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Copyright 1986, 1987, 1988, 1990, 1993, 1995, 1999, 2000, 2002, 2007, 2008
NORD is very grateful to Michael Brenner, PhD, University of Alabama at Birmingham, for assistance in the preparation of this report.
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).
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Organizations related to Alexander Disease
- Arc
1660 L Street, NW, Suite 301
Washington DC 20036
Phone #: 202-534-3700
800 #: 800-433-5255
e-mail: info@thearc.org
Home page: http://www.thearc.org
- Children's Brain Diseases Foundation
Parnassus Heights Medical Building
350 Parnassus Avenue, Suite 900
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
54521
Laxou Cedex None 61024
Phone #: 33 -383- 30 93 34
800 #: --
e-mail: ela@ela-asso.com
Home page: http://www.ela-asso.com
- Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
e-mail: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx
Home page: http://rarediseases.info.nih.gov/GARD
- Hunter's Hope Foundation, Inc.
PO Box 643
6368 West Quaker Street
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 Avenue, Suite 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 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 #: 914-997-4488
800 #: 888-663-4637
e-mail: Askus@marchofdimes.com
Home page: http://www.marchofdimes.com
- National Institute of Neurological Disorders and Stroke (NINDS)
P.O. Box 5801
Bethesda MD 20824
Phone #: 301-496-5751
800 #: 800-352-9424
e-mail: me20t@nih.gov
Home page: http://www.ninds.nih.gov/
- United Leukodystrophy Foundation
2304 Highland Drive
Sycamore IL 60178
Phone #: N/A
800 #: 800-728-5483
e-mail: office@ulf.org
Home page: http://www.ulf.org/
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