Alexander Disease and Related Conditions
Alexander (1949) described previously unrecorded morphologic changes in the brain of a 16-month-old boy who had suffered from progressive mental deterioration and enlargement of the head since the age of 7 months. Postmortem examination disclosed a leukodystrophy and an abundance of rodshaped eosinophilic deposits which are now generally recognized as Rosenthal fibers. The deposits were found throughout the tissue, with particular predilection for the pial and vascular interfaces of the nervous parenchyma. Alexander considered the disease a “progressive fibrinoid degeneration of fibrillary astrocytes”.
KeywordsWhite Matter Axon Swelling Alexander Disease Rosenthal Fiber Axon Dystrophy
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- Rewcastle, B.: Presentation to the Canadian Association of Neuropathologists, 1966. Cited in Herndon et al., 1970.Google Scholar
- Schlote, W.: Rosenthalsche „Fasern“ und Spongioblasten im Zentralnervensystem. II. Elektronenmikroskopische Untersuchungen. Behr. path. Anat. 133: 461–480, 1966.Google Scholar
- Sherwin, R. M., Berthrong, M.: Alexander’s disease with sudanophilic leukodystrophy. Arch. Path. (Chic.) 89: 321–328, 1970.Google Scholar