Objective To evaluate the applicability and effectiveness of the model for managing patients with convulsive epilepsy in rural China.Methods The model is under leadership of the government departments and medical institutions at all levels organized primary physicians for treatment and management.People with convulsive epilepsy in 24 rural areas were screened by local physicians and confirmed by neurologists.Patients diagnosed with convulsive epilepsy and agreed with managing were recruited into study.They were managed at primary care level and provided with phenobarbital monotherapy.Efficacy was assessed from the changes in seizure frequencies from baseline.Results There were 408 town hospitals and 979 trained primary care physicians participating in our study.Five thousand and thirty-eight patients were enrolled in 2 years,and 90％ patients with good compliance.Among the people who were treated with phenobarbital monotherapy,3 170 were managed for 12 months,and 2 005 (63.3％) of whom had a 50％ or greater reduction in seizure frequency and 1 246 (39.3％)patients remained seizure free;651 patients were managed for 24 months,and 491 (75.4％) of whom had a 50％ or greater reduction in seizure frequency and 202(31.0％)patients remained seizure free.Three hundred and forty-three (6.8％) patients withdrew from the study,and only 35 (0.7％) patients discontinued treatment because of side effects.Sixty-nine (1.4％) patients were dead and 8 (11.6％) of them were caused by status epilepticus.Conclusion This study confirms that this model is beneficial to patients with epilepsy and is suitable to the rural areas of China.

Objective To study the changes of cognitive function and brain microstructure of patients with benign epilepsy of childhood with centrotemporal spikes (BECTS) from neuropsychological test and voxel-based morphometry.Methods Sixteen patients with BECTS and 16 healthy volunteers,who were matched in gender,age and education,were enrolled in this study from May 2014 to May 2015 in the Affiliated Hospital of North Sichuan Medical College.The cognitive function and morphometry of the subjects were evaluated by neuropsychological test (full scale intelligence quotient (FSIQ),verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ)) and high-resolution T1-weighted imaging scanning.Thereafter,these data were analyzed with Student's t test,Chi-squared test and correlation analysis.Results (1) FSIQ(79.3±13.3),VIQ(82.3±12.1) andPIQ(81.3±10.3)ofBECTS group were significantly lower than the control group (107.0 ± 6.4,101.6 ± 6.5,114.8 ± 6.2;t =9.511,7.521,16.360,all P ＜0.01).(2) Patients with BECTS showed significantly increased gray matter volume in the bilateral putamen,paracentral lobule and right supplementary motor area (P ＜ 0.05,FDR-corrected).(3) A significant negative correlation was found between the gray matter volume of the left putamen and age of epilepsy onset (r=-0.586,P=0.017).Conclusions (1) There is a certain degree of cognitive impairment in patients with BECTS.(2) Patients with BECTS have some changes in brain microstructure.Genetic factors could indirectly influence the children's brain developmental trajectory,and the age of onset may be associated with the changes on the brain microstructure.

Objective To investigate the changes in cortical excitability and inhibitory circuits of patients with Alzheimer's disease (AD) or behavioral variant frontotemporal dementia (bvFTD) using transcranial magnetic stimulation (TMS).Methods Forty-four patients with AD,30 patients with bvFTD and 44 healthy controls were enrolled in the study.The epidemiological data of all subjects were collected.Each participant received a neurological evaluation and neuropsychologic tests which included Mini-Mental State Examination (MMSE),Activities of Daily Living (ADL) and Hamilton Rating Scale for Depression (HAMD).Neurophysiological evaluations including resting motor threshold (rMT),active motor threshold (aMT) and cortical silent period (CSP) were conducted by means of TMS.Neurophysiological parameters were compared among groups.Results There were significant differences in MMSE,ADL and HAMD assessments among 3 groups,but no significant differences between AD and bvFTD groups.There were significant changes in left rMT(46.52％ ± 8.77％,52.00％ ± 7.30％ and 52.14％ ± 8.27％,F =6.295,P=0.003),left aMT(29.68％ ±7.01％,35.13％ ±8.46％ and 35.39％ ±7.24％,F=7.735,P=0.001) and right rMT(47.82％ ±7.94％,52.07％ ±8.77％ and 53.12％ ±8.61％,F=4.772,P=0.010) among 3 groups.AD patients showed significantly reduced left rMT and aMT comparing to bvFTD patients (P =0.017 and 0.008 respectively) and controls (P =0.005 and 0.002 respectively).In addition,AD patients had a significant decrease in right rMT comparing to controls (P =0.011).There were no statistically significant differences in TMS parameters between bvFTD patients and controls.Conclusions AD is associated with hyperexcitability of the motor cortex,whereas the lack of changes in motor cortical excitability is found in bvFTD.TMS technique may be helpful in differential diagnosis between AD and bvFTD.

