Objective The study aimed to investigate the prevalence of cerebral small vessel disease (SVD), the prevalence of headache, and the relationship between SVD and headache in non-hereditary young stroke patients. Methods A retrospective analysis was made upon 200 patients who were hospitalized in department of neurology of Peking University Third Hospital due to first-ever ischemic stroke during January 2006 and January 2015. All patients were aged 18 to 45 years old, who underwent MR imaging (MRI) and MR angiography (MRA) within 7 days after stroke onset. Patients were divided into two groups: those with headache and those without headache. The prevalence of ischemic SVD in 2 groups, including asymptomatic lacunar infarcts (ALAs), white matter lesions (WMLs), and enlarged perivascular spaces (EPVS), were compared to study headache associated factors.Results Of the 200 patients,male had higher prevalence(male vs female=6.41:1).Of all,32.5% (65/200) had ALAs, 25.5% (51/200) had WMLs, and 30.5% (61/200) had EPVS. There were 11% (22/200) had headache during acute stroke onset. ALAs and EPVS were less frequently associated with headache (P=0.045 and P=0.021 respectively). The percentage of Non-large artery atherosclerosis type in Trial of Org 10 172 in Acute Stroke Treatment (Toast) was significantly higher in headache group than that in non-headache group[63.6%(14/22)vs 26.4%(47/178),P=0.001].The lesions located under the tentorium (brain stem or cerebellum) were significantly higher in headache group than that in non-headache group[63.6%(14/22)vs 26.4%(47/178),P<0.001].Culprit vessels in the posterior circulation were significantly higher in headache group than that in non-headache group [68.2%(15/22)vs 37.6%(67/178),P=0.006]. Conclusion Our data suggest that the lesions in young patients with headache onset during acute stroke mainly located under the tentorium (brain stem or cerebellum) and culprit vessels mostly in the posterior circulation. Onset of headache is significantly lower in the SVD group. Headache is significantly higher in the non-large artery atherosclerosis type in TOAST.

Objective To analyze the characteristics of headache and the possible cause of convexity subarachnoid hemorrhage (cSAH), so as to increase the understanding of cSAH. Methods A total of 14 cases of cSAH who came to Peking University third hospital between January 2010 and December 2014 were enrolled. Retrospective review was made upon the characteristics of headache, clinical manifestations, clinical medical records, and neuroimaging studies. Results Among 14 patients, acute headache (n=8, 57.1%) was the most common clinical manifestations, including 6 cases (75%) for unilateral. And 4 cases (50%) had a transient headache, usually lasting no longer than half an hour. There were 12 cases (85.7%) with clear etiology; there were 8 cases (57.1%) with severe stenosis or occlusion in ipsilateral internal carotid artery system of cSAH; and there were 4 cases: one with intracranial venous sinus thrombosis, one with cerebral arteriovenous malformation, one using anticoagulant and one with suspected cerebral amyloid vascular degeneration, respectively. Conclusion In the present study, acute headache was the most common clinical manifestation of cSAH. The patients with transient headache or with mild to moderate headache should be aware of the possibility of cSAH. Severe atherosclerotic stenosis or occlusion in the main branch of ipsilateral internal carotid artery system was an important cause of cSAH.

头痛(headache)是最常见的临床症状,引起头痛的疾病有许多种,其中,卒中(stroke)是导致我国人口死亡的最重要病因.不同时期,人们对头痛与卒中的关系有不同理解.二十世纪八九十年代,以头痛为主诉的患者经常被诊断为脑供血不足、血管性头痛等,并给予治疗卒中的药物治疗头痛.但现在已知,所谓"脑供血不足""血管性头痛"等的诊断本身已经存疑.实际上,绝大多数头痛属于原发性头痛(primary headache).一些观点认为,除一些特殊情况如脑出血(intracerebral hemorrhage,ICH)、蛛网膜下腔出血(subarachnoid hemorrhage,SAH)或静脉窦血栓(cerebral venous thrombosis,CVT)外,头痛与卒中的关系没有那么密切[1].这似乎从一种极端走向了另一种极端!那么,实际情况到底如何?应如何正确理解并认识头痛与卒中的关系呢?

