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ISSN: 2161-0665

Pediatrics & Therapeutics
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Case Report

Evaluation of Behavior and Cognitive Function in Children with Myoclonic Astatic Epilepsy

Taemi Niimi1, Yuji Inaba1*, Mitsuo Motobayashi1, Takafumi Nishimura1, Naoko Shiba1, Tetsuhiro Fukuyama2, Tsukasa Higuchi3, and Kenichi Koike1

1Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan

2Department of Pediatric Neurology, Nagano Children’s Hospital, Azumino, Japan

3Department of General Pediatrics, Nagano Children’s Hospital, Azumino, Japan

*Corresponding Author:
Yuji Inaba, MD
Department of Pediatrics
Shinshu University School of Medicine
3-1-1 Asahi, Matsumoto 390-8621, Japan
Tel: +81-263-37-2642
Fax: +81-263-37-3089
E-mail: [email protected]

Received Date: June 21, 2013; Accepted Date: July 21, 2013; Published Date: July 24, 2013

Citation: Niimi T, Inaba Y, Motobayashi M, Nishimura T, Shiba N, et al. (2013) Evaluation of Behavior and Cognitive Function in Children with Myoclonic Astatic Epilepsy. Pediat Therapeut 3: 159. doi:10.4172/2161-0665.1000159

Copyright: © 2013 Niimi T, et al. 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.

Abstract

Background: Myoclonic astatic epilepsy (MAE) is an idiopathic and generalized childhood epileptic syndrome. Although several studies have reported on cognitive function and intellectual outcome in MAE, little is known about the behavioral problems associated with this disease. The aim of this study was to clarify behavior and cognitive function in children with MAE.
Methods: Four children who were diagnosed as having MAE using the proposed criteria of the International Classification of Epilepsies were retrospectively analyzed using patient records with regard to clinical and neuropsychological findings such as age of seizure onset, semiology and severity of seizures, treatment course, behavioral problems, EEG findings, and WISC-III and Pervasive Developmental Disorders Autism Society Japan Rating Scale (PARS) scores that indicate the tendency of autistic behaviors.
Results: Disease onset in our cohort ranged from 9 to 35 months of age. Seizures were controlled within 3-8 months over a follow-up period of 4-12 years. All patients had borderline normal intelligence (mean IQ: 75.8 at 5-7 years of age) and exhibited impaired coordination, clumsiness, hyperactivity or impulsivity, and impaired social interaction after improvement of seizures. Though these autistic social problems manifested gradually, the children were able to adapt to school life with appropriate support. Interestingly, the behaviors of impaired social interaction and hyperactivity or impulsivity had already been observed before the onset of MAE in most patients, whose PARS scores were all higher than reference values (<9) during infancy.
Conclusion: Our results showed that children with MAE tend to have autistic behavioral problems and mild intellectual impairment along with wide ranges in WISC-III subtest scores. These behavioral and cognitive features appear to exist before the onset of epilepsy.

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