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NINDS Ohtahara Syndrome Information Page

Synonym(s):   Early Infantile Epileptic Encephalopathy

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What is Ohtahara Syndrome?

Ohtahara syndrome is a neurological disorder characterized by seizures.  The disorder affects newborns, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures.  Infants have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures.  Ohtahara syndrome is most commonly caused by metabolic disorders or structural damage in the brain, although the cause or causes for many cases can’t be determined.  Most infants with the disorder show significant underdevelopment of part or all of the cerebral hemispheres.  The EEGs of infants with Ohtahara syndrome reveal a characteristic pattern of high voltage spike wave discharge followed by little activity.  This pattern is known as “burst suppression.” Doctors have observed that boys are more often affected than girls.

Is there any treatment?

Antiepileptic drugs are used to control seizures, but are unfortunately not usually very effective for this disorder.  Corticosteroids are occasionally helpful.  In cases where there is a focal brain lesion (damage contained to one area of the brain) surgery may be beneficial.  Other therapies are symptomatic and supportive. 

What is the prognosis?

The course of Ohtahara syndrome is severely progressive.  Seizures become more frequent, accompanied by physical and mental retardation.   Some children will die in infancy; others will survive but be profoundly handicapped.  As they grow, some children will progress into other epileptic disorders such as West syndrome and Lennox-Gestaut syndrome. 

What research is being done?

The NINDS conducts and supports an extensive research program on seizures and seizure-related disorders.  Much of this research is aimed at increasing scientific understanding of these disorders and finding ways to prevent, treat, and potentially cure them.

NIH Patient Recruitment for Ohtahara Syndrome Clinical Trials

Organizations

Column1 Column2
Epilepsy Foundation
8301 Professional Place
Landover, MD   20785-7223
postmaster@efa.org
http://www.epilepsyfoundation.org
Tel: 301-459-3700 800-EFA-1000 (332-1000)
Fax: 301-577-2684

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291



Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated December 5, 2008