Dyskinetic and dystonic cerebral palsy and birth

Two hundred and nineteen cases of the dyskinetic and dystonic forms of cerebral palsy which were seen in the course of three decades at a single clinic have been analysed. Fifty-seven patients had kernicterus. In the remaining 162, 71% of whom were born at term, birthweight was below the expected mean in two-thirds. There was no relationship between birth weight, or abnormal birth, or asphyxia, and the ultimate clinical severity of the children. We conclude that abnormal birth and asphyxia are not direct causes of the cerebral damage, but are expressions of a pre-existing condition resulting in susceptibility to the stress of birth, whether it is normal or abnormal.     

阵发性运动障碍性疾病的临床特征与分类探讨

目的：总结各类阵发性运动障碍性疾病的临床特征与治疗反应，并对其发病机理、分类加以探讨。方法：对12例阵发性运动障碍性疾病患儿进行了临床观察与随访，及录像脑电图（VEEG）、头颅CT和（或）MRI、铜蓝蛋白、角膜K－F环和肌电图（EMG）检查。结果：本组中6例为阵发性运动性舞蹈手足徐动（PKC），6例为阵发性肌张力不全舞蹈手足徐动（PDC），无阵发性运动诱发的肌张力不全（PED）。CT和（或）MRI、角膜K－F环及EMG无特殊，3例PKC患儿EEG有痫样放电。PKC对抗癫痫药反应良好，PDC无有效治疗。结论：阵发性运动障碍包含了一组少见的反复发作的运动异常性疾病，可以分为PKC、PDC、PED、阵发性睡眠诱发运动障碍（PHD）和其他。PKC由突发运动所诱发，发作频繁，持续时间短暂，抗癫痫药治疗效果显著，其发病机理可能与癫痫有关。PDC与运动无关，持续时间长短不一，尚无有效的治疗方法。     

发作性运动性舞蹈徐动症诊断与鉴别

目的　分析易误诊的发作性运动性舞蹈徐动症的临床特点。方法　分析发作性运动性舞蹈徐动症 2 1例 ,部分患者随诊。结果　在发作前均有明显的诱发因素 ,大多为突然运动、紧张、过度换气或惊吓。临床表现为发作性肢体的偏转、扭曲或者姿势的静止 ,多为一侧性 ,持续时间 1s～ 5 min,发作频率为每天 10余次或数月 1次不等 ,发作时意识清楚 ,发作间歇期完全正常。 6例有家族史 ,发病年龄 7～ 13岁。其电生理和神经影像学检查无明确异常。卡马西平类药物治疗有满意的疗效。结论　发作性运动性舞蹈徐动症是一种由运动诱发的、短暂的、发作性局部或全身不随意运动 ,属于离子通路疾病 ,用抗癫痫药疗效好     

发作性舞蹈徐动症16例报告

目的 研究罕见的发作性疾病－－发作性舞蹈徐动症的临床特点。方法 回顾性分析典型的发作性舞蹈徐动症１６例,阐述该病的特异性,并综合文献,简述其发病机制及遗传规律。结果 男性１３例,女性３例,发病年龄７－２３岁（平均１３岁）。５例有家族史（３１％）,考虑为常染色体显性遗传。其临床表现为发作性一侧或双侧肢体、躯干不自主运动伴构障碍、双眼上视。诱发因素为突然运动、紧张、疲劳、惊吓。持续时间５－２０ｓ,不超     

发作性运动诱发性舞蹈指痉症

目的 对发作性运动诱发性舞蹈指痉症的临床特点、电生理表现及发病机制等进行分析。方法 对2001-2003年收治的4例发作性运动诱发性舞蹈指痉症患的临床资料进行分析并复习近年献。结果 4例患均为青年，无家族遗传史，临床表现均为在运动开始时突然出现一侧或双侧肢体及面部的不自主运动，持续数秒钟后可自行缓解，发作期间无意识障碍，发作后无任何不适。4例患神经系统检查、脑电图以及头部CT和(或)MRI检查均无异常发现，诊断为特发性发作性运动诱发性舞蹈指痉症。经服用卡马西平等药物后发作均得到有效控制。结论 发作性运动诱发性舞蹈指痉症可呈常染色体显性遗传，亦可散发；可为特发性，也可继发于多发性硬化、特发性甲状旁腺功能减退症等其他疾病。发作性运动诱发性舞蹈指痉症的发病机制尚不清楚，其临床特征为运动诱发的一侧或双侧上下肢及面部的不自主运动，对抗癫痫药物敏感，预后良好。     

阵发性运动源性舞蹈手足徐动症

阵发性运动源性舞蹈手足徐动症是一种比较罕见的异常运动发作,其发作呈强直样、扭转痉挛样或舞蹈手足徐动样,可被突然运动或惊跳所诱发,不伴意识障碍,可有家族史或为散发。本文报道4例并结合文献复习对其临床特点、分类、可能之病理生理机制、有关鉴别诊断及治疗等进行了简要阐述。     

d-Amphetamine-induced “Floating limb” syndrome in young rhesus monkeys

Acuted-amphetamine administration to young rhesus monkeys (N=10) caused a motor syndrome of hypoactivity and chorea-like postures and motor movements which we have termed floating limb. Frequently after subcutaneous injections of 0.3 or 0.6 mg/kgd-amphetamine, an affected monkey raised one or both legs or arms and held the limb(s) motionless in the air. Affected limbs were usually returned to a normal position if they appeared to enter the animal's visual field. In other cases, the monkey assumed bizarre and contorted postures which were held for prolonged periods. Such postures were often accompanied by gentle repetitive brushing of the ears and facial hair with extremities of the affected limbs. Quantification of the frequency of these movements showed that they occurred regularly for 90–150 min afterd-amphetamine. Hydroxyamphetamine, a peripherally-acting amphetamine analog, did not induce floating limb, indicating that the behavior was probably mediated by central actions ofd-amphetamine. A similar disorder has been reported occasionally in other studies with monkeys and cats. It may be related to the chorea that is seen in humans after the use of amphetamine and other stimulants.d-Amphetamine treatment in young monkeys may provide a viable model of human choreoathetoid disorders induced by disease or drug use.     

手足徐动症继发颈椎病的手术治疗

【摘要】目的总结报告手足徐动症继发颈椎病的手术治疗结果,分析其发生特点与治疗原则。方法对32例手足徐动症继发颈椎病患者的症状、体征、手术方法及手术并发症等进行分析,并与同期收治的93例颈椎病患者进行比较,不同手术方式的效果之间也进行统计学比较。结果(1)手足徐动症继发颈椎病患者的平均年龄为37.94岁,颈椎病患者的平均年龄为52.48岁,两者比较差异有非常显著性意义(P<0.001)。(2)手术节段与颈椎病对照组比较差异有非常显著性意义(P<0.001)。(3)手术后症状、体征的改善结果与颈椎病对照组比较差异无显著性意义(P=0.068)。(4)29例行颈椎前路手术者中加用钛板固定的植骨愈合时间明显缩短(P=0.041)。结论手足徐动症继发颈椎病的特点为患者年轻且病变严重。颈椎前路减压、植骨融合加钛板内固定为最佳的手术方法。