[Syndrome of Ohtahara].
BACKGROUND
A literature review using the term Ohtahara syndrome and early infantile epileptic encephalopathy revealed 51 cases.


METHODS
The characteristics of these cases were: 1) early onset of seizures; 2) tonic seizures; 3) poor response to treatment; 4) mental retardation; 5) poor prognosis; 6) burst-suppression EEG pattern; 7) evolution to West syndrome, and 8) multiple causes. These characteristics are non specific. No cause was found in most cases. The onset of seizures was between 7 hours and 86 days of age. The most frequent brain imaging abnormality was diffuse atrophy. ACTH was effective in a few cases and hemispherectomy was successful in one case.


CONCLUSIONS
We believe that Ohtahara syndrome and early myoclonic encephalopathy are the same entity.
Yelin
K
K
Departamento de Neurología, Miami Children's Hospital, FL 33155-4079, USA.
Alfonso
I
I
Papazian
O
O
D004830:Epilepsy, Tonic-Clonic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D011379:Prognosis; D011596:Psychomotor Disorders / Q000150:complications / Q000175:diagnosis*; D013036:Spasms, Infantile / Q000175:diagnosis; D013577:Syndrome