxic) paraparesis beginning in early adultZhood. Female heterozygotes may develop a slower, insidiously progressive spastic myelopathy beginning later in adulthood and without adrenal insufficiency. Diagnosis is usually made by demonstration of elevated levels of very long chain fatty acids in plasma and in cultured fibroblasts. Steroid replacement is indicated if hypoadrenalism is present, and bone marrow transplantation and nutritional supplements have been attempted for this condition without clear evidence of efficacy. Ref: Harrisons Principles of Medicine, 18th Edition, Page 3375