Full 2L QBank
Medicine General 7d24faba

Adrenomyeloneuropathy, a variant of adrenoleukodystrophy which manifests as adrenal insufficiency beginning in childhood later develop to a progressive spastic paraparesis is transmitted as:

A
X-linked disorder
B
Autosomal dominant
C
Autosomal recessive
D
Sporadic
High-Yield Explanation
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

Related Medicine MCQs

Practice 2,00,000+ NEET PG Questions Free

Timed mock tests, mistake queue analytics, audio lectures & zero attempt limits on i❤️Exams.

Start Free Mock Test Now