Moyamoya disease (MMD) is a chronic and progressive cerebrovascular disease which is characterized by the bilateral internal carotid artery ends and (or) stenosis or occlusion of anterior cerebral artery and middle cerebral artery initial segments,compensatory proliferation of small blood vessels in the skull base and formation of abnormal vascular network.Its etiology and pathogenesis remains unclear.The present studies speculate that MMD may be a polygenic disease,inflammation,immune response,abnormal cytokine secretion,endothelial progenitor cell change and nitric oxide level change are associated with the occurrence and development of MMD.This article reviews the advances in research on the genetics and pathophysiological mechanism of MMD.

Objective To test the association between mitochondrial DNA(mtDNA) point mutations and praecox Parkinson's disease (PPD),and to investigate the characteristics of mtDNA mutations in Chinese patients with PPD. Methods Screening mtDNA A4336C, G5460A,A10398G,A13780G point mutations in 40 patients with PPD and 48 in control group was carried out by using Polymerase Chain Reaction (PCR), dot blotting, radiant developing. And sequencing was given to the nucleotide position (np)10256～np10577mtDNA of 20 patients with PPD and 20 subjects in control group.Results Out of the 40 patients with PPD, 20 (50%)had A10398G mutation. 6 (15%) had G5460A, 5(12.5%) had A13780G, 2 (5%)had A4336C, 19 (47.5%)had C10400T mutation. Out of the 48 controls, 7(14.6%) had A10398G, 24.2% had G5460A, 1 (2.1%)had A13780G and 20 (41.7%)had C10400T, but no any A4336C mutation was found in the controls. Thus, the ratios of A10398G,G5460A,A4336C,A13780G in patients with PPD were separately higher than those in the control group. Moreover significant difference was found in A10398G point mutation (P

Objective To explore the efficacy and safety of endovascular therapy in the treatment of symptomatic M1 stenosis of middle cerebral artery as well as the causes of perioperative complications.Methods Two hundred fifty-six patients with symptomatic M1 stenosis of middle cerebral artery (＞90％) confirmed by TCD,cerebral CT angiography and DSA was treated by endovascular intervention.The success rate,the changes of stenosis,longterm vascular patency rate,in-stent restenosis rate were analyzed.Results endovascular stent was successfully placed in 251 patients with the M1 part of symptomatic middle cerebral artery stenosis and the successful rate was 98.05％.Fifteen patients had complications (5.86％) which caused neurology deficits and deaths.The degree of vascular stenosis was significantly reduced after stenosis (Before vs After:92.26％±2.11％ vs 15.40％±2.60％).The mean mRS and NIHSS scores was decreased significantly.The average follow-up duration was (21.70±0.80) months,249 patients underwent a second DSA and the mean stenosis was (21.70％±0.80％).Twenty-three patients developed instent restenosis (ISR) and ISR rate was 9.24％.Recurrence ischemic stroke and transient ischemic attacks occurred in 5 patients and recurrence rate was 2.01％.Conclusion Endovascular therapy of symptomatic M1 stenosis of middle cerebral artery is safety and efficacy with low complications.The follow-up results reveal good patency rate and excellent prevention of anterior circulation ischemia.

Objective To explore the risk factors for lung cancer-related cerebral infarction . Methods The hospitalized active lung cancer patients on anti-cancer therapy with no traditional stroke risk factors, who experienced an acute cerebral infarct in the First Affiliated Hospital of Guangxi Medical University from January 2005 to December 2015, were consecutively collected as the LCRS ( lung cancer-related stroke) group.The active lung cancer patients without cerebral infarction hospitalized at the same peroid matched with the LCRS group for age and gender were collected as the LC ( lung cancer ) group. Clinical data from the two groups were analyzed .Results A total of 139 LCRS patients and 139 LC patients were enrolled in the study , with 110 male and 29 female in each group , and there were no significant difference for the mean age between the LCRS group (52.1 ±10.4 years old ) and the LC group (52.1 ± 10.1 years old).Two or more acute ischemic lesions of the brain were showed by MRI in most patients in the LCRS group (117 cases, 84.2%).Compared with the LC group, more patients in the LCRS group were found with adenocarcinoma , metastasis, elevated plasma D-dimer, CA125 and CA199 levels [ 88 cases (63.3%) vs 47 cases (33.8%);98 cases (70.5%) vs 56 cases (40.3%);(468.38 ±291.37) μg/L vs (277.59 ±191.22) μg/L;(221.42 ±146.34) U/ml vs (106.84 ±69.97) U/ml;(254.68 ±185.84) U/ml vs (97.15 ±63.64) U/ml;with all P<0.001].By logistic regression analysis of multiple factors , the elevated plasma D-dimer, CA125 and CA199 levels were showed to be independent risk factors for the cerebral infarction (OR=1.003, 95%CI 1.001 -1.004; OR=1.006, 95%CI 1.003 -1.010; OR=1.011, 95%CI 1.007-1.015).Conclusions The elevated plasma D-dimer, CA125 and CA199 levels are the risk factors for the lung cancer related cerebral infarction , which may lead to hypercoagulation and induce cerebral infarction eventually .

