Adrenoleukodystrophy. Electron microscopic findings.
Ultrastructural and neurochemical studies were done on three male patients with adrenoleukodystrophy. In each case, the affected white matter contained enlarged glial cells filled with pathognomic intracytoplasmic inclusions consisting of electron-lucent spicules bounded by 25-Angstrom wide membranes. Similar inclusions were present in adrenocortical cells. These findings and a review of 47 reported cases indicate that adrenoleukodystrophy is a storage disorder caused by a sex-linked recessive error of metabolism.
D000302:Adrenal Cortex / Q000473:pathology; D000304:Adrenal Cortex Function Tests; D000307:Adrenal Gland Diseases / Q000150:complications / Q000473:pathology*; D000328:Adult; D001344:Autopsy; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000150:complications / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002855:Chromatography, Thin Layer; D003593:Cytoplasm / Q000648:ultrastructure; D003711:Demyelinating Diseases / Q000209:etiology / Q000473:pathology; D005625:Frontal Lobe / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D008214:Lymphocytes / Q000473:pathology; D008264:Macrophages / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D009929:Organ Size