Alexander Disease / Disease

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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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: [email protected]
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