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Articles » NINDS Aicardi Syndrome Information Page: NINDS

NINDS Aicardi Syndrome Information Page: NINDS

Article title: NINDS Aicardi Syndrome Information Page: NINDS

Conditions: Aicardi syndrome

What is Aicardi Syndrome?
Aicardi syndrome is a rare disorder characterized by the partial or complete agenesis (absence) of the corpus callosum (the structure that links the 2 hemispheres of the brain), infantile spasms (a form of childhood seizure), mental retardation, and an ocular (eye) abnormality called lacunae (lesions) of the retina of the eye. Aicardi syndrome may be associated with other brain defects such as microcephaly (small brain) or porencephalic cysts (cerebrospinal fluid-filled cavities or gaps in the brain). Onset of Aicardi syndrome generally begins between the ages of 3 and 5 months. The disorder affects only females.

Is there any treatment?
There is no cure for Aicardi syndrome nor is there a standard course of treatment. Treatment, which is symptomatic, generally involves management of seizures and intervention programs for mental retardation

What is the prognosis?
The prognosis for individuals with Aicardi syndrome varies according to the presence and severity of symptoms.

What research is being done?
The NINDS supports and conducts research on neurogenetic disorders such as Aicardi syndrome. The goals of this research are to find the genes involved in these disorders and to learn more about them. Finding the genes could lead to an effective means to treat and prevent disorders such as Aicardi syndrome.

Selected references

Bradley, W, et al (eds).
Neurology in Clinical Practice: The Neurological Disorders. vol. II, 2nd edition, Butterworth-Heinemann, Boston, pp. 1476-1477 (1996).

Magalini, S, et al (eds).
Dictionary of Medical Syndromes. 4th edition, J.B. Lippincott Co., Philadelphia, p. 19 (1997).

Rowland, L (ed).
Merritt's Textbook of Neurology. 9th edition, Williams & Wilkins, Baltimore, pp. 517-519 (1995). [see excerpt on agenesis of the corpus callosum].

Thoene, J (ed).
Physicians' Guide to Rare Diseases. Dowden Publishing Co., Inc., Montvale, NJ, pp. 288-289 (1992).


Aicardi Syndrome Foundation
450 Winterwood Drive
Roselle, IL 60172
Tel: 800-374-8518

Aicardi Syndrome Newsletter, Inc.
1510 Polo Fields Court
Louisville, KY 40245
Tel: 502-244-9152
Fax: Same

1010 Wayne Avenue
Suite 650
Silver Spring, MD 20910
Tel: 301-565-3842
Fax: 301-565-3843 or -5342

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203

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

National Eye Institute (NEI)
National Institutes of Health
Bldg. 31, Rm. 6A32
Bethesda, MD 20892-2510
Tel: 301-496-5248 Professionals 800-869-2020

National Institute of Child Health and Human Development (NICHD)
National Institutes of Health
Bldg. 31, Rm. 2A32
Bethesda, MD 20892-2425
Tel: 301-496-5133 800-370-2943

National Institute of Mental Health (NIMH)
6001 Executive Blvd.
Rm. 8184, MSC 9663
Bethesda, MD 20892-9663
Tel: 301-443-4513 TTY: 301-443-8431 Depression Info: 800-421-4211 Anxiety Info: 88-88-ANXIETY (269-4389) Panic Info: 888-64-PANIC (64-72642)
Fax: 301-443-4279

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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