Objective:To analyze the clinical, electromyographic and tremor characteristics in tremor patients with neuronal intranuclear inclusion disease (NIID).Methods:From May 2018 to April 2023, 34 patients with NIID diagnosed in the Department of Neurology, Beijing Tiantan Hospital of Capital Medical University were retrospectively included. Sixteen patients with tremor of at least one limb and (or) head were in tremor group, and 18 patients without tremor were in control group. The clinical, electromyogram and tremor data of all participants were summarized, the clinical features and electromyogram differences of the 2 groups were compared, and the tremor characteristics of patients with NIID were analyzed.Results:The proportion of female patients in the tremor group was higher than that in the non tremor group (12/16 vs 7/18, P=0.045). The proportion of upper and lower limb peripheral nerve damage in the tremor group was lower than that in the non tremor group (2/16 vs 9/18, P=0.030), with statistical significance. There was no significant difference between the 2 groups in higher cortex and autonomic nervous dysfunction. The amplitude of composite muscle action potential and sensory nerve action potential in all patients was normal or slightly decreased; some patients experienced a decrease in motor and sensory fiber conduction velocity. The proportion of motor and sensory nerve conduction velocity slowing in the non tremor group was higher than that in the tremor group [motor nerve:41.7%(30/72) vs 17.2%(11/64), χ 2=9.64, P=0.002;sensory nerve:38.9% (35/90) vs 20.0%(16/80), χ 2=7.19, P=0.007]. The number of cases of postural tremors in different parts among the 16 patients was as follows: 13 in the upper limbs, 7 in the lower limbs, and 6 in the head; static tremor: 8 cases in the upper limbs, 3 cases in the lower limbs, and 5 cases in the head. At rest, the frequency of tremors in different parts of the body was as follows: upper limb (5.3±1.1) Hz, lower limb (4.2±0.4) Hz, and head (3.9±0.6) Hz. The difference in tremor frequency among the 3 parts was statistically significant ( F=3.92, P=0.047); Pairwise comparison showed that the frequency of head tremor was lower than that of upper limb tremor, with a statistically significant difference ( P=0.020). In a postural state, tremor frequency in different parts was as follows: upper limb (5.4±0.9) Hz, lower limb (5.0±0.7) Hz, head (3.9±0.7) Hz. There was a statistically significant difference in tremor frequency among the 3 parts ( F=6.65, P=0.005). Further pairwise comparison revealed statistically significant differences in tremor frequency between the patient′s head, upper and lower limbs ( P=0.001, P=0.022). Synchronous tremor rhythm was predominant, with occasional alternations or synchronous+alternations. There was no harmonic tremor spectrum was observed. Conclusions:NIID patients with tremors were more common in female patients.The degree of peripheral nerve damage was milder than those without tremors. The site and form of tremor were diverse, with a dominant frequency of 4-6 Hz, mainly synchronous rhythm, and no harmonic spectrum. Postural tremors were common in the limbs.

目的 提高对周围神经高兴奋综合征(peripheral nerve hyperexcitability syndromes,PNHS)的认识.方法 回顾性分析以瘙痒为突出表现的PNHS患者2例,总结其临床特点、诊疗经过及随访转归,结合文献予以分析讨论.结果 病例1为60岁男性,为抗接触蛋白相关蛋白2(contactin-associated protein-like 2,CASPR2)/抗富含亮氨酸胶质瘤失活蛋白1(leucine rich glioma inactivated 1,LGI1)抗体双阳性患者,表现为双下肢疼痛、肉跳及严重瘙痒,病程中有多汗、二便异常及情绪、睡眠障碍.肌电图表现为周围神经高兴奋性及四肢交感皮肤反应(sympa-thetic skin response,SSR)异常.最终诊断为Morvan综合征;抗CASPR2/LGI-1抗体相关自身免疫性脑炎.患者2为37岁女性,为CASPR2抗体阳性患者,表现为双下肢的疼痛及肉跳、下腹部及会阴部持续瘙痒,病程中有多汗、二便异常及情绪、睡眠障碍,既往体健.肌电图可见肌颤搐电位及SSR异常.电流感觉阈值(Current perception threshold,CPT)检测提示2000 Hz电流刺激感觉神经纤维阈值降低.该患者最终诊断为Isaacs综合征.2例患者在免疫治疗及对症治疗后均达临床缓解.复查肌电图不同程度改善.结论 严重瘙痒可以为伴CASPR2抗体阳性的PNHS患者的突出表现,识别其临床表现并完善相应的血清学及肌电图检测有助于早期诊断,积极的免疫治疗可有效缓解病情.