目的 优化脊髓亚急性联合变性(SCD)的鉴别诊断.方法 重新分析文献病例.结果/结论 (1)李春岩院士等的文献一组3例"感觉性共济失调型格林-巴利综合征"很可能是VB12缺乏所致的后索病变合并了感觉神经病变的脊髓SCD亚型;"纯感觉型GBS"尚不一定存在;(2)对急性运动感觉轴索性神经病,临床应注意与经典脱髓鞘性格林-巴利综合征(或急性运动轴索性神经病)并发脊髓SCD鉴别诊断;严重缺氧刺激造血对神经系统VB12的竞争以及肠道感染(前驱腹泻)所致的VB12缺乏是SCD的病因;(3)对脊髓SCD临床,应重视Marchiafave–Bignami病、Wernicke脑病、肌型脚气病、营养性弱视、脊髓前动脉综合征及肌萎缩侧索硬化等疾病的鉴别.

Objective To investigate the clinical features of anti-NF155 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Methods We analyzed a CIDP patient with specific clinical features,detected the serum anti-NF155 IgG4 antibodies by flow cytometry (FCM) and immunohistochemistry and reviewed the literature.Results This patient has anti-NF155 antibodies of IgG4 subtype.He has a feature of younger onset,distal acquired demyelinating symmetric neuropathy with sensory ataxia and tremor.Symmetric hypertrophy of nerve roots can be seen on MRI.Myelinated fibers are moderately decreased and demyelinated.Conclusions The anti-NF155 IgG4 antibodies positive patient has specific features and is confirmed by screening the serum anti-NF155 IgG4 antibodies.Due to its poor response to Ⅳ immunoglobulin (IvIg),a better understanding and identification of this disease is necessary to optimal treatment.

在八年制见习带教中引入了腰椎穿刺术,基于《北京大学医学部临床基本技能操作指南》及《北京市住院医师规范化培训腰穿考核标准》规范操作标准,理论授课与实际操作相结合,通过仿真模型进行实际操作,提高了学习积极性,并且锻炼操作能力.同时,强调无菌操作和自我保护的重要性,并在操作过程中注重人文关怀,促使学生以后给真正的患者进行腰穿操作时能够顺利完成腰穿操作,减少并发症的出现.

慢性炎性脱髓鞘性多发性神经根神经病 ( chronic inflammatory demyelinating polyradiculoneuropathy , CIDP)是一类由免疫介导的慢性运动感觉性周围神经病. 该病在世界范围内较为常见,发病率为(1 ~9)/10 万[1]. 根据临床表现,CIDP可分为经典型和变异型. 经典型CIDP表现为对称的肢体无力和感觉障碍,腱反射减弱或消失,病程为慢性进展或缓解复发并持续≥8周. 2010年欧盟神经学会/周围神经学会诊断标准[2]将变异型CIDP分为远端获得性脱髓鞘性对称性神经病( distal acquired demyelinating symmetric )、非对称型( Lewis-Sumner syndrome )、纯运动型、纯感觉型和局灶型.

Objective To explore the significance of serum myoglobin detection in the diagnosis and evaluation of Kennedy`s disease (KD).Methods The level of serum myoglobin (Myo) was detected in 60 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients.Results The serum Myo level and abnormal rate in KD group were significantly higher than those in ALS group (all P<0.001).The serum Myo level was positively related with the disease course of KD (r=0.665,P<0.01).There was no significant difference of serum Myo level and disease course of ALS (r=-0.047,P>0.05).There was no overlap of serum Myo level between the two groups.There was no significant difference of serum Myo level and CAG repeated number of ALS (r=-0.193,P>0.05).Conclusion Myo is likely as an easy and sensitive biomarker, used to identify the KD and special type of ALS, and used in the evaluation of the KD condition in the future.

偏头痛与眩晕或头晕存在密切关系.在2013年"国际头痛学会"制订的"第三版国际头痛疾病分类—β测试版"中,首次在附录中增加了"前庭性偏头痛(vestibular migraine,VM)"的诊断,并制定了详细的诊断标准.本文将对"前庭性偏头痛"的诊断标准、临床表现、鉴别诊断和治疗等做一简要综述.

