Stiff-person syndrome(SPS) is a rare neurologic disorder with autoimmune features.It is characterized by progressive,severe muscle rigidity or stiffness most prominently affecting the spine and lower extremities.It is frequently associated with diabetes and other autoimmune diseases.The diagnosis of SPS is based on clinical grounds,however,aided by electromyography and high serum titers of antibodies against glutamic acid decarboxylase.Intravenous immunoglobulin infusions,plasmapheresis and steroids provide additional and lasting benefit.Moreover,benzodiazepines,baclofen,tiagabine and botulinum toxin A can afford symptomatic relief.

Objective To explore the expression of breast cancer susceptibility gene 1 (BRCA1) in human colorectal cancer and its correlation with the efficacy of platinum-based chemotherapy.Methods Seventy-eight cases of colorectal cancer patients treated with platinum-based chemotherapy after surgery were collected.The surgical specimens of them were taken.Fourteen normal colonic mucosa specimens and 12 non-cancerous tissues of colorectal cancer specimens were obtained.The expression of BRCA1 in tissues was detected by immunohistochemistry and analyzed by x2 test.The colorectal cancer patients were followed up for survival time.Comparison of survival analysis was performed by Kaplan-Meier survival curves and Log-rank test.Results The positive rate of the BRCA1 expression in colorectal cancer tissue (52.6 ％,41/78) was lower than that of para-cancer tissue (11/12,x2 =6.518,P=0.011) and normal colonic mucosa tissue (13/14,x2 =7.949,P=0.005).The poorer the differentiation of colorectal cancer,the lower the positive rate of BRCA1 (x2=14.160,P=0.001).The median disease-free survival time of BRCA1 negative colorectal cancer patients was 51.0 months (95 ％ CI:47.7 to 54.4 months),which was longer than that of BRCA1 positive patients (45.0 months,95 ％CI:36.6 to 53.4 months,x2 =4.367,P=0.032).Conclusions Receiving oxaliplatin based chemotherapy may be a survival benefit for BRCA1 negative colorectal cancer patients.The expression of BRCA1 may be an index for chemotherapy options and prognosis evaluation for colorectal cancer patients after surgery.

Objective To explore the indication,surgical procedure and the postoperative treatment of breast conserving modified radical mastectomy for early stage breast cancer .Methods The clinical data of 21 patients with early stage breast cancer underwent breast conserving modified radical mastectomy were analyzed retrospectively. Results All patient recovered uneventfully. All the external configuration of the breast were fine. There were no recurrence and no complications in this series. Conclusions The breast conserving modified radical mastectomy is recommended for the early stage breast cancer. The external configuration of breast is fine postoperatively, and patients have higher survival quality.But follow up is necessary in order to find and treat the recurrence of breast cancer.

Objective Neonatal hypoglycemia is a common metabolic disease during neonatal period and a few children with neonatal hypoglycemiamay show a series of neurologic impairment.This study was to explore the neurobehavioral development of preschool children with neonatal hypoglycemia.Methods 37 children with neonatal hypoglycemia in Neonate Department of Anhui Provincal Children's Hospital from March 2009 to May 2013 were investigated by Chinese Preschool-Children Temperament Scale and Conners Symptoms Questionnaire for Parents, together with the information collection of perinatal period, clinical data and family environment.At the same time 50 controls were randomly chosed of the same period.Results The children of hypoglycemia group and control group were mainly with easy temperament and inclined easy temperament.In the nine dimensions of temperament, there was statistical difference in approach-withdraw between two groups (P<0.05).The incidence of behavioral problem in hypoglycemia group is 24%, involving learning problem and psychophysical problem, and there were statistical difference between two groups in this aspect (P<0.05).Conclusion Neonatal hypoglycemia affects the neurobehavioral development of preschool children.Individualized treatment and education intervention should be taken according to the children's temperament and neurobehavioral development.

