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 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.

China ; Humans ; Nervous System Diseases ; pathology

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 study the locations of P-glycoprotein(P-gp),multi-drug resistanceassociated protein (MRP) and lung resistance.related protein(LRP)in brain tissue of focal cortical dysplasia (FCD) and to compare the quantity of these proteins in different brain tissues in an effort to study the mechanism of refractory epilepsy and to provide theoretical basis for medical treatment in patients with epilepsy.Methods The brain tissues of 16 cases with refractory epilepsy were collected during surgery,including 8 cases of focal cortical dysplasia type Ⅰ(FCD Ⅰ)and 8 cases of focal cortical dysplasia type Ⅱ (FCD Ⅱ) Five cases of glioma without history of epilepsy were included as the control group.Envision method was used to observe the location and intensity of expression of these three proteins.Western-Blot was used to analyze these proteins quantitatively.Results In brain tissue,the locations of P-glycoprotein,multi-drug resistance-associated protein and lung resistance-related protein were difierent.P-gp located mainly in capillary endothelial cells;MRP in neurons components;the location of LRP included capillary endothelial cells,balloon cells and the matrix of lesions.The expressions of three proteins in brain tissue of FCD were significantly higher than in control group(P-gp:0.520 ±0.121,MRP:0.132±0.018,LRP:0.092.±0.018,U=0.000,P<0.01).The expressions of P-gp and LRP in lesions of FCD Ⅱ(3.809 ±0.842 and 0.655±0.303,respectively)were higher than that in surrounding regions(2.636 ±0.622 and 0.290±0.096,U value were 6.000 and 4.500,both P<0.01).Conclusions P-gp,MRP and LRP expressed at different position of focal cortical dysplasia,suggesting that their roles were distinctive.

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.

at model is an ideal MS model for clinical MRI study.

Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.

Objective To compare the expression and distribution of drug resistance proteins Pglycoprotein (P-gp) and lung resistance protein (LRP) in brain tissues of patients with refractory epilepsy and to investigate the relationship between expression of drug resistance proteins and concentration of antiepileptic drug carbamazepine (CBZ).Methods We included the brain tissues of 26 cases with refractory epilepsy who had in the experiment.They had following pathologic diagnosis: focal cortical dysplasia (FCD) Ⅰb (n =10),FCD Ⅱ a (n =4),FCD Ⅱb (n =2) and brain tumorsincluding ganglioglioma (n =6) and dysembryoplastic neuroepithelial tumor(n =4).Immunohistochemistry staining using EnVision system was used to reveal the expression location of P-gp and LRP,and Western blot in SDS-polyacrylamide gel was used to quantitatively analyze the expression of P-gp and LRP.Fluorescence polarization immunoassay was used to determine concentration of CBZ.Results Both P-gp and LRP performed(μg/ml) prominent overexpression in brain tissues of patients with refractory epilepsy,especially in the lesions of both FCD type Ⅱ (P-gp: 2.593 ±0.829 vs 1.711 ±0.292,t =-2.201,P=0.028;LRP:1.352 ±0.445 vs 1.179 ±0.593, t =-2.516,P =0.028, respectively)and tumor(P-gp:1.567 ±0.092 vs 0.775 ± 0.101, t =2.698, P =0.024; LRP: 1.091 ± 0.239 vs 0.825 ± 0.297, t =3.997, P =0.003respectively).The concentration of CBZ in lesions of brain tumors were lower than which in surrounding regions(0.848 ±0.726 vs 0.948 ±0.785, t =-3.056,P =0.014), while P-gp and LRP were higher than which in surrounding regions in 80％ cases.There was no relationship between expression of drug resistance proteins and concentration of CBZ.Conclusions In processing of drug resistance, P-gp and LRP play important roles.However, there is no correlation between expression of drug resistance proteins and concentration of CBZ, suggesting epilepsy drug resistance to be a complicated mechanism.