Objective To investigate the apathy and neuropsychological characteristics of newly diagnosed Parkinson's disease (PD) patients.Methods Eighty-two newly diagnosed PD patients and 30 matched healthy controls by age,sex and education level were recruited in the present study.Apathy was assessed using Apathy Evaluation Scale (AES) and related factors,including age,sex,education level and disease duration were simultaneously evaluated.Unified Parkinson's Disease Rating Scale (UPDRS),Hamilton Depression Rating Scale (HAMD),as well as Montreal Cognitive Assessment (MoCA) were employed in order to respectively evaluate the motor function,depression and cognition.Results The AES scores in the PD patients were significantly higher when compared to the healthy controls.The prevalence of apathy and depression in the PD patients was 51.2％ (42/82) and 19.5％ (16/82),respectively.There were no statistically significant differences in age,sex,education level,UPDRS-Ⅱ/Ⅲ scores and MoCA scores between apathy (n =42) and no-apathy (n =40) PD patients (P ＞ 0.05),while the statistically significant difference in HAMD scores between apathy (n =42) and no-apathy (n =40) PD patients was shown (the HAMD scores of apathy PD patients were 10.61 ± 3.30,the HAMD scores of no-apathy PD patients were 5.96 ± 1.90,t =7.87,P ＜ 0.05).The correlation analysis indicated that there were no correlations between AES scores and the related factors,including age,education level,disease duration and UPDRS-Ⅱ / Ⅲ scores,but AES scores were positively correlated with HAMD scores.Conclusion In newly diagnosed PD patients,the apathy is independent of depression,motor dysfunction,as well as cognitive impairment,which may be an early signal for PD.

Objective To evaluate the usefulness of the short-segment nerve conduction studies (SSCSs,inching test) of the peroneal nerve across the fibular head in order to diagnose and localize the site of entrapment of the nerve.Methods Fifty-four patients with suspected peroneal nerve palsy and 30 controls were studied.The symptoms all occurred in unilateral leg,involving 25 left legs and 29 right legs.Both long segment motor nerve conduction studies (LSMCs) and SSCSs were performed in all patients and controls.SSCSs were obtained at 2 cm intervals,starting 6 cm proximal (P6,P4 and P2) and 4 cm distal (D2 and D4) ending to the fibular head prominence (P).Results When nerve conduction of the entire 10 cm segment across the fibular head was tested by the conventional method,only 40 showed reduction in amplitude or slowing of motor conduction velocity or both.However,with SSCSs,54 peroneal nerves were all discovered abnormal.The results of comparison of conventional methods suggested that there were significant differences in conduction velocity between the fibular head-Ankle segment and the knee-fibular head segment in the case group,the latter ((33.63 ± 9.29) m/s) being slower than the former ((47.92 ± 4.04) m/s;t =9.776,P =0.000),while there was no obvious abnormality in the control group.The results of the control group detected by SSCSs showed that there was no significant difference between the left and right sides of the mean amplitude of the stimulation points and the mean time of segmental nerve conduction.Therefore,we set the exception criteria as the segmental nerve conduction time is longer than the corresponding control group (i + 2 s),and the CMAP amplitude of proximal stimulation point decreased by more than 20％ than the adjacent distal segment.In accordance with this standard,we found that the lesions were located in P6 to P4 in 2/54 (3.7％) legs,P4 to P2 in 4/54 (7.4％) legs,P2 to P in 43/54 (79.6％) legs,P to D2 in 12/54 (22.2％) legs,D2 to D4 in 3/54 (5.6％) legs,respectively.Consequently,the P2 to P segment was most vulnerable to damage.Conclusions SSCSs are more sensitive in detecting the entrapment of the peroneal nerve across the fibular head than the LSMCs.SSCSs could precisely localize the entrapment lesions,might be a useful tool especially for the detection of mild entrapment which has normal LSMCs findings of the peroneal nerve across the fibular head.

