青少年肌阵挛癫痫治疗的长期反应

作者回顾性分析了50例青少年肌阵挛癫痫患者的临床资料,并通过随访估价药物的治疗反应。病人和方法青少年肌阵挛癫痫患者共50例,男16、女34例。随访时年龄18～46岁;随访时间2个月～9年。全部患者均有肌阵挛发作史;45例(90%)有多种全身发作;25例(50%)有肌阵挛发作和全身强直阵一挛发作史;2例(4%)有肌阵挛发作和失神发作;18例(36%)有肌阵挛发作、全身强直-阵挛发作和失神发作。8例(16%)有光源性抽搐反应。18例(36%)有原发性全身发作家族史。紧张刺激时全部患者都有症状加剧;情绪急剧变     

依托咪酯诱导肌阵挛与血流动力学及血钾的关系

目的观察依托咪酯诱导后肌阵挛与血流动力学及血钾的关系。方法择期全身麻醉手术患者100例,ASAⅠ～Ⅱ级,随机分为两组。观察组在静注依托咪酯前预注维库溴铵0.015 mg/kg,2 min后静注依托咪酯及维库溴铵行气管插管;对照组静注依托咪酯前不用维库溴铵。观察维库溴铵预处理后患者呼吸频率及血氧饱和度（SpO2）变化;两组肌阵挛的发生率;不同程度肌阵挛组平均动脉压（MAP）、心率（HR）及血K＋的变化。结果维库溴铵预处理后观察组呼吸频率及SpO2无明显变化（P〉0.05）;观察组无肌阵挛发生,对照组发生率为56%（P〈0.01）;2级及3级肌阵挛组MAP、HR较诱导前升高,3级肌阵挛组血K＋较诱导前升高。结论预注小剂量维库溴铵可减少肌阵挛发生率,依托咪酯引起的肌阵挛可能与其通过某种机制导致K＋外流引起神经肌肉接头后膜去极化有关。     

副癌性斜视性眼阵挛-肌阵挛-小脑性共济失调综合征研究进展

Orzechowski于1913年将"与注视方向无关的双眼完全性无节律地快速、冲动性和多向性的不规则异常眼球运动"命名为斜视性眼阵挛(opsoclonus).此后发现斜视眼阵挛可作为神经症状单独存在,也可与肌阵挛(头、躯干、四肢、软腭、咽喉、横膈)及小脑性共济失调并存,故又称斜视性眼阵挛-肌阵挛-小脑性共济失调综合征.     

家族性皮质肌阵挛震颤癫痫三例临床和电生理特点分析

家族性皮质肌阵挛震颤癫痫(FCMTE)是以皮质震颤、肌阵挛和双侧强直阵挛发作(BTCS)为主要表现的常染色体显性遗传性疾病,为探讨FCMTE的临床和电生理特点,作者对空军军医大学第一附属医院西京医院脑电监测中心收治的3例FCMTE患者的临床和电生理特点进行研究并随访。3例患者均为女性,表现为以上肢为主的震颤、肌阵挛,BTCS,癫痫性肌阵挛发作(包括眼睑肌阵挛发作),发作间期脑电图均为各导多棘波或(多)棘慢综合波,2例存在合眼敏感。给予抗癫痫药物(左乙拉西坦1例,丙戊酸钠1例,丙戊酸钠+氯硝西泮1例)治疗。随访1年以上,3例均无临床发作。说明需要关注FCMTE的癫痫性肌阵挛发作(包括眼睑肌阵挛发作)及合眼敏感,其在FCMTE中的意义尚需进一步研究。     

L-5-羟色氨酸治疗肌阵挛

作者以往曾报导用L-5-羟色氨酸(L-5HTP,5-羟色胺的前体)与Carbidopa(周围脱羧酶抑制剂)合并治疗3例缺氧性脑病所致意向性肌阵挛患者的效果(Lancet Ⅰ:1285,1974)。本文报导用此法治疗15例不同病因的肌阵挛(特发性癫痫、头部创伤、进行性肌阵挛性癫痫、家族性原发性肌阵挛、婴儿痉挛     

