Amyotrophic lateral sclerosis is a rare but fatal neurodegenerative disorder.Riluzole remains the only available drug for slowing the progression of the disease.In the past few years,significant advances have been made in both our understanding of pathogenesis and the development of new therapeutic approaches.The authors review the current understanding of the pathogeneic mechanisms and drug therapy.The outcome of phase Ⅲ clinical trials will benefit the further investigation in ALS.

Objective To investigate the pathogenesis of cerebral infarction after non-cardiac surgeries according to imaging.Methods Retrospective analyses of clinical and imaging data of 17 patients with postoperative cerebral infarction(average 68 years old,total incidence 0.049％)from departments of orthopedics and general surgery were conducted during 52 months.Results Cerebral infarction occurred 39.1 hours after operation on average.Among the 17 patients,eight were detected with disturbance of consciousness,ten with hemiplegia,six with speech disorder and two with unilateral sensory disturbance.Six (35.3％)had blood lipids tests.Five(29.4％)had neck vascular ultrasound and one had intracranial magnetic resonance angiography(MRA).When discharged,one patient was declared death and ten had impaired neurological function in various degrees.Among six patients with previous stroke,one(16.7％)received neurological consultation before surgery.According to the image manifestation,ten cases were territory circulation infarcts,four centrum ovale infarcts and three watershed infarcts.Conclusions This study suggests that total incidence of cerebral infarction after non-cardiac surgeries is lower than previously reported and there is greater involvement of atherosclerosis.Patients' conditions should be closely observed within at least four days after surgeries.Preoperative assessment should be strengthened in order to avoid occurrence of postoperative cerebral infarction.

Objective To discuss diagnosis ant treatment of post traumatic fat embolism syndrome.Methods Since 1997,9 patients with FES have been treated in our hospital.These cases were analyzed on clinic features and results of treatment.Results According to Sevitt classification,there were 2 in fulminant,2 in typical,and 5 in subclinical.2 patients in fulminant died,7 patients were cured because of early diagnose and correct treatment.Conclusion Identify this disease,early and correct treatment help to improve the result.Post traumatic resuscitation is effecrive in decreasing the incidence of FES.

AIM: To explore the therapeutic effects of flunarizine combined with aspirin in the treatment of migrainous cerebral infaction. METHODS: 38 cases of patients diagnosed as migrainous cerebral infaction were respectively given flunarizine 10 mg combined with aspirin 100 mg every night for a month. The observed indices included the dysfunction scores of nervous system, the total classes of living ability, and the accumulating rate of platelet and viscosity of plasma before and after the treatment. RESULTS: Before the treatment the dysfunction scores of nervous system and the living ability of the patients were 13.51 ? 4.78 and 3.45 ? 1.13 , and after the treatment the values were 4.34 ? 1.85 and 1.79 ? 0.72 respectively (P

Objective To observe the characteristics of midbrain hyperechogenicity of amyotrophic lateral sclerosis (ALS) patients by transcranial sonography (TCS).Methods A total of 107 ALS patients,enrolled from January to July 2015 in Beijing University Third Hospital,with the diagnosis of possible or definite ALS according to revised E1 Escorial criteria were examined by transcrinal B-mode sonography.The area of midbrain and substantia nigra and the area ratio of hyper-substantia nigra/midbrain (S/M) were measured and compared between ALS and 40 age-and gender-matched controls.Results There were 29.0％ (31/107) of ALS patients and 7.5％ (3/40) of controls who displayed abnormal midbrain hyperechogenic areas for groups comparison (x2 =22.708,P ＜ 0.01).The hyperechogenic substantia nigra area and S/M were (0.40 ± 0.14) cm2 and 9.5％ (6.0％,13.0％) in ALS group,whereas (0.20 ± 0.06) cm2 and 5.0％ (2％,6.0％) in control group respectively,the difference between the two groups being statistically significant (t =12.727,Z =16.545,both P ＜0.01).No correlations of hyperechogenic area sizes in ALS patients were found in regard to age,gender,ALS duration or ALS Functional Rating Scale score (r=0.043,-0.088,0.018,0.202;P=0.251,0.512,0.894,0.190).However,there was significant correlation between TCS severity and ALS subtype (bulbar vs spinal form,r =0.386,P ＜ 0.01).Conclusion Hyperechogenicity of the substantia nigra was found in patients with sporadic ALS with a frequency higher than in controls.

