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n. a genetically determined condition of neurological degeneration with childhood and adult forms. Inherited as an X-linked (see sex-linked) trait resulting in demyelination, it is characterized by progressive spastic paralysis of the legs, sensory loss, and cognitive impairment, associated with adrenal gland insufficiency and small gonads. The demonstration of a genetic defect in the metabolism of very long chain fatty acids aids diagnosis and has implications for future possible drug therapies. Prenatal diagnosis is possible.

Subjects: Medicine and Health.

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