73岁女性.左眼2个月前行经巩膜睫状体光凝术.全麻下行左眼超声乳化白内障吸除人工晶状体联合内窥镜下黏弹剂房角分离术.术中前房角分离时出现下方角膜后弹力层脱离,前房注入黏弹剂复位角膜后弹力层,术后降眼压、抗炎治疗,3周后角膜后弹力层复位.

目的 探讨周围神经病后震颤患者的临床及电生理特点.方法 回顾性收集2016年3月至2019年10月北京天坛医院神经病学中心收治的周围神经病后震颤患者15例为周围神经病组,另选取年龄、性别相匹配的特发性震颤患者11例为对照组.收集入组对象的临床资料及神经电生理结果,分析周围神经病后震颤的临床特点,并比较两组震颤部位、频率、半宽功率、全宽功率、节律形式等震颤特点.结果 (1)临床特点:单纯感觉异常3例,单纯力弱4例,感觉异常伴力弱8例;免疫介导性周围神经病12例(12/15)、腓骨肌萎缩症2例(2/15)、双侧肘管综合征1例(1/15).(2)肌电图特点:感觉、运动神经纤维均受累10例,纯运动纤维受累4例,仅感觉纤维受累1例.(3)震颤部位、频率、功率及节律:15例均有双上肢远端姿势性震颤,双上肢静止性震颤2例,双下肢姿势性震颤2例.双上肢姿势性震颤频率4.6～11.1 Hz,平均(7.78±1.99)Hz,持物1000 g震颤频率为(7.86±1.84)Hz,意向性震颤频率为(7.41±1.98)Hz,3种状态间震颤频率比较无统计学差异(F=0.225,P>0.05),而半宽、全宽震颤功率比较均有统计学差异(H=16.280,P<0.05;H=35.267,P<0.05);上下肢震颤节律形式为同步或同步+交替.(4)与特发性震颤区别:11例特发性震颤均有双上肢姿势性震颤,双上肢静止性震颤4例,头部震颤4例;周围神经病组姿势、持物1000 g、意向性震颤频率与特发性震颤组比较差异均无统计学意义(均P>0.05);周围神经病组意向性震颤出现率低于特发性震颤组(40％比100％,P<0.05);两组意向性震颤半宽、全宽功率比较差异有统计学意义(Z=-3.478,P<0.05;Z=-2.964,P<0.05).结论 周围神经病后震颤以免疫介导性周围神经病多见,常表现为双上肢姿势性震颤,频率较宽泛,与持物1000 g及意向性震颤频率无统计学差异,节律形式为同步或同步+交替,意向性震颤的出现率及震颤功率均低于特发性震颤者.

目的 总结脑血管疾病后Holmes震颤的临床、影像和电生理特点.方法 回顾性分析2015年8月-2019年8月就诊于首都医科大学附属北京天坛医院的4例脑血管疾病所致Holmes震颤患者,对其临床、影像及电生理资料进行分析总结.结果 4例患者中2例由高血压性脑出血引起,另外2例分别由脑动静脉畸形和脑海绵状血管瘤破裂出血引起.Holmes震颤出现于原发病后1～24个月,表现为病灶对侧肢体震颤,以上肢多见.头颅MRI检查显示2例患者病灶仅累及丘脑,2例同时累及丘脑和中脑.震颤分析显示静止、姿势、意向及持物1000g几种状态下震颤的峰频率均在2.6～3.8Hz,意向状态震颤半宽功率高于静止状态.主动肌与拮抗肌在静息时以同步收缩为主,姿势、意向和持物时以交替收缩为主.3例接受普拉克索治疗均有不同程度缓解.结论 Holmes震颤多由累及中脑、丘脑部位脑血管疾病引起,表现为2～4Hz低频震颤,意向状态震颤明显,部分患者多巴胺受体激动剂治疗有效.

