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Journal of K orean Epilepsy Society 2013 June;17(1):17-21.
Published online 2013 October 14
Copyright ⓒ 2010 Journal of Korean Epilepsy Society
뇌전증지속상태로 발현한 Wolf-Hirschhorn (4p-) 증후군 2예
전지영1⋅유희준1⋅임병찬2⋅이지훈1⋅채종희2⋅이문향1
1성균관대학교 의과대학 삼성서울병원 소아과학교실, 2서울대학교 의과대학 소아과학교실
Corresponding Author: Jeehun Lee, MD, PhD ,Tel: +82-2-3410-0910, Fax: +82-2-3410-0043, Email: jhlee0101@skku.edu
ABSTRACT
Wolf-Hirschhorn syndrome is a well-recognized malformation syndrome with multiple congenital anomalies, resulting from partial deletion of the short arm of chromosome 4 (4p-). All affected individuals have intrauterine and postnatal growth retardation with marked feeding difficulties, developmental delay, and intellectual disability. Additionally, most of patients have seizures from early infancy. Although seizures are common with this syndrome, presenting with status epilepticus (SE) is rare. We report two cases of Wolf-Hirschhorn syndrome presenting with SE.
Keywords: Wolf-Hirschhorn syndrome, Status epilepticus, Chromosome 4p deletion

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