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What is Alexander Disease?
DESCRIPTION: Alexander disease is a rare, genetic, degenerative
disorder of the nervous system. It is one of a group of genetic disorders called the leukodystrophies
that affect growth of the myelin sheath, the fatty covering--which acts as an
insulator--on nerve fibers in the brain. Alexander disease, which affects mostly males,
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.
Is there any treatment?
TREATMENT: There is no cure for Alexander disease, nor is there
a standard course of treatment. Treatment of Alexander disease is symptomatic and
What is the prognosis?
PROGNOSIS: The prognosis for individuals with Alexander disease
is generally poor. Most children with the infantile form do not survive past the age of 6.
In the juvenile form death usually occurs within 10 years after the onset of symptoms.
What Research is Being Done?
RESEARCH: The NINDS supports research on genetic disorders
including leukodystrophies such as Alexander disease. The goals of this research are to
find ways to prevent, treat, and, ultimately, cure these disorders.
Where can I find more information?
These articles, available from a medical library, are
sources of in-depth information on Alexander disease:
Arend, A, Leary, P, and Rutherford, G. "Alexander Disease: A Case Report with
Brain Biopsy, Ultrasound, CT Scan, and MRI Findings." Clinical Neuropathology,
10:3; 122-126 (1991).
Bobele, G, et al. "Neuroimaging Findings in Alexander Disease." Journal of
Child Neurology, 5:253-258 (July 1990).
Magalini, S, et al (eds). Dictionary of Medical Syndromes, 3rd edition, J.B.
Lippincott Co., Philadelphia, pp. 33-34 (1990).
Rowland, L (ed). Merritt's Textbook of Neurology, 9th edition, Williams &
Wilkins, Baltimore, pp. 521t, 600-601 (1995).
Schuster, V, Horwitz, A, and Kreth, H. "Alexander Disease: Cranial MRI and
Ultrasound Findings." Pediatric Radiology, 21:133-134 (1991).
Shah, M, and Ross, J. "Infantile Alexander Disease: MR Appearance of a
Biopsy-Proved Case." American Journal of Neuroradiology, 11:1105-1106
Additional information is available from the following organizations:
Nat. Org. for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
United Leukodystrophy Foundation
2304 Highland Drive
Sycamore, IL 60178
Information provided by NIH.