Abstract

Ohtahara syndrome (OS) is an epileptic syndrome with onset in neonatal period and has many clinicoelectrical characteristics, of which age dependency and evolutional change is specific. The most specific EEG feature is the suppression burst (SB). This pattern is characterized by high voltage bursts alternating with flat suppression phases at a regular rate. A term neonate presented with seizures on day three of life. Seizures were intractable. Metabolic workup for seizures was normal. Extended panel neurometabolic screen was normal and neuroimaging did not reveal any structural abnormality. EEG showed burst suppression pattern suggestive of ohtahara syndrome. Virtually all reported cases of early infantile epileptic encephalopathy (EIEE) are secondary to a congenital or acquired structural malformation of cortical development. The etiology of this syndrome remains obscure. All children with EIEE should be thoroughly investigated with MRI, CSF amino acid level determination, and detailed postmortem neuropathologic examination.

Keywords: Ohtahara Syndrome; Seizures; Epileptic Encephalopathy; EEG; Suppression Burst

References

1. Ohtahara Shunsuke and Yasuko Yamatogi. “Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy.” Epilepsy Research70 (2006): S58-67.
2. Stamberger Hannah., et al. “STXBP1 encephalopathy: A neurodevelopmental disorder including epilepsy”. Neurology10 (2016): 954-962.
3. Clarke M., et al. “Early infantile epileptic encephalopathy with suppression burst: Ohtahara syndrome”. Developmental Medicine and child Neurology4 (1987): 520-528.
4. Miller S P., et al. “Infantile epileptic encephalopathy (Ohtahara syndrome) and migrational disorder”. Pediatric Neurology1 (1998): 50-54.
5. Yamatogi Yasuko and Shunsuke Ohtahara. “Early-infantile epileptic encephalopathy with suppression-bursts, Ohtahara syndrome; its overview referring to our 16 cases”. Brain and Development1 (2002): 13-23.

Citation

Citation: Srijan Singh. “A Case of Early-infantile Epileptic Encephalopathy with Suppression-bursts- the Ohtahara Syndrome”. Acta Scientific Paediatrics 4.2 (2021): 06-10.

Copyright

Copyright: © 2021 Srijan Singh. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.