Objective To further understand the clinical manifestations and improve clinical diagnosis of patients with leptomeningeal metastasizing high-grade glioma.Methods Sixteen patients with leptomeningeal metastasizing high-grade glioma (WHO classification:grade Ⅲ-Ⅳ) in Department of Radiotherapy,the First Hospital of Jilin University from July 2010 to September 2015 were respectively analyzed.The pathological types included anaplastic gliomas (1),anaplastic oligodenastrocytoma (1),glioblastoma (12),small-cell glioblastoma (1),gliosarcoma (1).We reviewed the relative clinical manifestations of the patients,and further compared them with 163 patients with systemic malignant solid tumors at corresponding period.Results The median time from initial diagnosis to the diagnosis of leptomeningeal metastasis was 13.0 months (range 2-19 months).Plain and enhanced magnetic resonance imaging was obtained in all patients.The main radiographic characteristics included ependymal enhancement (11),leptomeningeal enhancement (3),nodules of implantation metastasis in spinal canal (1),cranial nerve enhancement (2),and ventricular dilatation (1).Eight patients received cerebrospinal fluid examination.The diagnosis of leptomeningeal metastasis in 15/16 patients was determined by radiographic findings.Comparing with leptomeningeal metastasis from systemic malignant tumors at the corresponding period,the incidence of headache in patients with high-grade glioma was significantly lower (6/16 vs 81.6％ (133/163);x2 =16.3,P ＜ 0.01);and the incidence of cranial nerve paralysis was also significantly lower (4/16 vs 56.4％ (92/163);x2 =5.79,P =0.016 1).The incidence of nerve root symptoms was lower than that of systemic malignant tumors,though without statistically significant difference (2/16 vs 26.4％ (43/163);x2 =1.49,P=0.222).Nine patients respectively received chemotherapy,intrathecal chemotherapy or intrathecal chemotherapy combined with whole brain radiotherapy.The median survival tine was 4.5 months (range 0.7-13.3 months).Conclusions The imaging examination played an important role in the diagnosis of high-grade leptomeningeal metastasizing glioma.Comparing with the systemic malignant solid tumors,the leptomeningeal metastasizing high-grade glioma had its unique clinical characteristics.

Objective To establish drug resistant models of temporal lobe epilepsy induced by amygdala kindling,and to investigate the changes of cAMP response element binding protein (CREB) and phosphorylated cAMP response element binding protein (p-CREB) expression in the hippocampus tissues in order to explore their roles in drug resistant epileptogenesis.Methods Eighty adult male SD rats were randomly divided into control group (n =10) and model group (n =70).The 70 rats were used to prepare the amygdaloid kindled model of epilepsy by chronic stimulation of amygaloid basal lateral nucleus.The successful kindled models were randomly selected as drug resistant epileptic group (n =10) and drug sensitive epileptic group (n =10) according to their response to the phenytoin and phenobarbital.On the basis of behavioral observation,electrophysiology,pathological HE staining,CREB and p-CREB expression changes,we verified the reliability of the models and explored the differences among the three groups above.The changes of CREB and p-CREB expression were detected by immunohistochemical method and Western blotting assay.Results In control group,the electroencephalogram (EEG) frequency was (8.700 ±1.494) Hz;in drug sensitive epileptic group,the EEG frequency was (14.700 ± 1.159) Hz;in drug resistant epileptic group,the EEG frequency was (19.800 ± 1.686) Hz.The frequency differences among the three groups were statistically significant (F =144.202,P =0.000).By immunohistochemical staining,a large number of CREB and p-CREB positive cells were observed in drug resistant epileptic group.As compared with the control group (CREB 0.197 ±0.058,p-CREB 0.260 ±0.176),the expression levels of CREB and p-CREB were increased in drug sensitive epileptic group (CREB 0.361 ±0.151,p-CREB 0.656 ±0.234) and in drug resistant epileptic group (CREB 0.591 ± 0.150,p-CREB 1.077 ± 0.400).The difference among the three groups had statistical significance (F =24.206,20.376,both P ＜ 0.01).Conclusions The expressions of CREB and p-CREB were significantly increased in drug resistant epileptic rats.These findings indicate that the expressions of CREB and p-CREB may play certain roles in the drugresistant epileptogenesis.

Objective To study the relationship between gene mutation and clinical phenotype in patients with tuberous sclerosis complex (TSC).Methods The clinical data of 76 patients with TSC diagnosed in Guangdong 999 Brain Hospital were collected between May 2007 and May 2014 and then TSC gene mutation analysis was performed.Genotype-phenotype analyses for all the patients were also carried out.Results Fifty of the 76 (66％) patients were male,and 26 (34％) were female,in which 19 (31％) patients presented with cyst-like cortical tuber,69 (92％) with skin lesions,16 (30％) with renal lesions,50 (69％) with mental retardation and 39 still suffered seizures after a year.In this study,22 (29％) cases showed TSC1 gene mutation,31 (59％) presented TSC2 gene mutation,and 15 (20％)cases had no mutation identified.The mutation ratio of TSC1 ∶ TSC2 was approximately 3 ∶ 5,while the mutation ratio of TSC1 ∶ TSC2 was 1 ∶ 1 for familial TSC patients,and 1 ∶ 2 for sporadic TSC patients.Comparing to those with TSC1 gene mutation and no mutation identified,patients with TSC2 gene mutation exhibited statistical meaning on the aspects of the onset age of seizure (Z =1.688,P =0.007),seizure onset before l-year-old (x2 =10.584,P =0.001),epilepsy duration (x2 =4.996,P =0.025),spasms onset (x2 =10.111,P =0.001),cyst-like cortical tuber (x2 =9.182,P =0.002),skin lesions (x2 =9.016,P =0.003),as well as renal lesions (x2 =6.079,P =0.014).No apparent relation was found between genotype and intelligence outcome.Conclusions The patients with TSC2 gene mutations presented severer symptoms in seizure onset than those with TSC1 gene mutation and no mutation identified.The patients with TSC2 gene mutation were characterized by early onset of seizure,especially before 1-year-old,others like spasms onset,cyst-like cortical tuber,skin lesions,as well as renal lesions being more vulnerable.Therefore,more active treatment should be given to the patients with TSC2 gene mutation.