ObjectiveTo observe the effect on stress response to indwelling gastric tube assisted by bronchofiberoscope and traditional way in patients with severe craniocerebral injury in intensive care unit(ICU). Methods 126 patients admitted in Department of Surgery ICU in Lanzhou University Second Hospital were randomly divided into bronchofiberoscope assisted indwelling gastric tube group(experimental group) and the traditional way of indwelling gastric tube group(control group), 63 cases in each group. In the experimental group, the rod of mirror, Olypus BF-P60, was coated with lidocaine gel, through the nasal cavity it was sent into interior, when arrived at epiglottis, the operator adjusted the mirror head and let it go into the esophagus(about 10 cm), then put a steel wire as a guide into a hole for biopsy, and simultaneously, as the operator pulled out the rod, the wire was continuously pushed slowly further inside; after the bronchofiberoscope was completely withdrawn, valelinum liquidum was used to ensure sufficient lubrication to the external and internal walls of the indwelling gastric tube ready to be sent into the stomach, afterwards the tube was slowly pushed along the guide wire to an appropriate location and then the wire was pulled out, the operator injected air, when he or she heard the gurgling sound as the gas passing water, fixed the tube. In the control group, the traditional way of indwelling gastric tube was applied. Before and after indwelling gastric tube for 1, 3, 5 minutes, the changes of systolic pressure(SBP),diastolic pressure(DBP),heart rate(HR), end-expiratory carbon dioxide partial pressure(PETCO2) and plasma concentrations of norepinephrine(NE), adrenaline, angiotensinⅡ(AngⅡ), glucose(GLU) were examined.Results The plasma levels of SBP, DBP, HR, PETCO2, adrenaline, NE, AngⅡ and blood GLU had no statistical significant differences before operation in comparisons between both groups(allP>0.05). Compared to those before indwelling the tube, the levels of SBP, DBP, HR, PETCO2, NE, adrenaline, AngⅡ and GLU at various time points after the indwelling in experimental group were of no statisticalsignificant differences(allP>0.05), while the levels of SBP, DBP,HR, adrenaline, NE, AngⅡand GLU in control group at various time points after indwelling were higher obviously than those before operation, and the level of PETCO2 was decreased significantly compared with that before operation. The differences in the above indexes were significant between the treatment and control groups at 1 minute after indwelling〔SBP(mmHg, 1 mmHg=0.133 kPa): 125.1±15.4 vs. 135.5±13.6, DBP(mmHg): 85.6±16.1 vs. 91.1±17.2, HR(bpm): 99.4±13.8 vs. 107.9±16.5, PETCO2(mmHg): 32.5±2.8 vs. 29.8±4.1, NE(ng/L): 365.4±29.7 vs. 475.7±49.9, adrenaline(ng/L): 75.4±7.2 vs. 83.6±7.4, AngⅡ(ng/L): 65.3±6.9 vs. 73.3±9.1, GLU(mmol/L): 10.1±1.9 vs. 13.4±3.0, allP<0.05〕; the differences in the above indexes remained significant between the treatment and control groups till 5 minutes after indwelling〔SBP(mmHg): 123.7±14.8 vs. 129.7±15.1, DBP(mmHg): 84.3±14.6 vs. 88.4±14.2, HR(bpm): 97.7±13.6 vs. 31.6±3.9, PETCO2(mmHg): 33.5±3.1 vs. 31.6±3.9, NE(ng/L): 363.9±31.3 vs. 457.7±48.4, adrenaline(ng/L): 74.6±7.8 vs. 83.5±8.5, AngⅡ(ng/L): 64.3±8.4 vs. 71.9±5.9, GLU(mmol/L): 9.6±2.3 vs. 12.7±3.1, allP<0.05〕.ConclusionCompared with traditional way, the indwelling of gastric tube assisted by branchofiberoscopy can induce milder stress response.

ObjectiveTo investigate the survival and migration of the neural stem cells(NSC) in the cerebrospinal fluid (CSF) circle of mice after ventricle transplantation. MethodsNSC labeled with green fluorescence protein (GFP) were implanted into the lateral cerebral ventricle of the mice. The mice were killed at time point of 24 h, 48 h, 2 weeks and 10 weeks after transplantation. The brain sections were observed and the behaviors of the mice were evaluated. ResultsGFP-positive cells were found in the lateral cerebral ventricle.Some of them migrated into the parenchyma and located in fibria-fornix, hippocampus,corpus callusum, septum,subventricle zone and beside the blood vessels at the time point of 2 weeks and 10 weeks. There were no obvious complications occured during operations which affected the outcome of growth and development. ConclusionNSC not only can survive, but also can migrate into the local parenchyma of the brain after ventricle transplantation. There were no obvious complications occured after the transplantation of NSC.

ObjectiveTo explore the characteristics of the metabolite concentrations of the precentral gyrus in healthy Chinese adults using proton magnetic resonance spectroscopy (1H-MRS).Methods 1H-MRS was carried out in 92 healthy Chinese adult volunteers. The metabolite ratios of NAA/Cr, Cho/Cr and NAA/Cho were compared and analysed.ResultsNo significant changes of NAA/Cr, Cho/Cr and NAA/Cho ratios were observed between the left and right precentral gyrus, nor between the male and female. However, the precentral gyrus NAA/Cr and NAA/Cho ratios were decreased gradually with aging and Cho/Cr ratios were increased gradually with aging.ConclusionThe characteristics of the precentral gyrus metabolite concentrations in healthy Chinese adults have been obtained.