偏头痛是中青年最常见的原发性头痛,女性与男性的比例为3:1 [1].在美国,偏头痛的患病率为12%[1],在亚洲地区,成年女性偏头痛患病率为10%,男性为3%[2].世界卫生组织将严重偏头痛归为影响生活的慢性疾病之一[3].根据临床特点,偏头痛有多种分型,如先兆型和无先兆型;根据发作频率,偏头痛又可分为慢性偏头痛(chronic migraine,CM)和发作性偏头痛(episodic migraine, EM)[4].国际头痛协会将CM定义为每月头痛天数≥15 d,持续至少3个月,同时头痛本身符合无先兆偏头痛的特点;将EM定义为每月头痛天数﹤15 d[5].CM约占全部偏头痛的20%,其人群总体患病率约为2%,对生活质量的影响更显著[6].

Objective To guide clinicians to strengthen tumor screening in patients with acute cerebral infarction by analyzing the clinical manifestation,laboratory examinations and imaging features of patients with acute cerebral infarction as the first manifestation of occult malignant tumors. Methods From January 2013 to January 2015,1139 patients with acute cerebral infarction admitted to the Department of Neurology,Peking University Third Hospital were collected. Eight of them (0. 7% )were found to have somatic malignancy during hospitalization. The clinical manifestations,risk factors,imaging changes,Ddimer levels and other laboratory results and prognosis were reviewed,and the related literature was reviewed and discussed. Results Eight patients with cerebral infarction as the first manifestation of malignant tumor,their ages were 58 -78 (70 ±3)years old,6 were males,2 were female. Five of them were lung cancer. Intrahepatic cholangiocarcinoma,colon cancer and prostate cancer were in 1 case respectively. Three patients who had multiple cerebral infarctions died of respiratory failure during hospitalization,and the intervals between diagnosis and death were 49,95 and 28 d,respectively. Five patients improved the Ddimer test,including 2 of them had elevated levels of D-dimer (0. 73 μg/ L and 3. 0 μg/ L respectively);in 8 patients with improved creatine kinase isoenzyme MB (CK-MB)test,the CK-MB levels were elevated in 2 patients (31 U/ L and 49 U/ L respectively),and one of the patients was diagnosed as acute myocardial infarction. All the patients improved head magnetic resonance imaging (MRI)diffusion-weighted imaging (DWI)and cerebral vascular examination,and only one patient with cerebral infarction was consistent with the stenotic vessels. Six patients had bilateral cerebral hemisphere infarctions,and multiple cerebral infarction occurred in the anterior and posterior circulation simultaneously,and MRI-DWI indicated that the cerebral infarction lesions were numerous small or large area cerebral infarctions in multiple vessels. Conclusion In patients with multiple cerebral infarction with unknown etiology,especially those with early recurrent stroke,attention should be paid to the possibility of somatic malignancy,and the early recurrence of acute cerebral infarction suggests poor prognosis.

1995年，美国国立神经疾病与卒中研究所（NINDS）发表研究，认为阿替普酶静脉溶栓是缺血性脑血管病超早期的标准治疗方法［1］。随着欧洲协作性急性卒中研究-Ⅲ和第3次国际卒中试验的公布，静脉溶栓的时间窗和年龄范围被进一步扩大，同时更加强调静脉溶栓的重要性［2-3］。近年来，动脉内取栓取得了突破性进展，并被新版美国卒中协会卒中急性期治疗指南所推荐［4］，但动脉内取栓是在静脉溶栓的基础上进行，且仅能在部分有条件的医疗中心完成。因此，对于大多缺血性卒中急性期患者，阿替普酶静脉溶栓仍是最重要和最基本的治疗手段。本研究以指南推荐、循证医学证据等为依据，针对静脉溶栓后抗血小板聚集的治疗时机、药物选择、使用剂量等问题进行综述回顾。

肯尼迪病（ Kennedy′s disease，KD）是一种晚发型的X连锁隐性遗传病。患者均为男性、起病隐袭，表现为缓慢进展的肢体近段和舌肌的萎缩、无力［1］。我们收治了1例经基因检测确诊的KD患者，尚未出现肢体无力等典型症状，但体检可见舌肌萎缩、男性乳腺发育、肢体姿势性震颤等体征，辅助检查如肌电图等亦有异常改变，报道如下。

