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KMID : 1031220130170010017
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Journal of Korean Epilepsy Society
2013 Volume.17 No. 1 p.17 ~ p.21
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Wolf-Hirschhorn (4p-) Syndrome Presenting with Status Epilepticus
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Jun Ji-Young
Yu Hee-Joon
Lim Byung-Chan
Lee Jee-Hun
Chae Jong-Hee
Lee Mun-Hyang
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Abstract
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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.
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KEYWORD
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Wolf-Hirschhorn syndrome, Status epilepticus, Chromosome 4p deletion
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