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Ohtahara Syndrome

Brain

What is a syndrome?

A syndrome is a group of signs and symptoms that, added together, suggest a particular medical condition. In epilepsy, examples of these signs and symptoms would be things like the age at which seizures begin, the type of seizures, whether the child is male or female and whether they have physical or learning disabilities, or both. The results of an electroencephalogram (EEG) are also used to help identify epilepsy syndromes.

Epilepsy Action has more information about seizure types, learning disabilities, and the EEG.

If you would like to know more about an epilepsy syndrome, please speak to your doctor. If you would like to know more about epilepsy in general, please contact Epilepsy Action.

Other names for Ohtahara syndrome

  • Early infantile epileptic encephalopathy with suppression bursts

Ohtahara syndrome

This is a very rare epilepsy syndrome. Seizures start before three months of age. Most babies have an underlying structural brain abnormality. This may be genetic in origin (passed on through the genes), or happen because of brain damage before or around the time of birth.


Symptoms

Seizures often start in the first 10 days of life. Sometimes the mother may realise her baby was having seizures during the last three months of pregnancy, when she sees her baby having seizures after birth. Seizure types are variable, but often include tonic spasms, where the baby suddenly has stiffening of the limbs lasting a few seconds. They will also have focal (partial) motor seizures with jerking of one limb, or one side of the body, lasting seconds to several minutes. Generalised seizures may happen later. After several months, seizures may change to infantile spasms (West syndrome)

Epilepsy Action has more information on seizures and West syndrome.


Diagnosis

A full account of what happens during the seizures is essential for the doctors making the diagnosis.

The EEG test, which records the electrical activity in the brain, is usually abnormal and shows evidence of abnormal discharges, or spikes and waves alternating with periods of looking flat and featureless. This type of record is referred to as suppression-burst. 

Epilepsy Action has more information on diagnosis and the EEG.

Treatment

The seizures are often resistant to epilepsy medicines. The choice of medicine is difficult, but often some of the older medicines, such as phenobarbital, are tried first. Other medicines that may be effective include clobazam (Frisium), clonazepam (Rivotril) or vigabatrin (Sabril). Some babies with a focal (in one area only) structural brain abnormality seen on an MRI scan may be able to have epilepsy surgery to remove the abnormal area of brain.

Prognosis (outlook)

Affected babies do not behave normally. They are very floppy and excessively sleepy (described as an encephalopathy) and often have difficulty with feeding. With time they may develop stiffness (spasticity) in the limbs. They also make very little developmental progress and remain totally dependent. They often feed poorly. Sadly, they often die within the first two years of life, because of complications, including repeated chest infections. Those who do survive are usually severely disabled and will continue to have seizures despite treatment.


Support organisation

Contact a Family
209-211 City Road
London EC1V 1JN
Phone: 0808 808 3555
Website: www.cafamily.org.uk

Because Epilepsy Action has not written this information, it is not produced under the terms of the Information Standard. Details of the Information Standard are available from www.theinformationstandard.org  

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Code: 
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Our thanks

Epilepsy Action would like to thank

  •  Dr Richard Appleton, and Dr Rachel Kneen, consultant paediatric neurologists at Alder Hey Children’s Hospital, Liverpool, UK and
  •  Dr Stewart Macleod, consultant paediatric neurologist at Royal Hospital for Sick Children, Glasgow, UK.
    They have kindly prepared the information on this page. It is based on their own research, experience and expertise.

This information is exempt under the terms of The Information Standard.

  • Updated November 2012
    To be reviewed November 2015

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