布托啡诺预处理对依托咪酯全麻诱导时诱发肌阵挛的影响

目的 观察布托啡诺预处理对依托咪酯全麻诱导时诱发肌阵挛的影响.方法 将100例ASA Ⅰ～Ⅱ级患者随机分为B组和S组各50例,分别给予布托啡诺0.015 mg/kg和等量生理盐水;给药2 min后,给予依托咪酯0.3 mg/kg开始麻醉诱导.依托咪酯给药2 min后,观察患者肌阵挛发生情况.结果 B组发生肌阵挛8例（16.0％）,其中轻度4例、中度3例、重度1例;对照组发生肌痉挛37例（74.0％）,其中轻度7例、中度11例、重度19例;两组肌阵挛发生率比较,P＜0.01.S组中男性患者肌阵挛发生率为80.7％、女性为75.0％,P＞0.05;20～35岁肌阵挛发生率为72.7％、36～ 50岁为82.3％、51 ～65岁为72.7％,P＞0.05.结论 依托咪酯全麻诱导前2 min静脉给予布托啡诺可有效抑制依托咪酯诱发的肌阵挛.     

伴眩晕的成人眼阵挛-肌阵挛综合征临床分析

回顾性分析2021年2月至2022年3月就诊于解放军总医院第一医学中心神经内科2例成人眼阵挛-肌阵挛综合征(OMS)患者临床表现、体征、辅助检查及诊治经过, 并对临床转归进行随访, 以提高对伴眩晕的成人OMS的认识水平。两例均为女性, 发病年龄分别为66岁和42岁, 病程中均有眩晕、眼阵挛及肌阵挛症状, 分别检出了抗γ-氨基丁酸B(GABAB)受体抗体及抗N-甲基-D-天冬氨酸(NMDA)受体抗体, 前者筛查出乳腺肿瘤。两例患者在给予免疫调节治疗后临床症状均得到缓解。OMS是一组罕见的临床综合征, 应规范其临床评估流程, 积极寻找潜在病因。     

神经副肿瘤综合征17例临床特点分析

分析17例神经副肿瘤综合征（NPS）患者的临床特点，I临床类型为Lambert-Eaton肌无力综合征8例，周围神经病6例，亚急性小脑变性2例，斜视眼阵挛-肌阵挛1例；原发肿瘤为肺癌、卵巢癌、肝癌等。分析认为。NPS临床表现形式多样，自身免疫是其可能发病机制，认识其临床特点有利于早期诊断，及早发现潜在的肿瘤。     

创伤性膈肌破裂早期诊治探讨

目的探讨影响创伤性膈肌破裂早期诊断和治疗效果的因素。方法对18例创伤性膈肌破裂患者的发病原因、部位、合并伤、诊断经过、手术方法进行回顾分析。结果所有患者均治愈。结论早期诊断及正确的手术方法是提高治愈率及减低并发症的关键。     

正常人及膈肌衰弱病人的膈神经刺激

受试者26例膈肌衰弱病人并以84例健康人作对照,年龄分别为26～73岁和21～79岁,身高分别为157～200cm 和152～205cm。用隔离刺激器发放的方波脉冲经皮刺激膈神经。刺激电极(直径5mm)置于环状软骨水平,胸锁乳突肌后缘。刺激频率1Hz作用时间0.1ms,电压80～160V。记录膈肌动作电位的电极置于第7和第8肋间隙。     

不同频率信号式功能性电刺激对老年综合性卒中单元偏瘫患者上肢运动功能的影响

目的 探讨不同频率信号式功能性电刺激对综合性卒中单元偏瘫老年患者上肢运动功能的影响.方法 90例综合卒中单元偏瘫老年患者随机分成3组,即100 Hz信号式功能性电刺激组设定为A组,50 Hz信号式功能性电刺激组设定为B组,无电刺激组设定为C组.所有老年患者均接受规范化康复治疗,分别在人组时、治疗10次后,评价他们的肌力水平（徒手肌力评定即MMT）、日常生活活动能力（ADL）评分（改良Barthel指数即MBI）和上肢运动功能评分（简化Fugl-Meyer量表上肢部分评定即FMA）,并进行统计学分析.结果 3组治疗后的MMT、MBI、FMA值较治疗前均有明显改善.治疗后A、B组MMT值有高于C组的趋势.治疗后A、B、C组MBI值无明显差异.3组治疗后的FMA值均较治疗前有明显改善.结论 100 Hz和50 Hz的信号式功能性电刺激对卒中单元偏瘫老年患者上肢运动功能有显著疗效,不同频率电刺激组之间无明显差异,但疗效优于无电刺激组.     