目的 探索教师标准化患者(TSP)+病例导引教学(CBL)模式解决短暂性脑缺血发作(TIA)课临床教学“老大难”问题的可行性.方法 分别对八年制临床医学系2012级(38人)和2011级(38人)的TIA课采用TSP+CBL教学法和单纯TSP教学法.学习结束时,比较分析两组教学课堂考核成绩和教学效果调查问卷自评分.用t检验评价2种方法的教学效果.结果 TSP+CBL组学生课堂考核平均分达(88.76±3.48)分,显著高于TSP组的(85.27±3.01)分(P＜0.05).TSP+CBL组尤在临床思维、与人合作和解决问题能力方面成绩明显高于TSP组(P＜0.01).TSP+CBL组对教学效果调查问卷的平均自评分为(92.29±1.56)分,显著高于TSP组的(85.27±3.01)分(P＜0.01).TSP+CBL组在“对教材内容理解掌握程度”、“对新教学法满意程度”、“有利理论与临床实践结合”3个方面显著高于TSP组(均P＜ 0.01).结论 以TSP+CBL的教学模式值得推广,对此类发作-间歇型疾病的临床教学具有普遍性意义.

缺血性卒中急性期约有60％～75％的患者血压增高（＞140／90 mmHg）［1］。即使既往无高血压病史的患者，在卒中急性期血压也可能增高［2］。长期以来，不主张在卒中急性期积极降压，机制在于卒中急性期血压增高是机体维持脑灌注压的代偿反应，此时降低血压有可能导致脑灌注压不足，扩大缺血半暗带，加重神经功能缺损［1］。但另一方面，大量研究数据显示，缺血性卒中急性期血压增高与短期死亡和长期残疾等不良预后明确相关［2-3］。在卒中急性期，高血压患者卒中复发、出血转化、脑水肿的发生率明显增加。

Objective: To investigate whether or not the involvement degree of extensor and flexor muscles in patients with Kennedy's Disease (KD) was different by measuring the diameter of biceps and triceps brachii muscles using Magnetic Resonance Imaging (MRI). Methods: All the subjects were outpatients or hospitalized patients from Department of Neurology, the Third Hospital of Peking University, 2011-2015. Subjects included 30 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients. The right upper arms of all subjects were examined by muscle MRI. The diameters of biceps and triceps brachii muscles were measured, then the ratios were calculated. Additionally, the forces of these muscles were recorded. Results: The ratio of diameters of biceps to triceps brachii muscles were 1.08±0.14 in KD patients and 0.99±0.15 in ALS patients. The difference was statistically significant (P<0.05). The ratio of forces of biceps to triceps were 1.17±0.12 in KD patients and 0.98±0.28 in ALS patients. The difference was statistically significant (P<0.01). In KD patients, the fatty infiltration was more serious in triceps than biceps. At the same time, in ALS patients, the fatty infiltration was similar in triceps and biceps. Conclusion: The involvement degree of extensor and flexor muscles in KD patients is different.

Objective To evaluate the changes and clinical significance of motor unit number estimation (MUNE) in patients with Kennedy’ s disease (KD) .Methods An automated incremental stimulating technique was used to estimate the motor unit number of thenar and hypothenar muscles from 30 healthy subjects without nerve and muscle disease and 20 patients definitely diagnosed as KD .At the same time ,compound muscle action potentials (CMAPs) of median nerves and ulnar nerves were detected in all subjects .MUNEs of the thenar and hypothenar muscles were compared between the two groups . The abnormal rates were compared between MUNEs and CMAPs in KD patients .At the same time ,the correlation of MUNEs and course of disease were analyzed .Results The MUNE value of 60 normal thenar muscles in 30 normal controls was 251.12 ± 68.89 ,and the value of the hypothenar muscles was 235.63 ± 63.02 , respectively . The MUNE values of thenar and hypothenar muscles in 20 cases with KD were 82.16 ± 49.37 and 94.26 ± 44.56 ,respectively .The differences of MUNE values of both thenar and hypothenar muscles between normal subjects and KD patients were significant (P<0.01) .The abnormal ratio of MUNE of thenar and hypothenar muscles in KD patients was 85.0% and 80.0% ,respectively ;compared with the abnormal ratio of CMAPs of median nerves and ulnar nerves in KD patients (15.0% ) ,the differences were significant (P<0.01) .The MUNE value was negatively correlated with duration of the disease (P<0.05) .Conclusions As an atraumatic electrophysiological technique ,MUNE may be helpful to early and accurately judge the lower motor neuron damage in KD patients .

