Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Alexander Disease


Synonyms of Alexander Disease
  • Dysmyelogenic Leukodystrophy
  • Dysmyelogenic Leukodystrophy-Megalobare
  • Fibrinoid Degeneration of Astrocytes
  • Fibrinoid Leukodystrophy
  • Hyaline Panneuropathy
  • Leukodystrophy with Rosenthal Fibers
  • Megalencephaly with Hyaline Inclusion
  • Megalencephaly with Hyaline Panneuropathy

Disorder Subdivisions



    General Discussion
    Alexander disease is named after the physician who first described the condition in 1949 (WS Alexander). It is an extremely rare, progressive, neurological disorder that most often presents during infancy or early childhood, but can also occur as late as the sixth decade of life. Alexander disease has historically been included among the leukodystrophies--diseases of the white matter of the brain. These diseases affect the fatty material (myelin) that forms an insulating wrapping (sheath) around certain nerve fibers (axons). Myelin enables the efficient transmission of nerve impulses and provides the whitish appearance of the so-called white matter of the brain. There is a marked deficit in myelin formation in most infantile cases of Alexander disease, and sometimes in juvenile cases, particularly in the front (frontal lobes) of the brain's two hemispheres (cerebrum). However, white matter defects are sometimes not observed in later onset cases. Instead, the unifying feature among all Alexander disease cases is the presence of abnormal protein aggregates known as Rosenthal fibers throughout certain regions of the brain and spinal cord (central nervous system [CNS]). These aggregates occur in astrocytes, a particular cell type in the CNS that helps maintain a normal CNS environment. Accordingly, it is more appropriate to consider Alexander disease a disease of astrocytes (an astrogliopathy) than a white matter disease (leukodystrophy).

    Organizations related to Alexander Disease
    • Children's Brain Diseases Foundation
      350 Parnassus Avenue
      San Francisco CA 94117
      Phone #: 415-665-3003
      800 #: --
      e-mail: Jrider6022@aol.com
      Home page: N/A
    • ELA - European Association against Leukodystrophies
      2, rue Mi-les-Vignes
      Laxou None 54520
      Phone #: 33 -383- 30 93 34
      800 #: --
      e-mail: ela@ela-asso.com
      Home page: http://www.ela-asso.com
    • Hunter's Hope Foundation, Inc.
      PO Box 643
      Orchard Park NY 14127
      Phone #: 716-667-1200
      800 #: 877-984-4673
      e-mail: info@huntershope.org
      Home page: http://www.huntershope.org
    • Let Them Hear Foundation
      1900 University Ave #101
      East Palo Alto CA 94303
      Phone #: 650-462-3143
      800 #: 877-735-2929
      e-mail: info@letthemhear.org
      Home page: http://www.letthemhear.org
    • MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
      150 Custer Court
      Green Bay WI 54301-1243
      Phone #: 920-336-5333
      800 #: 877-336-5333
      e-mail: mums@netnet.net
      Home page: http://www.netnet.net/mums/
    • Madisons Foundation
      PO Box 241956
      Los Angeles CA 90024
      Phone #: 310-264-0826
      800 #: N/A
      e-mail: getinfo@madisonsfoundation.org
      Home page: http://www.madisonsfoundation.org
    • March of Dimes Birth Defects Foundation
      1275 Mamaroneck Avenue
      White Plains NY 10605
      Phone #: 914-428-7100
      800 #: 888-663-4637
      e-mail: Askus@marchofdimes.com
      Home page: http://www.marchofdimes.com
    • National Institute of Neurological Disorders and Stroke (NINDS)
      31 Center Drive
      Bethesda MD 20892-2540
      Phone #: 301-496-5751
      800 #: 800-352-9424
      e-mail: braininfo@ninds.nih.gov
      Home page: http://www.ninds.nih.gov/
    • The Arc (a national organization on mental retardation)
      1010 Wayne Ave
      Silver Spring MD 20910
      Phone #: 301-565-3842
      800 #: 800-433-5255
      e-mail: info@thearc.org
      Home page: http://www.thearc.org/
    • United Leukodystrophy Foundation
      2304 Highland Drive
      Sycamore IL 60178
      Phone #: 815-895-3211
      800 #: 800-728-5483
      e-mail: office@ulf.org
      Home page: http://www.ulf.org/



    For a Complete Report

    This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html