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Objective@#To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease.@*Methods@#Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016.@*Results@#The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients.MRI demonstrated unihemispheric focal cortical atrophy in all cases. The histopathologic changes included variable degrees of lymphocytic infiltrate within the cortex, subarachnoid space and perivascular cuffing.Microglial nodules and neuronophagia were seen. Mild to severe neuronal loss was noted with variable degrees of reactive gliosis. Spongy edema and cavitation were observed in focal cortex. Inflammation involving hippocampus was seen in one case. Three cases were accompanied by focal cortical dysplasia (FCD) Ⅲd. Immunohistochemical staining showed that the infiltrative lymphocytes were positive for CD3, CD8, granzyme B and TIA1 and the proliferating microglial cells were positive for CD68. NeuN positive neurons decreased significantly and reactive astrocytes were GFAP positive.@*Conclusions@#Pathologic changes of RS are similar to viral encephalitis and the inflammation is progressive and multifocal involving the hemisphere. The diagnosis of RS relies on pathologic features combined with clinical findings and neuroradiological examinations.
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Objective To investigate the clinicopathologic features of cerebral hemisphere ulegyria in children with refractory epilepsy.Methods The clinical and pathologic findings were reviewed in 26 children who underwent resection of lesion and epileptogenic lobectomy operation in the Neurosurgery Department of Haidian Hospital,Haidian District of Peking University Third Hospital,from January 2011 to August 2015,and pathological diagnosis was cerebral hemisphere ulegyria.Results All children including 19 male and 7 female had medically intractable seizures.The mean ages of seizure onset and disease duration were 3.93 years old (from 10 days to 12 years old) and 5.42 years (from 1 month to 13 years),respectively.Eight cases had seizures because of perinatal period injury,and 12 cases developed seizures owing to infancy injury and others had no cause of disease.The mean operation age was 9.35 years old (5-14 years old).Fourteen cases underwent multilobar resection and the whole corpus callosum incision,and 12 cases were given modified anatomical cerebral hemisphere resection.The pathological diagnoses of brain tissues were cerebral hemisphere ulegyria with focal cortical dysplasia(FCD) Ⅲ d and dual pathology.Seizure outcome after the operation revealed that 19 cases (73.08％) had an Engel grade Ⅰ,3 cases (11.54％)had an Engel grade Ⅱ,2 cases (7.69％) had an Engel grade Ⅲ,and 2 cases (7.69％) missed the follow-up.Conclusions Acquired brain injury during the period of infant can cause ulegyria and cortical dysplasia,resulting in intractable epilepsy.The treatment of improved anatomical cerebral hemisphere resection and multilobar resection is significantly effective.
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Objective Sylvian Veins morphology analysis contributes to cortex veins protection for clear surgical fields, a better choice for operation plan evaluation. Methods 33 cases diagnosed as epilepsy, male∶female=19∶14, mean ages 18.7y (9-52). Results 4 types of Sylvian Veins spreading were observed from the procession of functional surgery: long stem pattern 49%(16/33), short stem pattern 15%(5/33), long truncks pattern 33%(11/33), and absent stem pattern 3%(1/33). Conclusion Sylvian Veins morphology analysis shows that there exist 4 main patterns, which may contribute to functional surgery about frontal operculum, temporal operculum and insular lobe or intracranial electrode placement.
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The earliest activities related to epilepsy surgery in China ma�� � y be tra�ce��d to the 19���50s an�� �� d 60s��. A�ter a lull during the cultural revolution, there was resurgence of epilepsy surgery from the 1980s, and rapid development from 2000. It is estimated that app�roximately 150- ���200 p hysi�cians �currentl�� � y pra�cti�ce epilepsy surgery in the country.�� In year 2��� 005, there were a total of 2,��500 �cases of e�pile�ps��y o�perate��d in whole of �China, close to half in Beijing, Tian�jin, �Shanghai, �Chongqing an��d Guang�zhou, an�� a �uarter in Beijing. There are three Chinese epilepsy surgery journals, 5 monographs on epilepsy surgery, and “��Epilepsy surgery” edite�� ��� ��� �� ��� ��� d by HO Luders has also �been translate��d into �Chinese��.
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There is rapid growth of pediatric surgery service in China in the recent years. A survey by the China Association �A gainst �Epi�lepsy underta�ken in �June ����� 2006���� on the ma�jor cities in different parts of the country showed a dramatic increase particularly in years 2004 and 2006. Surgery for temporal lobe epilepsy accounts for half of the operation. Surgery has an important role in the treatment of drug resistant pediatric epilepsy. With large population and vast geographical spread, it is important to further develop pediatric epilepsy surgery service in China
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Objective To study the expression of heat shock 27 000 associated protein 1 ( HSPBAP1, GenBank: AK096705) in the brain tissues of patients with drug-refractory epilepsy and discuss its function in the pathogenesis. Methods Fluorescent quantitative polymerase chain reaction ( FQ-PCR) and immunohistochemistry were used to test the expression of HSPBAP1 in the surgically removed brain tissues of patients with drug-refractory epilepsy from the brain bank of our department ( n = 36) , and the results were compared with that of normal controls (n = 8 ). Results The relative expression of HSPBAP1 mRNA in the brains of patients with drug-refractory epilepsy was more than 34. 11 times that of controls, and HSPBAP1 protein expression was significantly increased in temporal lobe cortex (0. 0507?0. 0003, P
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Objective To explore the expression of Tau protein in brains of the intractable epilepsy patients and discuss its function in the pathogenesis. Methods Immunohistochemistry was adapted to test the expression of Tau protein and the mossy fiber sprouting in the hippocampus and cortex of temporal lobe from 48 intractable epilepsy patients and age-mached 48 cases of controls. Results The expression of phosphorylated Tau protein in the CA_3 areas (0.0450?0.0115) and the molecular layers of dentate gyrus (0.0463?0.0120) in intractable epilepsy patients was increased, accompanied by the hippocampus mossy fiber sprouting (3.18?0.35). No difference on the expression of total Tau protein was observed between the intractable epilepsy patients and the controls. Conclusion The present findings demonstrate that the strengthening of phosphorylated Tau protein might play an important role for the synaptic reorganization of the hippocampus. Thus, the changes of phosphorylated Tau protein could improve clinical prognosis in the intractable epilepsy patients.