重复经颅磁刺激对急性缺血性卒中患者运动功能恢复的影响

目的 探讨高频(10 Hz)、低频(1 Hz)以及θ短阵快速脉冲模式(theta burst stimulation,TBS)重复经颅磁刺激(repetitive transcranial magnetic stimulation,rTMS)对急性期缺血性卒中偏瘫患者运动功能恢复的影响.方法 将72例急性缺血性卒中偏瘫患者采用随机数字表法随机分组,分别给予低频(18例)、高频(18例)、TBS(18例)rTMS或假刺激(对照组,18例),每天1次,连续治疗2周.所有入组患者在rTMS治疗前(第1 次治疗之前当天)和治疗后(最后1次治疗之后当天)应用Fugl-Meyer运动功能评分(Fugl-Meyer Assessment,FMA)和美国国立卫生研究院卒中量表(National Institutes of Health Stroke Scale,NIHSS)进行神经功能评价.结果 4组患者治疗后FMA和NIHSS评分均较治疗前出现显著改善(P均<0.05).高频组、低频组以及TBS组rTMS治疗后FMA和NIHSS评分均较对照组出现显著改善(P均<0.05),各治疗组间无显著性差异.结论 高频、低频和TBS rTMS均能改善急性缺血性卒中偏瘫患者的运动功能恢复,各治疗模式间无显著性差异.     

Effects of imaging parameters on automated cardiac flow measurement using color Doppler echocardiography

The color Doppler echocardiographic technique has been developed for automated cardiac flow measurement (ACM). This study evaluated the effect of imaging parameters on stroke volume measurement. Cardiac output derived from the ACM method was compared with that obtained from pulsed wave Doppler in 36 patients (26 men and 10 women, mean age 54 +/- 8 years) in whom clear two-dimensional and color Doppler images of the left ventricular outflow tract were obtained. The effects of frame rate, color gain and moving target indicator (MTI) filter on cardiac output were evaluated in 13 patients (8 men and 5 women, mean age 49 +/- 6 years). Using ACM at a frame rate of 30 Hz, optimal color gain setting and high-frequency MTI filter (cutoff frequency: 915 Hz), there was an excellent correlation in cardiac output between the ACM and pulsed wave Doppler methods (stroke volume: r = 0.91, SEE = 0.32 l/min). Using ACM at a frame rate of 30, 22 and 15 Hz, the differences in stroke volume were 4.4%, 5.2% and 8.6%, respectively. When color gain was reduced, left ventricular stroke volume reduction was 12.1% (-2 dB), 18.9% (-4 dB). In contrast, there was no significant change in stroke volume measurement when color gain was increased. There was a significant decrease in stroke volume using the low-frequency MTI filter [cutoff frequency: 467 Hz (-35.6%)] and medium-frequency MTI filter [cutoff frequency: 703 Hz (-13.4%)]. Color Doppler imaging parameters are extremely important for automated assessment of cardiac output.     

Ventilatory muscle function during exercise in air and oxygen in patients with chronic air-flow limitation

Ventilatory muscle function was examined at rest and during exercise on a cycle ergometer in 8 patients with moderate to severe chronic air-flow limitation (FEV1, 32 +/- 4% predicted) in air and in oxygen. The diaphragmatic electromyogram (EMG) was measured using an esophageal electrode. In addition, measurements of esophageal (Pes), gastric (Pga), and transdiaphragmatic (Pdi) pressures and abdominal wall movements were made. Patients exercised to exhaustion at a constant submaximal workload (80% of maximal power output) inspiring air or 40% O2 in random order on separate days. At end-exercise in air, tidal inspiratory Pes swings were 36 +/- 4% of static maximal inspiratory Pes, and inspiratory Pdi swings were 45 +/- 7% of the static maximal Pdi. Arterial oxygen saturation decreased from 91 +/- 2% at rest to 80 +/- 5% at end-exercise in air. During exercise in air, 5 patients demonstrated a persistent and greater than 20% fall in the ratio of high frequency (150 to 350 Hz) to low frequency (20 to 46 Hz) power (H/L) of the diaphragmatic EMG, indicating impending diaphragmatic fatigue, and 2 patients had paradoxical motion of the abdominal wall. Exercise time at the same constant work load increased from 3.0 +/- 0.6 min in air to 6.4 +/- 1.2 min in O2 (p less than 0.005). At the comparable time during exercise in O2 to end-exercise in air, minute ventilation was less by 13% (p less than 0.005), which was entirely attributable to a lower frequency of breathing. Mean inspiratory and expiratory flows and heart rate were all significantly lower.(ABSTRACT TRUNCATED AT 250 WORDS)     

