Botulinum toxin injection in patients with hereditary spastic paraparesis

In an open label study, we analyzed the efficacy of botulinum toxin injection at the lower limbs of patients with hereditary spastic paraparesis (HSP). Fifteen patients who showed disabling spasticity with no or poor effect of oral treatment were recruited consecutively. Botulinum toxin was injected (400 U; Botox^®) into the spastic muscles identified by clinical examination (equinus, varus, and pathological hip adduction). Patients were regularly assessed from the first day to the fifth month: spasticity (Ashworth), motor strength, range of movements, Functional Ambulation Categories (FAC), gait parameter, Rivermead Motor Assessment, self-analysis of benefit and satisfaction. We observed a moderate and significant ( P  < 0.05) reduction of ankle plantar flexor and hip adductor spasticity, with a partial increase in the range of the active and passive motion at the ankle and in gait velocity. At an individual level, six of 15 patients showed an increase in gait velocity. The FAC and RMA did not change. Patients often reported partial improvement in foot position and lower limb propulsion, and fair satisfaction. In conclusion, botulinum toxin injection can be effective in HSP patients with relatively ancient spasticity. This technique can be introduced into the therapeutic panel, which also includes physiotherapy, oral treatment and baclofen pump.     

Fibrovascular polyp of the esophagus requiring esophagectomy

Fibrovascular polyps of the esophagus are rare, with only 110 cases reported in the world literature to date. Dysphagia is the most common symptom. The diagnosis is usually made by barium swallow or upper endoscopy, but almost a third of cases can be missed with these studies. Treatment is surgical. Only four cases in the literature underwent esophagectomy for removal. We present a female patient with a fibrovascular polyp of the esophagus who required a transhiatal esophagectomy to safely remove this mass.     

介导感觉生理功能的瞬时受体电位通道蛋白(TRP)家族研究进展

瞬时受体电位通道蛋白(TRP)家族由一类特殊的阳离子通道蛋白组成,在神经细胞及其他非兴奋细胞中有重要作用,其中在介导多种感觉生理功能方面的作用尤其显著.TRP结构与功能的深入研究为阐明感觉生理功能的分子机制提供了重要线索.本文综述TRP家族在温度感受、机械刺激感受、光感受和化学信号感受等方面的研究进展.     

Abnormal movements in Rett syndrome are present before the regression period: a case study.

The suspicion of a diagnosis of Rett syndrome (RTT) is based on clinical criteria that are often not present in the first two stages of the disease, as many of its symptoms will appear at a later age. This sometimes postpones the genetic diagnosis and an early clinical intervention. We present the case of 19-months-old girl who came to the consultation because of an arrest of psychomotor development noticed 5 months earlier without change in sleep pattern, behavior, or social communication. In the observation of 1 hour videotape, she presented subtle stereotypic movements of the face and hands as well as repetitive dystonic posturing of her limbs. A genetic test confirmed the diagnosis of RTT, showing a truncating mutation in the MECP2 gene (R270X). This case confirms that stereotypic movement anomalies, albeit infrequent and subtle, are already present before the regression stage and while maintaining prehension and that, in addition, repetitive dystonic postures may occur. Recognition of these early movement disorders will improve clinicians' ability to perform an earlier diagnosis of RTT.     

自发性食管破裂的护理

目的 总结自发性食管破裂患者的护理措施，提高护理水平。方法 在保守及手术治疗的同时，严密观察病情变化，做好心理护理，加强呼吸道、消化道及各种管道的护理。结果 7例患者治愈出院。结论 精心的专业化护理在治疗自发性食管破裂中起重要作用。     

亚低温对大鼠短暂性脑缺血迟发性神经元死亡的影响

目的 观察亚低温（33℃）对大鼠短暂性脑缺血后神经元的保护作用。方法 32只DS大鼠分为假手术组、常温缺血组和即刻亚低温组，采用尼氏体亚甲蓝特殊染色观察存活神经元、原位细胞凋亡检测法（TUNEL染色）检测及电镜观察脑缺血后大鼠CA1区神经元凋亡情况。结果 与假手术组相比，常温缺血组海马CA1区存活的锥体细胞数目减少（P＜0．01）；与常温缺血组相比，亚低温缺血组海马CA1区存活的锥体细胞数目明显增多（P＜0．01）。亚低温缺血组大鼠海马CA1区TUNEL染色阳性细胞数目明显少于常温缺血组（P＜0．01）。结论 脑缺血后迟发性神经元死亡很可能通过凋亡途径，亚低温对缺血后神经元的保护作用与减少神经元凋亡有关。     

食管癌贲门癌术后胸胃排空障碍的临床分析

目的探讨食管癌贲门癌术后胸胃排空障碍的诱因、诊断和治疗。方法回顾性分析1990年1月-2003年12月我院施行1369例食管癌贲门癌切除术的病人临床资料。结果本组病例发生胸胃排空障碍17例,发生率1.24%,其中机械性胸胃排空障碍5例,功能性胸胃排空障碍12例,均发生于术后3～12d,再次手术治疗5例;治愈16例,死亡1例。结论上消化道造影、胃镜是鉴别机械性胃排空障碍和功能性胃排空障碍的重要方法,对食管癌术后机械性胃排空障碍应采取手术治疗,而功能性胃排空障碍采取保守治疗多能好转。     

国产与进口头孢他啶治疗小儿细菌性下呼吸道感染的成本-效果分析

目的对国产与进口头孢他啶治疗小儿细菌性下呼吸道感染进行成本—效果分析。方法对我院2008年10月-2009年10月间患小儿细菌性下呼吸道感染且单用头孢他啶治疗的住院患者136例进行回顾性分析,根据药品不同产地将其分为A、B两组,A组给与头孢他啶（国产）60mg/（kg·d）,分2次静脉滴注;B组给与头孢他啶（进口）60mg/（kg·d）,分2次静脉滴注,两组疗程均为7d,分别计算两组病人治疗后的细菌清除率、不良反应发生率,两组的治疗有效率和总成本,计算成本-效果比。结果疗程结束后A、B两组的药物治疗成本分别为：A组236.4元、B组982.5元,组间比较存在显著性差异（P〈0.05）;治疗有效率分别为：91.30%、94.03%;A、B两组的细菌清除率分别为：86.36%、90.70%;两组不良反应发生率分别为：5.80%、2.99%,组间比较差异均无显著性（P〈0.05）;两组成本-效果比分别为：2.59、10.45。结论国产头孢他啶的成本-效果比优于进口头孢他啶,为较佳的治疗方案。     

Post-operative esophageal leak treated with removable silicone-covered polyester stent

Self-expanding removable covered stents are increasingly being used for the treatment of benign esophageal diseases such as leaks or perforations and stenosis. They are easy to place and remove and good outcomes have been reported. We report a case of a postoperative esophageal leak successfully managed with a removable silicone-covered polyester stent.     

Is focal task‐specific dystonia limited to the hand and face?

Focal task-specific dystonia (FTSD) of the hand and face have been well described; however, FTSD of the leg is exceedingly rare. We describe and demonstrate by videotape 2 patients with FTSD affecting the leg, in both cases triggered specifically by walking down steps. Walking on a level surface, up steps, and down steps backward, and sideways were normal. An interoceptive sensory trick (imagining walking in a different modality) led to temporary improvement. Our patients appear to demonstrated that task-specificity in focal dystonia may not be limited to skilled, rehearsed actions and that FTSD may occur in an activity that is relatively autonomic.