Canadian Medical Guide > Diseases > Nervous System Diseases > Demyelinating Diseases > Hereditary Central Nervous System Demyelinating Diseases > Alexander Disease Terms and Definitions




Alexander Disease

Medical Definition: A rare inherited disorder of myelin formation. Alexander disease is a progressive leukencephalopathy whose hallmark is the widespread accumulation of cytoplasmic inclusions called Rosenthal fibers. The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with ALPHA-CRYSTALLIN B CHAIN. Rosenthal fibers are found predominantly in ASTROCYTES located in the subependymal, subpial, and periventricular areas of the BRAIN.

Alexander Disease Medical Definitions and Terms

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