Clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia IIId in children / 中华神经医学杂志

Objective:To investigate the clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia (FCD) IIId in children.Methods:Forty-nine children with epilepsy accepted surgical treatment in our hospital from January 2008 to July 2017 were chosen in our study; these patients were pathologically diagnosed as having FCD IIId. These children were divided into 7 groups according to different brain injuries: cerebral hemorrhage ( n=13), central nervous system infectious disease ( n=19), cerebral infarction ( n=1), intrapartum hypoxia ( n=5), two kinds of early brain injury ( n=2), febrile seizure ( n=6), traumatic brain injury ( n=3). Two years after surgery, Engel grading was used to evaluate the surgical efficacy of the children. The clinical and pathological characteristics of these children were retrospectively analyzed. Results:The average age of brain injury was 0 d-3.8 years, the average age of epilepsy was 4-5.3 years, and the average age of surgery was 7-10.2 years. The incidence of multiple lobe brain injury in the cerebral hemorrhage group (100%, 13/13) was significantly higher than that in the central nervous system infectious diseases group (36.8%, 7/19,) and febrile seizure group (50%, 3/6, P<0.05). There was a significant difference in Engel grading I ratio between cerebral hemorrhage group (84.6%, 11/13) and central nervous system infectious disease group (42.1%, 8/19, P<0.05); Engel grading I and II was noted in 80.0% children (4/5) of the intrapartum hypoxia group. All children had pathological changes and pathological characteristics of FCD after early brain injury. Conclusion:FCD IIId children with cerebral hemorrhage or intrapartum hypoxia have good surgical effect, which may be related to the early occurrence of early brain damage, clear pathological changes, and clear boundary between the focus and the surrounding brain tissues.

Clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia IIId in children / 中华神经医学杂志

Objective:To investigate the clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia (FCD) IIId in children.Methods:Forty-nine children with epilepsy accepted surgical treatment in our hospital from January 2008 to July 2017 were chosen in our study; these patients were pathologically diagnosed as having FCD IIId. These children were divided into 7 groups according to different brain injuries: cerebral hemorrhage ( n=13), central nervous system infectious disease ( n=19), cerebral infarction ( n=1), intrapartum hypoxia ( n=5), two kinds of early brain injury ( n=2), febrile seizure ( n=6), traumatic brain injury ( n=3). Two years after surgery, Engel grading was used to evaluate the surgical efficacy of the children. The clinical and pathological characteristics of these children were retrospectively analyzed. Results:The average age of brain injury was 0 d-3.8 years, the average age of epilepsy was 4-5.3 years, and the average age of surgery was 7-10.2 years. The incidence of multiple lobe brain injury in the cerebral hemorrhage group (100%, 13/13) was significantly higher than that in the central nervous system infectious diseases group (36.8%, 7/19,) and febrile seizure group (50%, 3/6, P<0.05). There was a significant difference in Engel grading I ratio between cerebral hemorrhage group (84.6%, 11/13) and central nervous system infectious disease group (42.1%, 8/19, P<0.05); Engel grading I and II was noted in 80.0% children (4/5) of the intrapartum hypoxia group. All children had pathological changes and pathological characteristics of FCD after early brain injury. Conclusion:FCD IIId children with cerebral hemorrhage or intrapartum hypoxia have good surgical effect, which may be related to the early occurrence of early brain damage, clear pathological changes, and clear boundary between the focus and the surrounding brain tissues.

Long-Term Follow-Up in Children With Focal Cortical Dysplasia IIId for Early Brain Injuries, Including Neuropathological Findings.

BACKGROUND: Early cerebral injury has a close relationship with epilepsy and focal cortical dysplasia Ⅲd. We investigated children with focal cortical dysplasia Ⅲd who underwent surgery for epilepsy. METHODS: We selected 49 patients from among 260 pediatric patients who had undergone epilepsy surgery, analyzing their clinical materials and pathology data. The selected patients had been followed for more than two years. RESULTS: The 49 patients were divided into seven groups based on different early brain injuries. There was a significant difference (P < 0.05) between Engel class I ratio of cerebral hemorrhage group (84.6%) and that of central nervous system infection group (42.1%) in two to eight years follow-up. The patients with prior cerebral hemorrhage had a wider scope (P < 0.05) of brain damage than those in the brain infection and febrile convulsion groups. Secondary polymicrogyria commonly existed. Neuron islands were located adjacent to polymicrogyria in cerebral hemorrhage and brain trauma patients, and missing neuronal laminations beside the polymicrogyria was noted in others. CONCLUSIONS: In children with focal cortical dysplasia Ⅲd, individuals with cerebral hemorrhage within the perinatal period exhibited a wider range of brain lesions, while the postoperative follow-up outcome was better. Secondary polymicrogyria existed along with focal cortical dysplasia Ⅲd and is related to the developmental lesion. The processes of secondary polymicrogyria caused by different early brain injuries might be different.

Rasmussen syndrome: a clinicopathologic study of four cases / 中华病理学杂志

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.

Single-stage total corpus callosotomy combined with different resective operations in children with Lennox-Gastaut syndrome / 中华神经医学杂志

Objective To study the outcomes of Lennox-Gastaut syndrome (LGS) with single-stage total corpus callosotomy combined with different resective surgeries. Methods Nine LGS patients, admitted to our hospital from May 2010 to May 2014, were chosen in our study. Their clinical data were retrospectively analyzed. According to the results of anatomy, electrophysiology and clinical comprehensive evaluation, all the 9 children received single-stage total corpus callosotomy combined with different resective operations. The differences of epileptic seizures of these children before and after surgery were compared. Results The 9 LGS children were followed up for 2 years;5 achieved Engel grade I, 3 achieved Engel grade II, and one achieved Engel grade III. The surgical effective rate was 88.9％ (8/9). The frequencies of drop seizures, convulsive seizures, tonic seizures and tonic-clonic seizures were decreased of different degrees, with drop seizures enjoying the best control. Three patients had transient silence, remarkable relief one week after surgery and total recovery half year after surgery. Conclusion Early single-stage total corpus callosotomy combined with different resective operations can help to control seizures in children with intractable LGS.

Clinicopathologic analysis of cerebral hemisphere ulegyria in 26 patients with refractory epilepsy / 中华实用儿科临床杂志

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.

Application of sylvian veins morphology in functional surgery evaluation / 实用医学杂志

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.

The pediatric epilepsy surgery in China

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

Epilepsy surgery in China: The history and current development

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��.

The expression of Tau protein in the brains of the intractable epilepsy patients / 中华神经科杂志

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.

The expression of heat shock 27 000 associated protein 1 in the brains of drug-refractory epilepsy / 中华神经科杂志

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