[Electron microscopic and neurochemical study of Alexander's disease (author's transl)].
The authors report the results of a cerebral and of a neuromuscular biopsies and of the autopsy findings in another infantile case of Alexander's disease in a girl. They review the 17 previously reported cases of this disease and the various etiopathogenic hypotheses mentioned. The presence of numerous, sometimes abnormal enlarged mitochondria and of abundant membranous cytoplasmic bodies in the astrocytic cytoplasm seems to be unreported elsewhere. Peripheral nerve changes are mentioned for the first time.
D001253:Astrocytes / Q000648:ultrastructure; D001706:Biopsy; D001921:Brain / Q000648:ultrastructure; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000473:pathology*; D005260:Female; D006130:Growth Disorders / Q000473:pathology; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000473:pathology; D008854:Microscopy, Electron; D008928:Mitochondria / Q000648:ultrastructure; D009132:Muscles / Q000648:ultrastructure; D010525:Peripheral Nerves / Q000648:ultrastructure; D013577:Syndrome
Etude en microscopie electronique et neurochimique d'un cas de maladie d'Alexander.