Objective To study the transduction efficiency of expressing human neprilysin by using a lentiviral vector (Lenti-NEP) in mouse embryonic neural stem cells (NSC) in vitro.Methods Primary NSC were harvested from C57BL/6J pregnant mouse at embryonic day 11.5 and transducted with LentiNEP.Immunofluorescent stainingand Western blot were performed to detect NEP protein expression in NSC.Degradation of amyloid beta 1-40 (Aβ1-40) by NEP protein transduced with Lenti-NEP in NSC was analyzed using ELISA and HPLC.Results Over 90％ NSC were successfully transduced with Lenti-NEP via observation of fused protein green fluorescent protein under the microscopy.Expressions of NEP transduced with Lenti-NEP in NSC and of the markers of NSC (nestin) and neuron (MAP2).The enzyme activity of 2.5 μg (21.00 ± 2.51) and 1.0 μg (15.00 ± 0.54) NEP on degrading Aβ1-40 was shown to improve significantly compared to 0.5 μg NEP(8.00 ±0.81,t =40.4 and 12.7,respectively,both P ＜0.01).The activity of NEP was inhibited in the presence of 50 μmol/L phosphoramidon (0.5 pg:0.08 ±0.01 ;1.0 μg:0.04 ±0.01 ;2.5 μg:0.05 ±0.01,t =17.2,51.3 and 14.1,respectively,all P ＜0.01).The hydrolytic cleavage on degrading Aβ1-40 by NEP was 11.4％,28.4％ and 93.7％ with incubation for 1 h,4 h and 12 h,respectively.Conclusions Lentiviral vector successfully delivers NEP gene to NSC in vitro.Targeting on NEP and NSC may provide potential therapeutic tool for Alzheimer' s disease.

Objective To explore the efficacy,safety and short-term effects of endovascular therapy in the treatment of the symptomatic high-grade basilar artery stenosis.Methods Two hundred thirteen patients with the symptomatic high-grade basilar artery stenosis (＞90％) confirmed by MRA,CTA or DSA was treated by endovascular intervention,the changes of clinical symptoms,the success rate and short-term follow-up results was analyzed.Results Endovascular stent was successfully placed in 209 patients with symptomatic high-grade basilar artery stenosis and the success rate was 98.12％.The degree of vascular stenosis was significantly reduced after stenosis (Before vs After:93.70％±2.51％ vs 11.60％±3.90％).Eight patients had complications (3.76％) including 7 cases of ischemic stroke and 1 case of subarachnoid hemorrhage.The average follow-up duration was 18.70±3.80 months.Two hundred two patients underwent a second DSA and the mean vascular stenosis was (13.80％±4.20％).Five patients developed in-stent restenosis (ISR),of which one was symptomatic.Conclusion Endovascular therapy of the symptomatic high-grade basilar artery stenosis is safety and efficacy.The 1.5 years follow-up results reveal good patency rate and excellent prevention of posterior circulation ischemia.

Objective To investigate the effect of leptin on endoplasmic reticulum stress related protein after focal cerebral ischemia in rats. Methods Ischemia was induced by occluding the middle cerebral artery in rats brain using the filament occlusion method. Forty SD rats were randomly divided into sham operation group, cerebral is-chemia group and leptin-preconditioning group. Leptin was injected subcutaneously before occlusion of blood vessel. Longa 5 score neurological function scale, body weight and brain edema changes were measured 6 hours after MCAO, and the brain was removed to detect the endoplasmic reticulum marker protein: glucose-regulated protein 78 (GRP78) and C/EBP-homologous protein (CHOP) by immunohistochemical method. Results There was no difference in the body weight changes between leptin-preconditioning group and ischemic group. In the leptin-preconditioning group, the neurological function score (1.90±0.31 vs. 2.50±0.52, P<0.05) and the degree of brain edema (3.60±0.52 vs. 7.70±0.94, P<0.001) were significantly lower than those in the cerebral ischemia group. Moreover, the expression of GRP78 in leptin-preconditioning group was significantly higher than that in ischemia group (48.69 ±5.06 vs. 35.78± 4.35, P<0.01), and the expression of CHOP was significantly lower than that of ischemia group (60.24 ±4.11 vs. 38.81±5.34, P<0.01). Conclusion Leptin can reduce the neurological deficit and may be associated with the up-reg-ulation of GRP78 protein, and down regulation of CHOP protein to weaken the endoplasmic reticulum stress caused by cerebral ischemia