Dyspnea and ventilatory muscle function during exercise on air and oxygen breathing in patients with chronic obstructive pulmonary disease (COPD)]

Exercise performance and dyspnea in COPD patients have been shown to be improved with supplemental oxygen, although the exact mechanisms resulting in the improvement are still unclear. The purpose of the present study was to investigate a possible relationship between ventilatory muscle function and sensation of dyspnea (modified Borg Scale) during exercise on 20% O2 and 30% O2. Eight patients with COPD (FEV1 1.06 L +/- 0.30 L) exercised on a treadmill two times breathing compressed air or 30% oxygen with a one hour rest in between. The electrical activity of the diaphragm (EMGdi) was recorded with esophageal electrodes, and that of the sternomastoid muscle (EMGsm) was recorded from the fine wire electrodes. The ratio of high frequency (150 to 350 Hz) to low frequency (20 to 47 Hz) power (H/L) of EMGdi was analyzed to assess diaphragmatic fatigue, which was defined as a 20% fall of H/L ratio from the control value. Flow, volume, O2 Saturation (SaO2), esophageal pressure (Pes) and transdiaphragmatic pressure (Pdi) were measured. Tension time index (TTdi) was calculated from Pdi and the ratio of inspiratory time to total time for one cycle (Ti/Ttot). At rest, we measured maximal esophageal pressure (Pesmax), maximal transdiaphragmatic pressure (Pdimax), maximal integrated EMGdi (EMGdimax) and EMGsm (EMGsmmax). Incremental exercise was discontinued by dyspnea. The walking distance achieved was increased in all patients on 30% O2. Dyspnea and desaturation were significantly improved on 30% O2 breathing, and the onset of diaphragmatic fatigue was delayed.(ABSTRACT TRUNCATED AT 250 WORDS)     

创伤性膈疝的临床诊断与手术治疗效果研究

目的探讨创伤性膈疝的临床诊断与手术治疗效果。 方法随机选取2011年4月至2016年7月,新疆维吾尔自治区人民医院胸外科收治的42例创伤性膈疝患者,回顾性分析患者的临床资料,研究创伤性膈疝临床诊断与手术治疗效果。 结果42例创伤性膈疝患者手术均获得成功,手术成功率为100%。平均手术时间为(121.4±23.79)min,平均住院时间为(15.8±2.37)d。所有患者均无并发症,痊愈后出院。 结论创伤性膈疝,临床诊断存在难度,患者入院后,需动态监测临床症状,联合影像学检查,为早期诊断提供保障。以患者实际病情为依据,给予适宜的手术治疗,效果显著,值得广泛推广。     

AN INTRACRANIAL ARTERIOVENOUS MALFORMATION AND PALATAL MYOCLONUS RELATED TO PSEUDOXANTHOMA ELASTICUM

Pseudoxanthoma elasticum is a rare hereditary disorder of elastic tissue with central nervous system manifestations due to occlusive vascular disease and aneurysm formation. Here we report the first recorded case of an intracranial arteriovenous malformation (AVM) in a patient with pseudoxanthoma elasticum. The AVM, which was located in the pons, also had an unusual manifestation, namely palatal myoclonus.     

Dyspnea and inspiratory muscle function during exercise in severe chronic obstructive pulmonary disease (COPD)]

Role of inspiratory muscle function in the genesis of dyspnea in COPD patients has yet to be fully studied. The present study investigated the possible relationship between respiratory muscle function and the sensation of dyspnea (modified Borg Scale) during exercise in eight patients with severe COPD (FEV1 0.61L +/- 0.15L). The electrical activity of the diaphragm (EMGdi) was recorded with esophageal electrodes, and that of sternomastoid muscle (EMGsm) was recorded from the surface electrodes. The ratio of high frequency (150 to 350 Hz) to low frequency (20 to 47 Hz) power (H/L) of EMGdi and EMGsm was analyzed to assess inspiratory muscle fatigue, which was determined by a 20% fall of H/L ratio from the control value. Flow, volume, esophageal (Pes) and transdiaphragmatic pressure (Pdi) were measured. Tension time index (TTdi) was calculated from Pdi and the ratio of inspiratory time to total time for one cycle (T1/TTOT). At rest, we measured maximal esophageal pressure (Pesmax), maximal transdiaphragmatic pressure (Pdimax), maximal EMGdi (EMGdimax) and EMGsm (EMGsmmax). Progressive treadmill exercise test was performed, stating with 3 minutes' walk at a speed of 0.75 mph at 0% grade, subsequently increasing the velocity at a rate of 0.25 mph and the elevation at a rate of 4% per stage. Exercise was discontinued at maximum respiratory effort sensation. Six of the eight patients showed diaphragmatic fatigue at their maximal exercise. With diaphragmatic fatigue, these patients were extremely dyspneic (Borg scale 9 or 10), and terminated the exercise. There were high correlations between the Borg scale and VE/MVV, and Pes/Pesmax and EMGsm/EMGsmmax, however, TTdi and EMGdi/EMGdimax showed less correlation with the Borg scale.(ABSTRACT TRUNCATED AT 250 WORDS)     