Objective ToanalyzethechangesofF-wave minimum latencies (Fmin),distal motor latencies (DML)intheearlystageofpatients withamyotrophiclateralsclerosis (ALS)andthecorrelation betweenFmin,DMLandthecompoundmuscleactionpotentials(CMAP),negativeareaofCMAP,toseekthe evidencesupportingpreferentialvulnerabilityofthefastestmotorneuronintheearlystageofALS. Methods Forthisstudyweenrolled42patientswhomettherevisedEIcriteriafordefiniteandprobablediagnosisALSand selected46healthyvolunteerswith matchingsexcomposition,ageandheightasthecontrolgroup. Standard electrophysiologystudies were performedin allthe participantsand DML,Fmin,Fmax,CMAP negative amplitudeandnegative waveareainthe mediannerve werecollected. Wecompareddifferencesoftheabove indicatorsbetweenthetwogroupsandused Pearsoncorrelationanalysisto measurethecorrelationbetween DML,Fmin,FmaxandCMAPnegativeamplitude,negativewaveareainthemediannerve. Results Compared withthecontrolgroup,Fminwaslonger(P<0.01)intheALSgroup. AlthoughDMLtenttobeaprolongedin theALSgroup,butnostatisticallysignificantdifferencewasfound.CMAPnegativeamplitudeintheALSgroup decreasedsignificantlywhencompared withthecontrolgroup (P<0.01),sodidthenegative waveareaof CMAP (P<0.01). The mediannerveCMAPnegativeamplitudeandnegativewaveareaintheALSgroup werenegativelycorrelated with DMLafterheightadjustment (r=-0.433,P=0.005;r=-0.320,P=0.041),sodidthesamepatterntotheFmin (r=-0.669,P<0.001;r=-0.597,P<0.001),butno correlationwasfoundwiththeFmax (r=-0.283,P=0.072;r=-0.220,P=0.166).Conclusions In earlyALSpatients,thesignificantlynegativecorrelationbetweenFminandCMAPnegativeamplitude,negative wavearearevealedpreferentialvulnerabilityofthefastestmotorneuronsintheearlystageofALS.

Objective To optimize the method to elicit the facial nerve F wave and to establish its reference values and its related parameters, in order to provide an electrophysiological basis for facial nerve assessment in patients with hemifacial spasm (HFS). Methods Thirty-six healthy volunteers and 22 patients with HFS underwent this project. Compound muscle action potentials (CAMP, or M waves) and F waves were elicited by stimulating the marginal mandibular branch of the facial nerve and recorded with needle electrodes placed in the mentalis muscle. The association between F parameters and Cohen Grading of the HFS patients were analyzed. Results There were no significant differences in F parameters between men and women or between the two sides in control subjects. Minimal latency of F wave (Fmin) and mean latency of F wave (Fmean) had positive correlations with head circumference (Fmin r=0.449, P=0.013; Fmean r=0.391, P=0.033), but had no correlations with age nor height. Patients with HFS had prolonged duration of F wave (Fdura) in spasm side, compared with normal side (13.1 ms vs. 9.5 ms, P<0.01) and healthy subjects (13.1 ms vs. 9.7 ms, P<0.001), and increased ratio of F-wave amplitude and M-wave amplitude (F/M) in spasm side, compared with normal side (6.9% vs. 3.8%, P<0.001) and healthy subjects (6.9% vs. 3.7%, P<0.001). F/M exhibited a positive correlation with Cohen Grading (r=0.538, P=0.001). Conclusion A clear facial nerve F wave can be achieved by stimulating the marginal mandibular branch of the facial nerve, which provides an objective basis for evaluation of the facial nerve function in HFS patients with increased F/M and prolonged Fdura.

