Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
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A Study of Ulegyria as Pathognomonic Aspects of Congenital Bilateral Perisylvian Syndrome

선천성 양측성 Sylvius 주위 피질 증후군의 병인론으로서의 뇌회반흔증에 대한 고찰

  • Kim, Han-Woong (Department of Neurosurgery, Gwangju Christian Hospital) ;
  • Cho, Kyu-Yong (Department of Neurosurgery, Gwangju Christian Hospital) ;
  • Lee, Min-Cheol (Epilepsy Center, CNU Research Institute of Medical Science, Chonnam National University Hospital) ;
  • Kim, Hyung-Ihl (Department of Neurosurgery, Gwangju Veterans Hospital) ;
  • Woo, Young-Jong (Epilepsy Center, CNU Research Institute of Medical Science, Chonnam National University Hospital) ;
  • Kim, Myeong-Kyu (Epilepsy Center, CNU Research Institute of Medical Science, Chonnam National University Hospital)
  • 김한웅 (광주 기독병원 신경외과) ;
  • 조규용 (광주 기독병원 신경외과) ;
  • 이민철 (전남대학교병원 간질센터 및 전남대학교 의과학 연구소) ;
  • 김형일 (광주 보훈병원 신경외과) ;
  • 우영종 (전남대학교병원 간질센터 및 전남대학교 의과학 연구소) ;
  • 김명규 (전남대학교병원 간질센터 및 전남대학교 의과학 연구소)
  • Published : 2005.02.28
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Abstract

Objective: Congenital bilateral perisylvian syndrome(CBPS) has been defined as a characteristic malformative perisylvian polymicrogyria(PMG) in patients with clinical symptoms of pseudobulbar palsy and epileptic seizures. For the present study, we investigate clinicopathologic features of CBPS associated with timing of lesion formation. Methods: Clinicopathologic features of CBPS from 6 patients with surgical resection of the cerebral lesions due to medically intractable seizures were studied. Results: Seizure onset ranged from 1 to 10years (average 6.7years) of age, and average duration of seizure was 23years. All had complex partial seizures, and two patients had additional tonic clonic seizures. Magnetic resonance (MR) images showed polymicrogyria, atropic gyri with gliosis. In the histopathologic examination, the cortical lesions revealed features of ulegyria; atrophic and sclerotic gyri, laminar loss of neurons, extensive lobular gliosis throughout the gray and white matter, neuronoglial nodule formation, and many amyloid bodies. Unlayered or four-layered PMG was not identified. Conclusion: Above data suggest that CBPS might be caused by ulegyria resulting from developmental cortical defect during early fetal stage or acquired hypoxic/ischemic injury in prenatal or postnatal life.

Keywords

References

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