Objective To evaluate the olfactory functions in patients with Parkinson's disease (PD) with a smell identification test developed for the Chinese population and to examine possible factors contributing to PD patients' olfactory performance.Methods Fifty-five patients with idiopathic and nondementia PD and 55 demographically matched healthy controls (HC) were recruited and their olfactory identification tested using the Chinese Smell Identification Test developed by the Institute of Psychology at Chinese Academy of Sciences.Results Olfactory identification score was significantly lower (t =-6.827,P＜0.01) in the PD group (19.3 ±5.4) as compared with the HC group (26.5 ±5.7).Of all PD patients,63.6％ scored over 1 SD below the age norm.In particular,of those with Hoehn-Yahr ratings between 2 to 3,77.8％ scored over 1 SD below the age norm.Receiver operating characteristic (ROC) curve analysis indicated that a cut-off score of 22.5 on the Chinese Smell Identification Test distinguished between PD patients and HC with a sensitivity of 74.6％ and specificity of 70.9％.Within the PD group,age,education,Hoehn-Yahr rating and Mini-Mental State Examination score were not found to correlate with olfactory performance (all P ＞ 0.05).Conclusion PD patients in China show significant impairment of olfactory identification,which is effectively captured by the Chinese Smell Identification Test.

Objective To investigate the relationship between the plasma galectin-3 level and the severity of cerebral artery atherosclerosis as well as the prognosis of patients with large artery atherosclerosis (LAA) stroke.Methods According to the TOAST classification,105 patients with LAA stroke,50 patients with small artery occlusion (SAO) stroke,33 patients with asymptomatic cerebral artery stenosis,and 60 healthy controls were enrolled.The plasma galectin-3 level was measured using enzyme-linked immunosorbent assay.According to the number of cerebral arteries with atherosclerosis,the LAA group was divided into single-branch lesions group (n =30),double-branch lesions group (n =30) and multi-branch lesions group (n =45).Plasma galectin-3 levels were compared among the three subgroups,and the associations between galectin-3 and the severity of cerebral atherosclerosis were analyzed.The LAA group patients were followed up for three months,and the value of galectin-3 on predicting the prognosis of patients with LAA stroke was assessed using the receiver operating characteristic (ROC) curve and the modified Rankin scale (mRS) scores.Results The plasma galectin-3 level in LAA group ((13.64 ± 3.08) ng/ml) was significantly higher than in SAO group ((12.20 ± 2.88) ng/ml) and control group ((11.89 ± 2.93) ng/ml;t =2.790,3.617,P =0.006,0.000).Besides,the plasma galectin-3 level in asymptomatic stenosis group ((13.94 ± 2.89) ng/ml) was significantly higher than in SAO group and control group (t =2.695,3.238,P =0.009,0.002).However,the differences between asymptomatic stenosis group and LAA group,SAO group and control group were not statistically significant.In LAA group,the plasma galectin-3 level in multi-branch lesions group ((15.02 ±2.94) ng/ml) was significantly higher than in double-branch lesions group ((13.47 ± 2.88) ng,/ml) and single-branch lesions group ((11.73 ± 2.43) ng/ml;t =2.261,5.080,P =0.027,0.000).The plasma galectin-3 level in double-branch lesions group was significantly higher than in single-branch lesions group (t =2.532,P =0.014).The plasma galectin-3 level and the range of atherosclerosis and mRS scores were positively correlated (r =0.433,0.629;P =0.000,0.000).The area under the ROC curve of plasma galectin-3 level and prognosis was 0.812 (95％ CI O.726-0.897,P =0.000).Conclusions The plasma galectin-3 level was found associated with the severity of cerebral artery atherosclerosis,but not with acute onset of LAA and SAO stroke.Galectin-3 may be involved in the inflammatory pathogenesis and development of cerebral atherosclerosis,and may have the potential to become a plasma marker for evaluating the severity of cerebral artery atherosclerosis and judging the prognosis of patients with LAA stroke.