“A RARE CASE OF EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY”

Author:

Mounica K. Joy1,Kumar N. Hemanth1,Sundarachary NV2

Affiliation:

1. Post Graduate in Neurology, GMC, Guntur

2. MD, DM, Prof & HOD Neurology, GMC, Guntur

Abstract

Early infantile epileptic encephalopathy (EIEE), also known as Ohtahara syndrome, is characterized by intractable tonic seizures in the setting of a severe encephalopathy and a burst-suppression background pattern on EEG. Incidence has been estimated at 1/100 000 births in Japan and 1/50,000 births in the U.K. Approximately 100 cases total have been reported but this may be an underestimate. Ohtahara syndrome is a rare clinico-EEG syndrome. Gold standard investigation is genetic panel for epilepsy in case of nil structural abnormalities. It is refractory to any mode of treatment including ketogenic diet and surgery and carries highest mortality rate, die in infancy.

Publisher

World Wide Journals

Subject

General Economics, Econometrics and Finance,General Social Sciences,Health, Toxicology and Mutagenesis,Public Health, Environmental and Occupational Health,Multidisciplinary,General Chemical Engineering,Law,Surfaces, Coatings and Films,General Energy,Mechanical Engineering,Industrial and Manufacturing Engineering,Strategy and Management,Computer Science Applications,Industrial relations,Management Information Systems,Marketing,General Engineering,Developmental and Educational Psychology,Education,Engineering (miscellaneous),Instrumentation

Reference3 articles.

1. Swaiman’s Pediatric Neurology Principles and Practice sixth edition chap 70 pg 554

2. Swaiman’s Pediatric Neurology Principles and Practice sixth edition chap 70 pg 555

3. Baxter PS, Gardner-Medvin D, Barwick DD, Ince P, Livingston J, Murdoch-Eaton D. Vigabatrin monotherapy in resistant neonatal seizures. Seizure 1995;4:57-9.

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