Objective To investigate the diffusion changes in ipsilateral substantia nigra after a chronic striatum infarction with diffusion tensor imaging ( DTI ) and its connotation for clinical lecture.Methods Participators underwent a DTI scan and were divided into three groups. The striatum infarction (SI) group consisted of twenty patients with chronic basal ganglia infarction with striatum involved, while the non striatum infarction (NSI) group consisted of another twenty patients with chronic basal ganglia infarctions without striatum involved. The control group consisted of twenty healthy volunteers. Before the DTI scan all patients underwent a clinical evaluation with Modified Rankin Scale (mRS) and Barthol Index,and the four patients of SI group with symptoms like Parkinson disease underwent an additional evaluation with the third subscale of Unified Parkinson' s Disease Rating Scale ( UPDRS Ⅲ ). Results Compared with NSI and control groups, the infarct side substantia nigra MD of SI group increased by 30. 86 percent (t =40.07,P=0.000) and 31.42 percent (t =42. 64,P =0.000). The FA values from the three groups were not different. There were four patients with some symptoms like Parkinson disease in SI group. Compared with those patients without symptom like Parkinson disease in SI group, the infarct side substantia nigra MD of these four patients increased by 22 percent(t = 18.03, P =0. 01 ). Moreover, the infarct side substantia nigra MD of these four patients was correlated with their UPDRS Ⅲ positively ( r = 0. 97, P = 0. 03 ).Conclusions The secondary degeneration in the ipsilateral side substantia nigra after striatum infarction could be detested quantitatively with diffusion tensor imaging. The secondary degeneration in substantia nigra may be responsible for the symptoms like Parkinson disease in striatum infarction patients.

OBJECTIVETo investigate the clinical features of ischemic stroke in patients with nasopharyngeal carcinoma.

METHODSThe clinical data of the nasopharyngeal carcinoma patients with ischemic stroke treated at the First Affiliated Hospital of Guangxi Medical University between January 2006 and December 2013 were collected.

RESULTSAmong 3 822 patients with nasopharyngeal carcinoma 10 patients suffered from acute ischemic stroke. The 10 patients were males and their age ranged from 39 to 70 years old, with an average age of 51 years. All of the patients were found with squamous cell carcinoma, with metastasis in 7 of them. Among the 10 patients, only 3 patients had with traditional risk factors for ischemic stroke, but no traditional risk factor was found in other 7 patients; 2 patients showed single lesion in brain and 8 patients with two or more lesions due to the blockage of multiple cerebral arteries; 7 patients showed an elevated plasma D-dimer level.

CONCLUSIONIt is suggested that the acute ischemic stroke occurring in the patients with nasopharyngeal carcinoma commonly lacks traditional risk factors, with elevated plasma D-dimer levels and multiple lesions due to involvement of several cerebral arteries.

Adult ; Aged ; Brain ; pathology ; Carcinoma ; Carcinoma, Squamous Cell ; complications ; China ; Fibrin Fibrinogen Degradation Products ; analysis ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; complications ; Neoplasm Metastasis ; Risk Factors ; Stroke ; complications

Objective To explore the effect of apolipoprotein E (ApoE) on matrix metalloproteinases-9 (MMP-9) expression and its mechanism in astrocytes.Methods (1) Astrocytes in ApoE gene knock out (ApoE-/-) and wild type (WT) C57BL/6J suckling mice were cultured in vitro;glial fibrillary acidic protein antibodies were employed to identify the astrocytes;MMP-9.antibodies were employed to detect the MMP-9 expression in the astrocytes.(2) Astrocytes from the ApoE-/-and wild type WT C57BL/6J suckling mice were divided into activation group,antibody groups and control group randomly;cells in the control group did not give any treatment;cells in the activation group were given whooping cough toxin and thermal inactivation-mycobacterium tuberculosis H37Ra;cells in the antibody groups were given anti-interleukin-6 (IL-6),interferon gamma (IFN--γ),tumor necrosis factor-α (TNF-α) and IL-12,respectively,to inhibit their inflammatory factors.ELISA was performed to detect the concentrations of MMP-9 and tissue inhibitor of metalloproteinase-1 (TIMP-1),and the inflammatory factors concentrations ofTNF-,IL-12,IL-6,and IFN-γ.Results These two kinds of rat astrocytes could both qualitatively express MMP-9;as compared with the control group,the activation group had significantly higher MMP-9 concentration (P＜0.05);the activation group had significantly higher MMP-9 concentration as compared with the antibody groups (P＜0.05);activation group from ApoE-/-C57BL/6J suckling mice had significantly higher MMP-9 concentration than that from wild type C57BL/6J suckling mice (P＜0.05).The concentration of TIMP-1 was not significantly different among various groups (P＞0.05).The concentrations of inflammatory factors in the activation groups from two kinds of mice were significantly higher than those in the control group (P＜0.05);the concentrations of IL-6,IFN-γ TNF-α and IL-12 in each antibody group were significantly lower than those in the activated group (P＜0.05);the concentrations of inflammatory factors in ApoE-/-rats were significantly higher than those in WT rats (P＜0.05).Conclusion ApoE can regulate the MMP-9 expression by regulating the secretion of proinflammatory cytokines,which can affect the integrity of blood brain barrier.