November 1972

Schilder's DiseaseSex-Linked Recessive Transmission With Specific Adrenal Changes

Author Affiliations

Bronx, NY
From the C. S. Kubik Laboratory of Neuropathology, Massachusetts General Hospital, Boston (Drs. Schaumburg, Richardson, and Johnson); Department of Pathology, Beth Israel Hospital, Boston (Dr. Cohen); and the Department of Pathology (Neuropathology), Albert Einstein College of Medicine, Bronx, NY (Drs. Powers and Raine).

Arch Neurol. 1972;27(5):458-460. doi:10.1001/archneur.1972.00490170090014

The adrenal glands from ten patients with Schilder's disease, of whom four were familial and nine were male, were examined. Many cells of the zona reticularis and fasciculata were ballooned and often contained crystalline aggregates. The X-linked recessive inheritance and this derangement of the adrenal cortex in males with Schilder's disease strongly indicate the presence of a metabolic process.