Adrenoneuropathy: characteristic gross findings and association with Hashimoto's thyroiditis and accelerated...

New understanding in the pathogenesis of adrenoneuropathy (ANP), formerly called adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AML), has led to greater awareness of the disease with resultant increase in the number of new cases being diagnosed. We report adrenoneuropathy in two siblings, one of whom had associated affective symptoms and died undiagnosed in prison. The other sibling died at the age of eight with severe cerebral demyelination. Affective symptoms have been described in ANP, but the association of Hashimoto's thyroiditis and accelerated atherosclerosis in this case is unique. We would like to stress the characteristic gross appearance of the adrenals in ANP that may help in identifying undiagnosed cases of adrenoneuropathy in surgical and autopsy specimens. Genetic counseling and family-pedigree analysis with diet restriction and diet therapy will be invaluable in such cases.