Seizures and Epilepsy
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Ohtahara Syndrome
Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.This is a very rare epilepsy syndrome. Seizures start before three months of age. Most babies have an underlying structural brain abnormality which may be genetic in origin (passed on through the genes) or may be due to 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 the baby having seizures after birth.
Seizure types are variable but often include tonic spasms and partial motor seizures Generalised seizures may happen later. After several months, seizures may change to infantile spasms (West syndrome)
Diagnosis
The electroencephalogram (EEG) shows evidence of abnormal discharges or spikes and waves alternating with periods of flatness. This type of record is referred to as suppression-burst.
All infants are neurologically very abnormal. Often they are very floppy and excessively sleepy. With time they may develop stiffness (spasticity) in the limbs.
Treatment
The seizures are often resistant to medication. The choice of medication is difficult but often some of the older medications such as phenobarbitone are tried first.
Some babies with a focal structural brain abnormality (clear abnormality) may be able to have epilepsy surgery to remove the abnormal area of brain.
Prognosis (outlook)
Infants with Ohtahara Syndrome make very little developmental progress. They remain totally dependent on others and often feed poorly. They often die within the first two years of life because of repeated chest infections. Those who do survive will be severely disabled.
Support organisation
Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, http://www.cafamily.org.uk/
If you would like any more information about epilepsy, then please contact Epilepsy Action via the Email Helpline or, if you live in the UK, by phoning the Freephone Helpline on 0808 800 5050.
- Myoclonic astatic epilepsy (Doose syndrome)
- syndromes
- Panayiotopoulos syndrome
- Early Infantile Epileptic Encephalopathy
- Ohtahara Syndrome