Objective To recognize the developments and achievements of the clinical neuropathology in China from 1955 to 2009.Methods Retrospective survey of literatures related clinical neuropathology published in Chinese Journal of Neurology (CJN) from the first issue in 1955 to the No.12 issue in 2009 was conducted; Total literatures of CJN and literatures of the clinical neuropathology were respectively counted and the percentage of the latter was analyzed in different periods; Proportion of autopsy and biopsy literatures was also analyzed.According to categories of nervous system diseases,their relative percentages were respectively counted.The percentages of clinical neuropathological literatures and autopsy literatures of CJN were compared with the data of the same period of J Neurol Neurosurg Psychiatry (JNNP).Results (1) The percentages of clinical neuropathological literatures in total literatures of CJN were 18.11％ (65/359),20.81％ (103/495),17.05％ (22/129),15.50％ (230/1484),11.58％ (176/1520) and 16.04％ (309/1927),respectively,in 1955-1959,1960-1969,1970-1979,1980-1989,1990-1999,2000-2009.The percentage variation trend in different periods from 1955 to 2009 had significant difference (trend x2 =7.977,P =0.005) ; But there was no significant difference in the variation trend of the percentage of clinical neuropathological literatures between CJN and JNNP from 1955 to 2009.(2) The percentages of autopsy in the total clinical neuropathological literatures of CJN from 1995 to 2009 appeared gradually to decline; Compared with JNNP,the percentages of autopsy in the total clinical neuropathological literatures of CJN during 1980-2009 were lower,and there was significant difference in the variation trend of the percentage of autopsy in the total clinical neuropathological literatures between CJN and JNNP.(3) The main types of neurological diseases in the neuropathological literatures were tumors,infectious diseases and cerebrovascular diseases from 1955 to 1989,while myopathies became the most main disease type during 1990-2009; Compared with the same period JNNP,the clinical neuropathological literatures of the rare and difficult diseases and neurodegenerative diseases in CJN were relatively less.Conclusions Neuropathology is important in the clinical neuroscience research.From 1955 to 2009,main achievements of clinical neuropathological researches in China mainland lie in tumors,cerebrovascular diseases,infectious diseases and myopathies.But in recent years,due to the lack of autopsy cases,it is to the disadvantage of exploring pathogenesis of neurodegenerative diseases and understanding some rare and difficult diseases.

Objective To investipate the cerebral autosomal dominat arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) through the clinical features , pathology, neuroimage, and molecular genetics as well as the family tree of a case as to exploring the diagnostic method of the disease .Methods Systematic study on the clinical manifestations, genetic site, neurologic and muscular biopsy was performed. Results The main clinical features including poor memory and history of stroke were found. Neuroimaging examination showed multiinfarct lesions and leukoencephalopathy. Biopsy indicated the arteriolar hyalinosis,GOM and osmiophilic granule. A family history was confirmed. A mutation on the fourth exon of notch 3 gene was revealed. And no risk factors of hypertension and arteriosclerosis were found. All these features are in conformity with those of CADASIL.Conclusion CADAIL is not rare and is possible to be defined in vivo by way of dermal biopsy,examination of gene and neuroimage characteristics.

Objective To investigate the effect of angiotenisn ⅡI (Ang Ⅱ) on RIN-m β-cell,and to explore the mechanism of β-cell function impairment caused by Ang Ⅱ.Methods RIN-m cells were cultured with various concentrations of AngⅡ (0.1,1,10,100 nmol/L).After incubation for 24 hours,the basal(3.3 mmol/L) and glucose-stimulated(16.7 mmol/L) insulin secretion(GSIS)were detected by radioimmunoassay,mRNA and protein expressions of uncoupling protein 2(UCP2)were determined by RT-PCR and Western blot,respectively.The intracellular ATP content was measured by luciferase bioluminescence.The mitochondrial membrane potential and cellular Ca~(2+) concentration were detected by flow cytometry.Results (1) Various concentrations of Ang Ⅱ had no significant influence on the basal insulin secrection of RIN-m cell(F=0.644,P = 0.634).Except for 0.1 nmol/L AngⅡ,the other concentrations of Ang Ⅱ markedly reduced GSIS of RIN-m cells(F= 118.528,P = 0.000).(2) Compared with the control group,Ang Ⅱ significantly increased mRNA and protein expression of UCP2(F= 1 370,P = 0.000;F=675.175,P = 0.000).(3)Except for 0.1 nmol/L Ang Ⅱ,the other concentrations of Ang Ⅱ significantly decreased the mitochondrial membrane potential,cellular ATP content,and cellular Ca2+ concentration of RIN-m cell(F=4.035,P=0.008;F=3.353,P = 0.013;F=5.867,P = 0.001).Conclusion Ang Ⅱ impairs GSIS of p-cell,the mechanism of impairment may be interpreted that Ang Ⅱ can increase the expression of UCP2,furthermore,it can reduce mitochondrial membrane potential,decrease the content of cellular ATP and the concentration of cellular Ca~(2+),can finally impair the function of β-cell.

