title: [Pathologic features of ulegyria in refractory epilepsy by modified anatomic hemispherectomy: a clinicopathologic study of 39 cases].
abstract: OBJECTIVE
  To investigate the clinicopathologic features of the brain tissue diagnosed as ulegyria from modified anatomic hemispherectomy for refractory epilepsy.
  
  
  METHODS
  The clinical and pathologic findings were reviewed in 39 patients who underwent modified anatomic hemispherectomy and diagnosed as ulegyria in the Epilepsy Center of Tsinghua University Yuquan Hospital from 2007 to 2011.
  
  
  RESULTS
  All patients including 30 males and 9 females had medically intractable seizures, and the mean age of seizure onset and disease duration were 4.0 years and 7.3 years respectively. Significant history included febrile seizure in 14 patients (35.9%), cerebral hemorrhage in 8 patients (20.5%), fetal distress and surgical trauma each in 6 patients (15.4%), vascular malformation and cerebral hemorrhage in 1 patient (2.6%), and unclear history in 4 patients (10.2%). Histologically, all cases were characterized by cortical destruction, with neuronal loss and gliosis. All cases were accompanied by varying degree of cortical dysplasia, which were diagnosed as focal cortical dysplasia IIId. Hippocampus sclerosis was identified in 2 cases. Seizure outcome after surgery revealed 37 patients (94.9%) had an Engel grade I, two patients (5.1%) had an Engel grade II.
  
  
  CONCLUSIONS
  Febrile seizure, cerebral hemorrhage, fetal distress and surgical trauma in childhood can lead to refractory epilepsy. Histopathological change in the brain is ulegyria accompanied by focal cortical dysplasia IIId. Modified anatomic hemispherectomy is an effective therapy to treat those patients with extensive changes of one hemisphere.
authors.affiliation: Department of Pathology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China.
authors.forename: Dan-dan
authors.initials: DD
authors.lastname: Wang
authors.forename: Qiu-ping
authors.initials: QP
authors.lastname: Gui
authors.forename: Jiu-luan
authors.initials: JL
authors.lastname: Lin
authors.forename: Zhong
authors.initials: Z
authors.lastname: Zheng
authors.forename: Shu-jie
authors.initials: SJ
authors.lastname: Yang
authors.forename: Jing-xia
authors.initials: JX
authors.lastname: Hu
authors.forename: Shi-lun
authors.initials: SL
authors.lastname: Wang
authors.forename: Yan-fang
authors.initials: YF
authors.lastname: Shi
authors.forename: Yang
authors.initials: Y
authors.lastname: Luo
authors.forename: Guang-ming
authors.initials: GM
authors.lastname: Zhang
authors.forename: Wen-jing
authors.initials: WJ
authors.lastname: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D017252:CD3 Complex / Q000378:metabolism; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D038421:Hemispherectomy / Q000379:methods*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D054220:Malformations of Cortical Development / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D017252:CD3 Complex