Objective To investigate the diagnostic value of spontaneous activities in genioglossus of amyotrophic lateral sclerosis (ALS).Methods A retrospective analysis of 79 patients diagnosed with ALS from January 2014 to December 2015 in Beijing Tiantan Hospital Affiliated to Capital Medical University was made.The patients were divided into two groups according to the clinical examination:with (44 patients) or without bulbar symptoms (35 patients).The course of disease,spontaneous potentials and ALS-Functional Rating Scale (ALS-FRS) scores were discussed,together with the association between semi-quantitative evaluation of spontaneous potential of the genioglossus and ALS-FRS score.Results In ALS patients,the overall positive rate of glossal spontaneous potentials was 69.6％ (55/79),among which the positive rate was 82.9％ (29/35) in patients with bulbar symptoms and 59.1％ (26/44) in patients without bulbar symptoms,with statistically significant differences between the two groups (x2=5.206,P＜0.05).While the positive rate in sternocleidomastoid was only 13.9％ (11/79),and there was no statistically significant difference between patients with or without bulbar symptoms.Semi-quantitative evaluation of fibrillation potentials in genioglossus muscle was correlated with ALS-FRS score (r=-0.258,P＜0.05).Conclusion Compared with sternocleidomastoid muscle,genioglossus muscle has a higher positive rate of spontaneous potentials and a higher diagnostic value in patients with subclinical bulbar symptoms.

Objective To compare the difference of lowering effect by phacoemulsification cataract extraction combined with endoscopic cyclophotocoagulation (ECP) between 210 degree and 240 degree treatment.Design Comparative case series.Participants 70 cases (70 eyes) with refractory glaucoma in Beijing Tongren Hospital were included.Methods 70 eyes were randomly divided into two groups,35 cases were undergoing 210 and 240 degree ECP treatment respectively.All the patients were selected by clear corneal incision phacoemulsification (PE) cataract extraction combined with ECP and goniosynechialysis.The average follow-up period was (21.69± 8.93) months.Main Outcome Measures Intraocular pressure (IOP) and IOP lowering pressure medications.Result Preoperative IOP was (26.13±10.58)mmHg in group 210 degree and (24.85±10.78)mmHg in group 240 degree (P=0.620).IOP decreased (7.81±12.55) mmHg and (8.57±11.40)mmHg respectively in the two groups at last follow-up(P=0.131).The average number of medications was (2.71± 1.66)in group 210 degree and (2.40±1.65) in group 240 degree before operation(P=0.429),which decreased to (0.46±0.98) in group 210 degree and (0.34±0.80) in group 240 degree at last follow-up (P=0.595).No serious complication during the operation was observed.Conclusion There was no statistical significance in the control of IOP between the 210 degree group and 240 degree group by phacoemulsification cataract extraction combined with ECP.

[目的]探讨急性脑梗死阿替普酶静脉溶栓后出血并发症的时间特征及出血发生后对病人预后的影响。[方法]选取在神经内科进行阿替普酶静脉溶栓治疗病人100例，以出血并发症为主要观察指标，观察病人各部位出血并发症第一次发生时间及总体发生时间，并比较两组病人的出入院时日常生活能力(Barthel)评分、压疮(Braden)评分和美国国立卫生研究院卒中(NIHSS)评分。[结果]年龄、收缩压、Barthel 评分、Braden 评分和 NIHSS 评分是影响静脉溶栓后出血的危险因素。溶栓后24 h 是出血并发症的高发期。[结论]卒中单元护士应根据溶栓后不同部位出血并发症的时间特征进行给予有效的病情观察和护理干预。