习总书记在年初的中央财经领导小组会议上提出,2016年工作的重点是“供给侧改革”.“供给侧改革”的根本目的是提高社会生产力,落实好以人民为中心的发展思想.从生产领域加强优质供给,减少无效供给,扩大有效供给,使供给体系更好适应需求结构变化.自此,“供给侧改革”作为结构性改革的重要一极开始被真正重视.

Background The Chinese Government has announced plans to reform provider-payment methods at public hospitals by moving from fee-for-service to prospective and aggregated methods including diagnosis-related groups (DRG) to control health expenditures. In 2012, Beijing pioneered China's first DRG payment system in six hospitals. The aim of this study was to explore the effectiveness of Beijing's DRG pilot reform in reducing expenditures, out-of-pocket payments, and potential behaviors to circumvent payment control, including readmissions, selection, and cost-shifting. Methods We obtained discharge data from the Beijing Health Insurance Bureau for the period January, 2010, to September, 2012, from 14 tertiary general hospitals, which included six hospitals piloting DRGs within a portion of their payment system and eight control hospitals that implemented purely fee-for-service-based payment. Using a differences-in-differences design, we used hospital discharge data to assess the pilot's impact on cost containment. We did regression analyses to assess associations of DRG payment with outcome variables of health expenditures, length-of-stay, and out-of-pocket payment. The study was approved by Peking University Committee for Biomedical Researches. Findings Post-reform, there were 36 780 cases using DRG payments and 50 322 cases in the control hospitals paid through fee-for-service. Our findings showed that DRG payment led to ¥1251 (6·2%) reductions in health expenditure and ¥647 (10·5%) reductions in out-of-pocket payment per admission. Length of stay or cost-shifting did not differ between DRG and non-DRG cases. Readmission rates were reduced (–1·4%) with DRG payment relative to non-DRG. About 19 814 (35%) potential DRG cases, however, were reverted back to fee-for-service-based payment. Hospitals processed the payment of older patients with more complications through fee-for-service payment, circumventing the effectiveness of payment reform. Interpretation For China and other low-income and middle-income countries to fully implement prospective payment systems, such as DRG payments, without allowing circumvention seen in a partially implemented system, it might first be necessary to strengthen core monitoring and technological systems that would support feasibility of DRG payments. Continuous evidence-based monitoring and assessment, partnered with adequate management systems, are necessary to enable these countries to broadly implement DRGs and refine payment systems. Funding China Medical Board (grant number CMB-OC-12-120).

Objective To develop a scale to assess Kennedy's disease (KD) based on patients' clinical characteristics and to validate its application in patients.Methods We developed KD1234 Scale following the model of Amyotrophic Lateral Sclerosis-Functional Rating Scale.Using this new scale,we assessed patients with genetic diagnosis of KD and evaluated the reliability and validity of the new scale.Reliability was analyzed using Cronbach' s α coefficient and split-half reliability.Validity was analyzed by content validity and structure validity.Results The Cronbach' s α coefficient of the total scale was 0.789 and split-half reliability coefficient was 0.868.The α of breathing with total score was 0.3-0.4,α of written,language and swallowing with the total score was 0.4-0.5,α of other items with the total score was ＞ 0.5 (P ＜ 0.01 in each of above α).Four common factors extracted by exploratory factor analysis had well correspondence between the scale construction and the theoretical construction.Factor loading was ranged from 0.541 to 0.864 for each item.The duration of the disease showed some correlation with the score of KD1234 Scale.Conclusions The KD1234 Scale demonstrates high reliability and validity.The duration of the disease indicates negative correlation with KD1234 Scale scores.The KD1234 Scale can be used in clinical research to evaluate the condition of KD patients quantitatively.