Ohtahara syndrome is a very rare and devastating form of epileptic encephalopathy of very early infancy. Onset of seizures is mainly by 1 month of age.

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Dr Tina Williams PLEAT Frimley Park Hospital June 2011.

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Presentation transcript:

Ohtahara syndrome is a very rare and devastating form of epileptic encephalopathy of very early infancy. Onset of seizures is mainly by 1 month of age and often within the first 10 days of life, sometimes prenatally or during the first 2–3 months after birth. Characteristic clinico-EEG features are tonic spasms and, less commonly, erratic focal motor seizures and hemiconvulsion with S-B pattern in the EEG occurring in both sleep and waking states. Although the etiologies of Ohtahara syndrome are heterogeneous, prenatal brain pathology such as a malformation of cortical development is suspected in most cases and metabolic disorders are rare. Evolution into West syndrome is often observed in surviving cases.4,6 Source: Epileptic Encephalopathy, Atlas of Pediatric EEG Citation: Laoprasert P. Atlas of Pediatric EEG; 2011 Available at: https://neurology.mhmedical.com/DownloadImage.aspx?image=/data/books/1042/lao001_fig_07-02.gif&sec=59079706&BookID=1042&ChapterSecID=59078729&imagename= Accessed: November 08, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved