Free radical plays a key role in the process of cerebral ischemic injury. It aggravates cell membrane damage through the peroxidation of unsaturated fatty acids, and then results in neuronal death and brain edema. Edaravone, a novel potent free radical scavenger, exerts its neuroprotective effect by inhibiting endothelial injury in cerebral ischemic areas and relieving neuronal damage. It also reduces thrombolytic therapy-induced brain edema and bleeding events after reperfusion. Clinical experiences suggest that the therapeutic time window of edaravone is very wide, and when it combines with thrombolytic therapy, it may reduce stroke mortality and promote the recovery of neurological deficit. This article reviews the course of development of edaravone from laboratory to clinical stage.

Carotid artery stenosis is one of the major causes of ischemic stroke. Recent studies have found that homocysteinemia and matrix metalloproteinase and its endogenous tissue inhibitor play important roles in carotid artery stenosis. The interventional strategies that aim at the 2 new risk factors may have extensive prospects of application in the future prevention and treatment of ischemic cerebrovascular disease.

Objective To assess the normative value of motor unit action potential of trapezius electromyography(EMG)and the amplitude of compound muscle action potential(CMAP)of accessory nerve.Methods Standard EMG and CMAP of accessory nerve were recorded from upper trapezius muscle in 100 healthy volunteers.For accessory nerve,it was stimulated in the upper sternocleidomastoid muscle and was recorded in the junction of the neck and shoulder.For trapezius EMG,it was recorded also in the junction of the neck and shoulder,includiag spontaneous activity,motor unit action potential and recruitment pattern.Results The amplitude,duration,polyphasic wave of motor unit action potential was(610.7±79.2)μV,(11.2±1.5)ms,(11.7±1.2)% respectively.The amplitude of compound muscle action potential of accessory nerve was(8.7±2.3)mV.Conclusion The electromyography of trapezius and CMAP of accessory nerve can be examined accurately.

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 investigate the relationship between plasma level of homocysteine (Hcy) and amyotrophic lateral sclerosis (ALS).Methods The differences of Hcy,Vitamin B12,and folate levels between 150 outpatients with ALS and 137 age and sex matched controls were compared in a cross-sectional study.Results Compared with the healthy controls,patients with ALS had a higher plasma Hcy level [ ( 18.47 ± 13.09) μmol/L vs ( 11.91 ± 5.37 ) μmol/L,P ＜ 0.001 ] and a lower folate level [ ( 8.39 ±4.45) ng/ml vs (10.38 ±5.01) ng/ml,P ＜0.001].In a logistic regression model using ALS as a dependent variable,plasma level of Hcy was significantly associated with ALS.A trend for higher Hcy levels in patients with shorter interval from symptom onset to diagnosis ( ODI ) was also found [ Hcy levels (20.73 ± 15.79 ) μmol/L with ODI ＜ 19 months vs ( 16.41 ± 9.73 ) μmol/L with ODI ≥ 19 months,P =0.035 ].Conclusions Plasma Hcy level is significantly increased in patients with ALS.The individuals with shorter time to diagnosis present higher Hcy level,suggesting that it may associate with the progression pace of ALS.

Objective To describe the clinical characteristics of 4 patients with concomitant amyotrophic lateral sclerosis (ALS) and Sj(o)gren' s syndrome.Methods The clinical features,laboratory findings,and electrophysioiogical manifestations etc.of these patients were analyzed.Results Four female patients all developed progressively aggregated weakness in their limb/limbs at the fifth to sixth decades of their lives.According to their neurologic findings,3 met the criteria of clinical probable ALS,while the other was diagnosed with probable laboratory-supported ALS.All these patients showed diffused patterns of neurogenic changes in electromyography tests.Motor symptoms of 2 patients transiently improved after immunomodulation treatments.Conclusion Careful screening of the Sj(o)gren' s syndrome symptoms in patients with ALS is necessary so as to deal with the treatable concomitant disease in time.

Objective To explore the clinical features and SQSTM1/p62 gene mutations in Chinese Han patients with familial amyotrophic lateral sclerosis linked superoxide dismutase 1 (SOD1) mutation (FALS-SOD1).Methods A total of 13 FALS-SOD1 probands and 100 healthy controls were studied,with DNA extracted from the peripheral blood.Sequencing was carried out at 8 exons,intron-exon boundaries and promoter region (2-kb upstream from the coding sequence) of SQSM1/p62.Clinical data were collected and all patients were followed-up.Phenotype-genotype relationship was analyzed.Results The insertion of T was found in intron 5 of SQSTM1/p62 gene [+ 1 insert T (TT ＞ TG)] in a FALS-SOD1 G16A male proband,with limbs as the symptom onset and faster disease progression than the other two SOD1 G16A probands without SQSTM1/p62 gene mutation.Conclusions The insertion of T in the intron 5 of SQSTM1/ p62 gene may promote the ALS progression by damaging p62 function in the FALS-SOD1 G16A proband.

10.3969/j.issn.2095-4344.2013.24.022

Objective:To establish a method of organotypic cerebral culture.So as to pave the way for building some neurodegenerative disease models.Methods:Organotypic cerebral cultures were prepared from prefrontal brain of neonatal SD rats. After culturing 7 to 14 days, 3 weeks, 4 weeks and 8 weeks, respectively, cerebral slices were fixed, dehydrated and sectioned in cryostat. The sections proceeded with Nissl staining and neurofilament high molecular weight (NFH) immunohistochemical staining.The difference was observed between controls and cultured slices using normal rats as controls. Results:Nissl staining showed that pyramidal neurons in cultured slices were increased in volume and lightened in staining. The delaminating construction was clear from 1 to 4 weeks after culturing. In cultured slices, immunohistochemical staining showed that NFH positive pyramidal cells appeared on layer Ⅴ on the tenth day and on both layers Ⅴ and Ⅲ after culturing 12 days. In the control group, NFH positive pyramidal cells appeared on layer Ⅴin 5-day-old rats, and appeared on both layers Ⅴand Ⅲ in over 3-week-old rats. In cultured cerebral slices, the number of pyramidal neurons on layer Ⅴ in M1 area was invariable from 12 days to 2 months. Conclusion:Orgaotypic cerebral culture can be used to study postnatal development for neocortex and build some in vitro models for neurodegenerative diseases.

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.