Adrenomyeloneuropathy, a variant of adrenoleukodystrophy which manifests as adrenal insufficiency beginning in childhood later develop to a progressive spastic paraparesis is transmitted as:
Correct Answer: X-linked disorder
Description: 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
Category:
Medicine
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now