脑卒中是指急性起病,由于脑局部血液循环障碍所导致的神经功能缺损综合征,症状持续至少24h[1].根据2012年中国卫生统计年鉴,脑血管病是我国城市居民的第3位死亡原因,是农村居民第2位的死亡原因.缺血性卒中是最常见的脑卒中类型,在脑卒中患者中约占85％[2].1996年美国食品和药物管理局(Food and Drug Administration,FDA)批准使用静脉注射重组组织型纤溶酶原激活剂(Recombinant Tissue plasminogen Activator,RT-PA)治疗急性缺血性脑卒中患者,国际准则(欧洲行程计划)推荐静脉注射阿替普酶作为一线治疗药物应用于症状发作3h内的患者[3].

目的 分析以白内障摘除联合人工晶状体(IOL)、张力环植入为主要手术方式的联合手术治疗晶状体半脱位继发青光眼的疗效.设计 回顾性病例系列.研究对象 2011年7月至2013年9月北京同仁眼科中心年龄(62.3±6.9)岁的晶状体半脱位继发青光眼患者12例.方法 回顾患者手术前后的病历资料.所有患者均囊袋内植入IOL,超声乳化白内障摘除11例,白内障囊外摘除1例,张力环植入6例,联合黏弹剂房角分离7例,联合小梁切除术5例,联合术中经巩膜睫状体光凝1例,术中玻璃体水囊抽吸4例.术后平均随访(13.3±10.0)个月.主要指标 视力、眼压、并发症.结果 最后一次随访时,8/12例患者视力提高,平均提高(4.9±2.8)行;3/12例视力同术前.全部患者在未使用降眼压药物情况下眼压控制在21 mmHg以下.术中、术后无严重并发症发生.结论 本文的小样本量短期回顾性研究结果显示,以白内障摘除IOL植入联合张力环植入为主的联合手术是治疗晶状体半脱位继发青光眼的安全有效方法.

To investigate the advantage of using the medical sodium hyaluronate gel undergo goniosynechialysis, and to compare the difference of the viscoelastic agent and perfusion fluid by clinical control study. ●METHODS:Totally 103 cases (103 eyes) patients who had angle- closure glaucoma (ACG) with cataract all in Beijing Tongren Hospital affiliated to Capital Medical University, from September 2012 to February 2013. All the patients had primary glaucoma or recurrence ACG with cataract. Their intraocular pressure (lOP) could be controlled in normal value using one or two anti -glaucoma medications. We divided all the patients into two groups by chance. Experimental group had 53 cases (50 eyes), and control group had 50 cases (53 eyes). Two groups all had been performed phacoemulsification combined with goniosynechialysis by the same experiential operator. But experimental group did goniosynechialysis using the medical sodium hyaluronate gel, control group did goniosynechialysis using perfusion fluid. We observed the change of lOP, central anterior chamber depth and anterior chamber structure of two groups in postoperative 1mo. ●RESULTS: SPSS19. 0 statistics software was utilized to analyze the data, This study selected one- side test α =0. 05, adopted paired t - test or two- sample t - test. We obtained that experimental group lOP decreased 12. 42 ± 0. 04mmHg, anterior chamber depth increased 1. 276 ± 0. 201mm; that control group lOP decreased 6. 56 ± 0. 08mmHg, anterior chamber depth increased 0. 852 ± 0. 132mm. And experimental group anterior chamber angle opened more widely than the other group, the comparison had statistical significance (P<0. 05). ●CONCLUSlON: ln phacoemulsification combine with goniosynechialysis,use medical sodium hyaluronate gel do goniosynechialysis can more effectively decrease lOP. This because the medical sodium hyaluronate gel has high glutinousness, it can separate angle of anterior chamber and controlling lOP effectively.

近几年随着交通事故不断增多,车祸造成脊髓损伤的人数也呈上升趋势.我科自2002年12月至2003年6月收治脊髓损伤患者131例,采用嗅鞘细胞移植术进行治疗,取得较好效果,现将护理体会报告如下.