Objective:To observe the effect of the proteininhibitor of activated STAT 3 (PIAS3) on the proliferation and apopto-sis of U251 glioma cells after PIAS3 expression was inhibited by RNAi. Methods: Three RNAi expression vectorstargeting PIAS3 were constructed and transfected into CHG-5 cells by liposomein vitro. The most efficient RNAi vector was subsequently selected by examiningthe mRNA expressions of PIAS3 in the transfected cells by semi-quantitativeRT-PCR. The selected RNAi vector was then transfected into U251 cells. After 48h of transfection, the mRNA and protein expressions of PIAS3 in glioma cellswere examined by semi-quantitative RT-PCR and western blot. Apoptosis wasobserved by flow cytometry using a double-staining method with FITC-con-jugatedannexin V and PI. Flow cytometry was also applied in cell cycle assay. Results:RNAidownregulated the mRNA (P<0.01) and protein (P<0.01) expressionsof PIAS3 in transfected cells.RNAi promoted the resistance of U251 cells to apoptosisand subsequently al-tered the cell cycle. A high percentage of G2 phaseand a low percentage of Sphase were observed in U251 cells. Conclusion:The down-regulation of PIAS3arrested U251 cells in the G2 phase andinduced the resistance of U251 cells to apoptosis.

Objective To determine the relationship between normal weight obesity (NOW) and cardiovascular risk factors.MethodsA total of 940 adults who received a health examination in out hospital were recruited in a cross-sectional study,and 407 with a body mass index (BMI) of18.5 to 25 kg/m2were enrolled for further analysis.Body fat percentage ( BF％ ) was measured by bioelectrical impedance analysis (BIA),and the subjects were assigned to the NOW group ( BF％ ≥25％ for male or BF％ ≥35％for female) or the control group ( BF％ ＜ 25％ for male or BF％ ＜ 35％ for female).Cardiovascular risk factors and their detection rates were compared between the two groups by using independent sample t test and x2 test.The correlationbetweenNOW and cardiovascular risk factors was assessedbylogistic regression.Results The prevalence of NOW in men and women were13.1％ and14.9％,respectively.The prevalence of NOW was increased with age ( x2 =6.90,P ＜0.05 ).Systolic blood pressure (SBP),diastolic blood pressure ( DBP ),total cholesterol ( TC ),triglycerides ( TG ),low-density lipoprotein cholesterol ( LDL-C) and serum uric acid (SUA) were significantly increased in the NOW group (t values were 2.97,2.44,2.54,5.09,2.71and 3.91,respectively; all P ＜ 0.05 ) ; whereas high-density lipoprotein cholesterol ( HDL-C) was significantly decreased in the NOW group (t =-3.90,P ＜ 0.05 ).The prevalence of hypertension,hyperglycemia,high triglyceride,low HDL-C,dyslipidmia and hyperuricemia was increased in the NOW group in comparison with the control group ( x2 values were 6.76,5.58,14.50,11.97,10.97 and 8.76,respectively;allP＜ 0.05 ).LogisticregressionshowedNOWincreasedtheriskof hypertension,hyperglycemia,dyslipidmia or hyperuricemia by 2.186,2.120,2.088 or 4.175 times.After adjustment for age and gender,the risk for hyperuricemia was decreased to 3.491,but remained statistically significant higher.Conclusions NOW may be correlated with cardiovascular risk factors,and those with NOW could be at higher risk for cardiovascular diseases.

Objective To investigates the feasibility of diffusion tensor imaging (DTI) and diffusion tensor tracking (DTT) for evaluation of the effect of childbearing history on female pelvic floor muscles. Methods Forty-six healthy females were divided into two groups: nulliparous and primiparous. MR conventional sequences and DTI were acquired. The optimized FA threshold value was obtained by regulating the FA to fiber tracking. The two groups were compared in terms of ADC, FA, VRA and T2-WT. Results (1)The DTT of FA 0.18 got the highest score in fiber tracking . ( 2 ) The ADC of nulliparous subjects and the subjects who had given birth were (1.24 ± 0.11) ×10-3 mm2/s, (1.33 ± 0.11) ×10-3 mm2/s (P = 0.017). There were no statistical differences in FA, VRA and T2-WT between the two groups (P > 0.05). Conclusions The optimized FA threshold of fiber tracking in pelvic floor muscles is 0.18. DTI and DTT may be used to evaluate the effect of childbearing history on female pelvic floor muscles.

