Blakeley Jaishri, Jankovic Joseph
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA.
Mov Disord. 2002 Jul;17(4):726-34. doi: 10.1002/mds.10178.
Paroxysmal dyskinesias (PxDs) are involuntary, episodic movements that include paroxysmal kinesigenic (PKD), paroxysmal nonkinesigenic (PNKD), and paroxysmal hypnogenic (PHD) varieties. Although most PxDs are primary (idiopathic or genetic), we found 17 of our 76 patients with PxD (22%) to have an identifiable cause for their PxD (10 men; mean age, 41.4 years). Causes included peripheral trauma (in three patients), vascular lesions (in four), central trauma (in four), kernicterus (in two), multiple sclerosis (in one), cytomegalovirus encephalitis (in one), meningovascular syphilis (in one), and migraine (in one). The latency from insult to symptom onset ranged from days (trauma) to 18 years (kernicterus), with a mean of 3 years. Nine patients had PNKD, two had PKD, five had mixed PKD/PNKD, and one had PHD. Hemidystonia was the most common expression of the paroxysmal movement disorder, present in 11 patients. Both of the patients with PKD had symptom durations of <5 minutes. Symptom duration ranged from 10 seconds to 15 days for PNKD and from 5 minutes to 45 minutes for mixed PKD/PNKD. There were no uniformly effective therapies, but anticonvulsant drugs, clonazepam, and botulinum toxin injections were the most beneficial. Awareness of the variable phenomenology and the spectrum of causes associated with secondary PxD will allow for more timely diagnosis and early intervention.
发作性运动障碍(PxDs)是一种非自主性、发作性的运动,包括发作性运动诱发性(PKD)、发作性非运动诱发性(PNKD)和发作性睡眠相关性(PHD)等类型。尽管大多数PxDs是原发性的(特发性或遗传性),但我们发现76例PxD患者中有17例(22%)的PxD有明确病因(10例男性;平均年龄41.4岁)。病因包括外周创伤(3例)、血管病变(4例)、中枢创伤(4例)、核黄疸(2例)、多发性硬化(1例)、巨细胞病毒性脑炎(1例)、脑膜血管梅毒(1例)和偏头痛(1例)。从损伤到症状发作的潜伏期从数天(创伤)到18年(核黄疸)不等,平均为3年。9例患者为PNKD,2例为PKD,5例为PKD/PNKD混合型,1例为PHD。偏侧肌张力障碍是发作性运动障碍最常见的表现形式,11例患者出现该症状。2例PKD患者的症状持续时间均<5分钟。PNKD的症状持续时间为10秒至15天,PKD/PNKD混合型为5分钟至45分钟。目前尚无统一有效的治疗方法,但抗惊厥药物、氯硝西泮和肉毒毒素注射最为有效。认识到继发性PxD的可变临床表现和相关病因谱将有助于更及时的诊断和早期干预。