Objective To study the histopathological characteristics and the correlations between the cortical tissues from ictal discharge (ID) area and interictal epileptiform discharge (IED) area in epilepsy patients due to focal cortical dysplasia (FCD), in order to further discuss the mechanism of epileptogenicity. Methods Twenty-two subjects who underwent epilepsy surgeries consecutively in our institute since April 2005 to August 2006. All patients underwent intracranial electrode implantations and long-term video-EEG monitoring before the resective surgeries and the postoperative pathologies proved to be FCD. According the long-term EEG monitoring results, the cortex with intense IED and the cortex with ID onset were resected separatively in the operation for further histopathologic studies. Twenty cases were collected. Based on the Palimini' s pathologic subtype classification for FCD as well as quantificational scoring for immunocytochemistry for the calcium-binding protein parvalbumin (PV) which we designed by ourself, the specimens of IED and ID were studied and compared. Results The resected specimens from 20 cases were examed. ID specimens showed more severe abnormalities in the laminar cortical architecture, alterations in the morphology of neurons and in the appearance of abnormal balloon cells. With the PV quantificational scoring, we found significant difference between IED (6.4±2.1) and ID (4.4±1.8) from FCD Ⅱ specimens (P=0.042). No difference was found between ID subtypes (F=2.734, P=0.093 ). Conclusions ID cortical area showed more severe abnormalities in histopathologic changes than lED area. Our results suggested that the ID area of FCD had more severe damage in inhibitory synaptic circuits and neural networks, which meant it was more epileptogenic than IED. No difference was identified between each ID subtype in term of epileptogenicity, which meant all of them should be resected during the surgery.

ObjectiveTo investigate thc clinical and radiological characteristics of cortical vein thrombosis for early diagnosis and treatment.Methods Retrospective analysis was carried out with the clinical cases of cortical vein thrombosis in 2010.The symptoms,sign,neuroimaging were analyzed and related literatures were reviewed.ResultsFour patients were collected,average age was forty years old.The main symptoms were headache and focal neurological signs in varying degrees,intarction or hemorrhage in one or two sides of parietal lobe could be found in CT or MRI.Hemorrhage was found in two patients,infarction was found in one patient,hemorthage and infarction were both found in another patient.ConclusionsHeadachc and focal neurological signs are the common sings and symptoms of patients with cortical vein thrombosis.CT and MRI are effective methods for the diagnosis of cortical vein thrombosis.

Objective To explore the clinicopathological features and imaging characteristics of non-Langerhans cell histiocytosis in central nerve system,thus to facilitate the diagnosis and differential diagnosis.Methods A total of ten cases were enrolled in the study,with seven cases of Rosai-Dorfman disease(RDD) and three cases of xanthoma disseminatum (XD).Data on the clinicopathological features,imaging,immunophenotype and prognosis were collected and analyzed.Results Seven patients with RDD,5 males and 2 females with the mean age of 46.7 years old,all presented as dural-based or intraparenchymal hypo-to isointense lesions on T1 and T2 with post-contrast enhancement.The polymorphous admixture of histiocytes,lymphocytes and plasma cells was observed in a fibrous stroma,with emperipolesis of some histiocytes.The immunohistostaining of CD11c,CD68,MAC387 and S-100 was positive in the histiocytes,while the staining of CD1α was negative.Five patients recovered after the operation,while one patient died of the disease.All the 3 XD patients were female,with the median age of 20.7 years old.All XD patients presented as multiple intraparenchymal hypointense lesions on T1 and hyperintense lesions on T2 with post-contrast enhancement.The infiltration of foam-like histiocytes,a few Touton giant cells,lymphocytes and eosnophils was observed in all XD patients.The immunohistostaining of CD68 and CD11c was positive in the histiocytes and that of MAC387 partly positive,while the staining of S-100 and CD1α was negative.One XD patient survived well,while another one died of the disease.Conclusions The diagnosis of RDD and XD should be based on their typical morphology and immunophenotype and should be differentiated from Langerhans cell histiocytosis and other non-Langerhans cell histiocytosis.Non-Langerhans cell histiocytosis in central nerve system often presents untypical clinical presentation and imaging features,thus the communication and cooperation between clinician and pathologist is needed.

Objective To investigate the effect of angiotensin Ⅱ(AngⅡ) and losartan on ? cells,and its related molecular mechanisms.Methods Normally cultured RIN-m cells were divided into three groups:control group(cultured with RPMI 1640 medium),AngⅡ group(treated with 100 nmol/L AngⅡ),losartan group(treated with 1 ?mol/L losartan 15min before addition of 100nmol/L AngⅡ).After incubation for another 48h,the apoptosis rate of RIN-m cells was quantified by flow cytometry(FCM) with Annexin-V FITC/PI dual staining.The expressions of Bcl-2 and Bax mRNA and protein were determined by RT-PCR and Western blotting,and the activities of Caspase 3 and Caspase 9 were detected by spectrophotometry.Results The apoptosis rate of RIN-m cells was significantly higher in AngⅡgroup than in both control and losartan group(P0.05).In comparison to the control group,the expressions of Bcl-2 mRNA and protein significantly declined,while of Bax mRNA and protein increased obviously in AngⅡ group(P0.05),but the expressions of Bcl-2 mRNA and protein were significantly higher(P0.05).Conclusion AngⅡ may induce the apoptosis of ? cells through mitochondrial pathway,and pre-intervention with losartan,which partly reverses the effect of AngⅡ,may play a protective effect on ? cells.

