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NINDS Alexander Disease Information Page: NINDS

Article title: NINDS Alexander Disease Information Page: NINDS

Conditions: Alexander Disease

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What is Alexander Disease?
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, is usually nonfamilial and 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?
There is no cure for Alexander disease, nor is there a standard course of treatment. Treatment of Alexander disease is symptomatic and supportive.

What is the 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?
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.

Selected references

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. 4th edition, J.B. Lippincott Co., Philadelphia, p. 27 (1997).

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).

Springer S, et. al.
Alexander disease--classification revisited and isolation of a neonatal form. Neuropediatrics, 31:2:86-92 (April 2000).

 Organizations

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

United Leukodystrophy Foundation
2304 Highland Drive
Sycamore, IL 60178
ulf@tbcnet.com
http://www.ulf.org/
Tel: 815-895-3211 800-728-5483
Fax: 815-895-2432

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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