Objective To explore the effect of giving sedatives according to the circadian rhythm in prevention of occurrence of delirium and the prognosis of patients undergoing mechanical ventilation in intensive care unit (ICU). Methods A prospective double-blinded randomized controlled trial (RCT) was conducted. The patients admitted to Department of Critical Care Medicine of the Second Hospital of Lanzhou University from July 2014 to February 2015, undergoing invasive mechanical ventilation over 12 hours were enrolled. All the patients were given fentanyl for analgesia, and they were randomly divided into simulated circadian clock group (study group, n = 35) and non-simulated circadian clock group (control group, n = 35). The patients in each group were subdivided into three subgroups according to the kinds of sedative drugs, namely dexmedetomidine group (n = 8), propofol group (n = 14), and dexmedetomidine combined with propofol group (combination group, n = 13). Visual analogue scale (VAS) standard and Richmond agitation-sedation scale (RASS) were used to control the analgesic and to quantify the depth of sedation by titrating the dose of sedative drugs, the simulated circadian clock was set to control the RASS score at 0-1 during the day, and -1 to -2 at night in study group. The RASS score in the control group was set at -1 to -2 day and night. The urine 6-hydroxy acid melatonin (aMT6s) levels at different time points in the first diurnal rhythm (06:00, 12:00, 18:00, 24:00) were determined by enzyme linked immunosorbent assay (ELISA). The incidence of delirium, severe hypotension, severe bradycardia and other adverse reactions, duration of mechanical ventilation and the time of extubation, length of ICU stay, amount of sedative and analgesic drugs used were recorded. The correlation between delirium and other indexes was analyzed by using Spearman correlation analysis. Results ① There were no significant differences in gender, age, acute physiology and chronic health evaluation Ⅱ (APACHEⅡ) score among groups. ② Urine aMT6s levels did not show circadian rhythm in both groups, aMT6s level at 06:00 in study group showed an increasing tendency as compared with the control group, but the difference was not statistically significant. ③ Compared with the control group, the incidence of delirium was significantly lowered in the study group (14.3% vs. 37.1%, P = 0.029), but no significant differences were found in the incidence of severe hypotension or severe bradycardia (20.0% vs. 25.7%, 11.4% vs. 20.0%, both P > 0.05). In simulated circadian clock group, the incidence of delirium in dexmedetomidine group was significantly lower than that of the propofol group (6.3% vs. 32.1%, P < 0.05). ④ Compared with control group with the same sedative, the duration of mechanical ventilation, extubation time, length of ICU stay were significantly shortened, and the dosage of sedative drugs used was reduced in study group (all P < 0.05). In simulated circadian clock group, the duration of mechanical ventilation in dexmedetomidine group was significantly shorter than that of propofol group and combination group (hours: 75.75±26.78 vs. 102.00±26.31 and 100.31±25.38, both P < 0.05), and the length of ICU stay was significantly shorter than that of propofol group (days: 5.75±1.04 vs. 7.00±1.52, P < 0.05). ⑤ The occurrence of delirium was positively correlated with duration of mechanical ventilation (r = 0.705), extubation time (r = 0.704), length of ICU stay (r = 0.666, all P = 0.000), and no correlation was found between the occurrence of delirium and aMT6s level at 06:00, 12:00, 18:00, and 24:00 (r = -0.135, r = 0.163, r = 0.269, r = -0.077, all P > 0.05). Conclusions Administration of sedatives according to simulating circadian time could decrease the duration of mechanical ventilation, extubation time, and the length of ICU stay, decrease the dosage of sedative drugs, and reduce the incidence of delirium. Dexmedetomidine could reduce the incidence of delirium, and improve the prognosis of patients. Trial registration Registration of clinical trials in China, ChiCTR-IPR-15006644.

Objective To establish a method which could measure tongue muscle thickness and evaluate the fasciculation of the tongue muscle by submental way ultrasonic detection , and to obtain the reference values of tongue muscle thickness in healthy volunteers , exploring its clinical application in the patients with amyotrophic lateral sclerosis (ALS).Methods In two-hundred healthy volunteers who took physical examination at the Medical Examination Center of Peking University Third Hospital , the tongue thickness was determined by measuring the distance between the upper and lower surfaces of the lingual muscles in the center of the plane perpendicular to the Frankfurt horizontal plane of the frontal section with a 3.5 MHz convex array transducer .The fasciculation of tongue muscle was evaluated using a 7.5 MHz linear array transducer in the same position .This method was used to measure the thickness of tongue muscle and to evaluate tongue muscle fasciculation in 30 patients with ALS who visited neurological clinic with the diagnosis of possible or definite ALS according to revised El Escorial criteria during the same period .We compared the tongue muscle thickness between 30 ALS patients and the same number of age-and gender-matched healthy volunteers .Results The mean value of tongue muscle thickness of males was significantly higher than that of females ((4.09 ±0.52) cm vs (3.70 ±0.56) cm, t=5.108, P<0.01), but there was no significant difference in the tongue muscle thickness between the different age groups .The mean value of tongue muscle thickness was significantly correlated with the body mass index value in healthy subjects ( r=0.412, P<0.01).No tongue muscle fibrillation was detected in healthy volunteers .In ALS group, the tongue was significantly thinner than that in healthy subjects ((3.77 ±0.58) cm vs (4.05 ±0.49) cm, t=-0.253, P<0.05).Of the total 30 ALS patients, we recognized tongue muscle fasciculation in 12 (40%).Conclusion The method to measure tongue muscle thickness and evaluate tongue musclefibrillation is reliable , and has good clinical application value in related diseases such as ALS .