Alexander disease is a rare, genetically determined degenerative disorder of the central nervous system. It is one of a group of disorders known as the leukodystrophies, diseases that result from imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fibers in the brain. Myelin, which lends its color to the "white matter" of the brain, is a complex substance made up of at least ten different chemicals. Each of the leukodystrophies affects one (and only one) of these substances. In Alexander disease, the destruction of white matter in the brain is accompanied by the formation of fibrous protein deposits called Rosenthal fibers. It is caused by mutations in the gene for glial fibrillary acidic protein (GFAP). The majority of cases are sporadic (not inherited), but there are families in which more than one child will have the disorder. Alexander disease primarily affects males and usually begins at about 6 months of age. Symptoms may include mental and physical retardation, dementia, enlargement of the brain and head, spasticity (stiffness of arms and/or legs), and seizures. In addition to the infantile form, juvenile and adult onset forms of the disorder have been reported. These forms occur less frequently and have a longer course of progression.

GoldBamboo

... Alexander disease is a rare disorder of the nervous system that usually begins during infancy or early childhood. Onset can also occur, although less commonly, during the school-age years (juvenile form) or adulthood. The disorder is characterized by abnormal deposits of proteins, called Rosenthal fibers, in specialized brain cells (astrocytes). Alexander disease is considered one of the leukodystrophies, a group of disorders where the primary abnormality is the inability to maintain the fatty covering (myelin) that insulates nerve fibers in the brain. ...
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Alexander disease is a slowly progressing fatal neurodegenerative disease. ...
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Alexander Disease
... What is Alexander Disease? Alexander disease is a rare, genetically determined degenerative disorder of the central nervous system. It is one of a group of disorders known as the leukodystrophies, dis...
Source: Cleveland Clinic

Alexander Disease Information Page
... Alexander disease is a rare, genetically determined degenerative disorder of the central nervous system. It is one of a group of disorders known as the leukodystrophies, diseases that result from impe...
Source: National Institute of Neurological Disorders and Stroke

   

Alexander Disease Articles

  • Alexander disease
    ... Alexander disease is a rare disorder of the nervous system that usually begins during infancy or early childhood. Onset can also occur, although less commonly, during the school-age years (juvenile fo...
  • Alexander Disease Information Page
    ... Alexander disease is a rare, genetically determined degenerative disorder of the central nervous system. It is one of a group of disorders known as the leukodystrophies, diseases that result from impe...

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