Effect of pulmonary vein isolation on the left-to-right atrial dominant frequency gradient in human atrial fibrillation

BACKGROUND: We previously demonstrated the existence of a left-to-right atrial dominant frequency gradient during paroxysmal but not persistent atrial fibrillation (AF) in humans. One possible mechanism of the left-to-right dominant frequency gradient involves the role of the pulmonary veins (PVs) in AF maintenance. OBJECTIVES: The purpose of this study was to examine the effect of PV isolation on the dominant frequency gradient and outcome after PV isolation. METHODS: Patients with either paroxysmal or persistent AF were studied. Recordings were made from catheters in the coronary sinus (CS), posterior right atrium (RA), and posterior left atrium (LA) during AF before and after PV isolation. Mean left-to-right dominant frequency gradient was measured before and after segmental PV isolation. Patients were followed for AF recurrence after PV isolation. RESULTS: Twenty-seven patients with paroxysmal (n = 15) or persistent (n = 12) AF were studied. In the paroxysmal group, baseline dominant frequency was greatest in the posterior LA with a significant left-to-right atrial dominant frequency gradient (posterior LA = 6.2 +/- 0.9 Hz, CS = 5.8 +/- 0.8 Hz, posterior RA = 5.4 +/- 0.9 Hz; P <.001). After PV isolation, there was no regional difference in dominant frequency (5.9 +/- 0.7 Hz vs 5.7 +/- 0.6 Hz vs 5.7 +/- 0.7 Hz, respectively; P = NS). In the persistent AF group, there was no overall difference in dominant frequency among sites before or after PV isolation (P = NS); however, patients with long-term freedom from AF after PV isolation had a higher left-to-right dominant frequency gradient compared with patients with recurrent AF (0.4 vs 0.1 Hz; P <.05). CONCLUSION: PV isolation results in a loss in the left-to-right dominant frequency gradient in patients with paroxysmal AF. This finding supports the critical role of PVs in the maintenance of ongoing paroxysmal AF. Patients with persistent AF and a baseline left-to-right dominant frequency gradient have a better success rate with PV isolation alone compared with patients without a dominant frequency gradient.     

Protective role of collaterals in patients with coronary artery occlusion

We reviewed the clinical, hemodynamic and angiographic data of 105 patients with right coronary artery occlusion and of 82 patients with left anterior descending coronary artery occlusion, subdivided into 3 groups by the presence and quality of collaterals to the occluded coronary (absent, poor or good collaterals). We found that patients with right coronary artery occlusion and good collaterals had a lower frequency of diaphragmatic myocardial infarction (60%) than patients with absent collaterals (100%) (P < 0.01). In addition, in patients with old diaphragmatic myocardial infarction, both poor and good collaterals were associated with a lower frequency of severe asynergy of the diaphragmatic left ventricular segments at left ventriculography (54% and 14%, respectively), compared to patients with no collaterals to the right coronary artery (92%, P < 0.02 vs. poor collaterals, P < 0.001 vs. good collaterals). In contrast, in patients with left anterior descending coronary artery occlusion, the presence of either poor or good collaterals to the left anterior descending coronary artery was not associated with a lower frequency of old anterior myocardial infarction, or, in patients with old anterior myocardial infarction, with a less severe asynergy of the anterior left ventricular segments.Our results suggest that collaterals are effective in protecting the diaphragmatic left ventricular wall in patients with right coronary artery occlusion, but not the anterior left ventricular wall in patients with left anterior descending coronary artery occlusion.