Objective:To observe the effects of Rhizoma coptidis decoction on the myoelectric activity of womb of virginal rats and to discuss its mechanism. Methods: The changes of uterine myoelectric activity of rats were recorded by Biolap 98 biological system with a pair of mini bipolar Ag AgCl electrodes implanted on the uterine serosal surface. Rhizoma coptidis decoction was injected to find their effects on uterine myoelectric activity of rats, then M, H 1,?,L calaium channel and prostaglandin synthetase were blocked or inhibited respectively to be observed their influence Rhizoma coptidis decoction. Results:Rhizoma coptidis decoction can significantly increase uterine myoelectric activity of rats. After M and H 1 receptors were blocked, the effects of Rhizoma coptidis decoction were partially decreased. The effects of Rhizoma coptidis decoction were disappeared with L calcium channels blocked. But, nothing happened if ? receptor was blocked or prostaglandin synthetase was inhibited. Conclusions: Rhizoma coptidis decoction can significantly increase uterine myoelectric activity of rats, which may be associated with M receptor, H receptor and L calcium channels, nothing with ? receptor and synthesis or releasing of prostaglandin.

Objective To investigate the clinicopathologic features of the brain tissue from multilobar resection or hemispherectomy for refractory epilepsy. Methods The clinical and pathologic findings of 46 cases seen at Xuanwu hospital from 2005 to 2009 were reviewed retrospectively. Results The mean age of seizure onset and disease duration were 3.9 years and 10.2 years, respectively. There were 33 cases of hemispherectomy and 13 cases of multilobar resection. Temporal lobe abnormality was seen in all cases. The pathologic subgroups were as follows: ulegyria (31/46), malformation of cortical development (MCD, 7/46 ) and infection (8/46). Microscopic examination of ulegyria showed cortical architectural disturbances, neuronal loss, reactive gliosis and appearance of corpora amylacea. We also noted deposition of hemosiderin (13 cases), calcification (9 cases) and island-like neurons (5 cases). All ulegyria cases were accompanied by varying degree of cortical dysplasia, and hippocampus sclerosis were identified in 7 cases. MCD comprised of 5 cases of focal cortical dysplasia ( FCD), including 3 cases of FCDⅠB, 1 case of FCDⅡA and 1 case of FCDⅠA, 1 case of polymicrogyria and 1 case of porencephaly. Among 8 infection eases, there were 5 cases of Rasmussen encephalitis ( RE), l case of cysticercosis, 1 case of tuberculous meningitis and l case of Cytomegalovirus encephalitis. Conclusions The most common pathological category of specimens from hemispherectomy or multilobar resection is ulegyria with obvious temporal lobe abnormality. This is followed by MCD ( with FCDⅠB as the main type) and central nervous system infection (RE as the most frequent abnormality).

Objective To investigate the elinieopathologie features of refractory epilepsy. Methods The clinical and pathologic features of refractory epilepsy of 273 cases undergoing surgical treatment in Xuanwu Hospital from January 2005 to December 2007 were reviewed retrospectively. Results The mean age of seizure onset and disease duration were 11.0 years and 11.2 years respectively. The following pathologic subgroups were identified: malformation of cortical development (MCD, 158/273) including focal cortical dysplasia (FCD) type Ⅰ (104, the mean age of seizure onset and disease duration were 11.1 years and 11.2 years respectively), FCD type Ⅱ (30, the mean age of seizure onset and disease duration were 7. 9 years and 12. 7 years respectively) , mild MCD (6) , tuberous sclerosis complex (6) and other types (9) , brain turnouts (26/273, the mean age of seizure onset and disease duration were 14. 5 years and 6. 3 years respectively), ulegyria (63/273), brain infections (13/273), vascular malformation (3/273), cyst (3/273), cholesteatoma (1/273) and other unknown types (6/273). Dual pathology was identified in 31 of 158 MCD cases. Eighty-six of 134 (64. 2%) FCD cases were type Ⅰ B predominantly seen in temporal lobe. Twenty of 26 (76. 9%) brain tumours were mixed neuronal-glial tumours predominantly located in temporal lobe. Cortical dysplasia was often seen in these cases. Conclusions The 3 most common causes of refractory epilepsy are MCD, ulegyria and brain tumours. The predominant subtype of FCD is type Ⅰ B often located in temporal lobe, in which associated hippoeampal sclerosis is often seen. Brain tumors in patients with refractory epilepsy are almost benign neoplasms located in temporal lobe, in which the most frequent type is mixed neuronal-glial tumour.