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.