Objective At present, the risk factors for cognitive impairment in epilepsy patients are not quite clear.This study was to explore the impacts of the clinical features, emotional health and quality of life (QOL) on the cognitive function of the adult patients with mild cognitive impairment (MCI).Methods Using the Montreal Cognitive Assessment (MoCA) and Mini Mental State Examination (MMSE) scales, we evaluated the cognitive functions of the 109 adult epileptics of the outpatient clinic of neurology in Jinling Hospital.We assessed their emotional health with Hamilton Depression Scale-24 (HAMD-24), estimated their QOL with Quality Of Life in Epilepsy-31 (QOLIE-31), and collected their baseline clinical data by questionnaire survey.Results There were 67 cases of MCI (61.5%) among the 109 patients.The residential area was the strongest predictor of MCI in the adult epileptics (OR=0.226, 95% CI: 0.082-0.627).Among other risk factors of post-epileptic MCI were the total scores of HAMD-24 (OR=0.770, 95% CI: 0.644-0.921) and QOLIE-31 (OR=0.712, 95% CI: 0.575-0.880), QOL (OR=1.070, 95% CI: 1.015-1.128), cognitive function (OR=1.120, 95% CI: 1.043-1.203), and social function (OR=1.103, 95% CI: 1.035-1.175).Conclusion The incidence of MCI is high in adult patients with epilepsy.The development and progression of post-epileptic MCI can be delayed by more emphasis on the evaluation of cognitive function, emotional health, and quality of life.

Objective To investigate the electrophysiological characteristics of atrial tachycardia (AT) originating from the side of the bundle of His, and to analyze the efficacy and safety of transcatheter radiofrequency ablation via the right side of the bundle of His and left noncoronary sinus of aorta for the treatment of AT. Methods A total of 12 patients with AT originating from the side the bundle of His, which was confirmed by conventional electrophysiological study and atrial activation patterns, were included in this study. The patient’s age ranged from 12 to 64 years old with a mean of (47.4±14.6) years. The mapping was carried out at the right side of the bundle of His and radiofrequency ablation was performed. When the ablation procedure failed, or the junction zone rhythm or atrioventricular block occurred, ablation via the left noncoronary sinus of aorta was employed. Results Atrial stimulation could repeatedly induce and terminate AT in all the 12 patients, the average cycle length was (327±76) ms. TA was terminated within 10 seconds after the start of ablation in 10 patients during their AT attacks. Ablation was unsuccessful in 2 patients. Ablation via the right side of His bundle was successfully accomplished in 2 patients, and the ablation via the left noncoronary sinus of aorta was successfully carried out in 8 patients. The average follow-up time was 1-6 years, and no recurrence of AT was observed. Conclusion Atrial tachycardia originating from the side of the bundle of His has certain electrophysiological characteristics, and transcatheter radiofrequency ablation is safe and effective for its treatment. Ablation via the left noncoronary sinus of aorta should be considered as a strategy of priority.

Objective To investigate the effects of B.adolescentis and L.acidophilus in treatment of dextran sulphate sodium (DSS)-induced ulcerative colitis(UC) in mice and their potential mechanisms. Methods Seventy-five BABL/C mice were randomly divided into control,saline (NS),B.adolescentis BF0624 treatment (B),L.acidophilus LT0637 treatment (L) and salicylazosulpha-pyridine treatment (S) groups.Except control group,the other four groups were received DSS to induce ulcerative colitis. The weight-loss,fecal trait and bleeding were recorded every day.Colonic length and histological scores were evaluated on day 3,5 and 7.The gene and protein expression of hot shock protein (HSP)70, glucocorticoid receptor (GR),interleukin (IL)-10 and tumor necrosis factor (TNF)-α were detected by Western blot and reverse polymerase chain reaction (RT-PCR) respectively.Results B.adolescentis BF0624 and L.acidophilus LT0637 could relieve the inflammatory reaction of the experimental UC.The DAI scores were 1.84±0.4 in L group on day 3,which was lower than that in NS group (2.8±1.0).The DAI scores in all treatment groups were decreased on day 5.Compared with NS group[(8.1±0.6)cm ], the colon length on day 8 were (9.0±0.6)cm in B group,(9.35±0.6)era in L group and (8.8±1.1)cm in S group (P＜0.05).The colonic mucosa was improved pathohistologically in L group (6.0±1.0) on day 8.The expression of HSP70 and IL-10 in B and L groups were up-regulated and the expression of TNF-α was down-regulated.Conclusions Both B.adolescentis BF0624 and L.acidophilus LT0637 were effective in treatment of acute ulcerative colitis.The potential mechanism of two probiotics may be related with up-regulation of HSPT0 and IL-10 expressions and down-regulation of TNF-α expression.