Houser M K, Soland V L, Bhatia K P, Quinn N P, Marsden C D
Department of Clinical Neurophysiology, National Hospital for Neurology and Neurosurgery, London, UK.
J Neurol. 1999 Feb;246(2):120-6. doi: 10.1007/s004150050318.
Paroxysmal kinesigenic choreoathetosis (PKC) is a neurological condition which results in abnormal involuntary movements that are precipitated by sudden movement. Because of its rarity, large case series of PKC have not been published. We studied 26 patients with PKC, which represents the largest series thus reported. We reviewed our cases with respect to attack characteristics, aetiology, family history, and treatment response. Our population consisted of 23 men and 3 women. Seven patients had a family history of paroxysmal dyskinesia. None of our patients had clear evidence of symptomatic PKC. Two-thirds of our patients had attacks lasting between 30-60 s, and over one-half experienced one to ten attacks per day. Attack distribution varied widely, and most experienced pure dystonia rather than choreodystonic movements. Most patients responded very well to anticonvulsant therapy. We also report the PET results from two of our patients and Bereitschaftspotential abnormalities recorded from two others.
发作性运动诱发性舞蹈手足徐动症（PKC）是一种神经系统疾病，会导致由突然运动引发的异常不自主运动。由于其罕见性，尚未发表大量关于PKC的病例系列报道。我们研究了26例PKC患者，这是迄今报道的最大病例系列。我们从发作特征、病因、家族史和治疗反应等方面对病例进行了回顾。我们的研究对象包括23名男性和3名女性。7例患者有发作性运动障碍的家族史。我们的患者均无明确的症状性PKC证据。三分之二的患者发作持续30至60秒，超过一半的患者每天发作1至10次。发作分布差异很大，大多数患者表现为单纯肌张力障碍而非舞蹈样动作。大多数患者对抗惊厥治疗反应良好。我们还报告了两名患者的PET结果以及另外两名患者记录到的运动前负电位异常。
