Cockerell O C, Rothwell J, Thompson P D, Marsden C D, Shorvon S D
Epilepsy Research Group, National Hospital for Neurology and Neurosurgery, London, UK.
Brain. 1996 Apr;119 ( Pt 2):393-407. doi: 10.1093/brain/119.2.393.
Epilepsia partialis continua (EPC) is defined clinically as a syndrome of continuous focal jerking of a body part, usually localized to a distal limb, occurring over hours, days or even years. The anatomical and physiological origin of EPC has been the subject of much speculation. It has been argued that EPC is a form of focal cortical myoclonus, but subcortical mechanisms have also been proposed. We describe a series of 36 patients ascertained over a period of 1 year in the UK using the British Neurological Surveillance Unit. The commonest aetiologies identified were Rasmussen's syndrome (n = 7; 19%) and cerebrovascular disease (n = 5; 14%). Rasmussen's syndrome was the most common diagnosis in patients under 16 years. In seven patients the cause remained unknown. Eight patients (22%) had focal epileptiform scalp EEG abnormalities, and 56% had generalized background scalp EEG disturbances. Lesions on MRI or CT were found in 20 cases (56%), half of whom showed predominant cortical involvement. The muscle jerking resolved in four patients (with no treatment in one), with a partial response to treatment in seven (19%) patients. A cognitive or neurological decline had been noted retrospectively in 13 (36%) patients (and in all of the patients with Rasmussen's syndrome). We personally saw 16 patients who underwent detailed clinical and neurophysiological assessments. Only six of the patients had EEG and EMG evidence for a cortical origin of their jerks; five others had indirect evidence for a cortical origin, from EMG, magnetic stimulation, and other investigations. Two patients did not have myoclonus of cortical origin, but some other source (brainstem and basal ganglia). The origin in the remaining three patients was uncertain. The clinical appearance of the muscle jerks was similar in all patients despite the different origins. We propose that the definition of EPC is best restricted to "continuous muscle jerks of cortical origin'. Continuous muscle jerking that arises from other sites in the nervous system should be termed "myoclonia continua'.
持续性部分性癫痫（EPC）在临床上被定义为身体某一部位持续局灶性抽搐的综合征，通常局限于远端肢体，持续数小时、数天甚至数年。EPC的解剖学和生理学起源一直是诸多猜测的主题。有人认为EPC是局灶性皮质肌阵挛的一种形式，但也有人提出了皮质下机制。我们描述了在英国通过英国神经监测单位在1年时间内确诊的36例患者。确定的最常见病因是拉斯穆森综合征（n = 7；19%）和脑血管疾病（n = 5；14%）。拉斯穆森综合征是16岁以下患者最常见的诊断。7例患者病因不明。8例患者（22%）头皮脑电图有局灶性癫痫样异常，56%有广泛性背景头皮脑电图紊乱。20例（56%）患者的MRI或CT检查发现有病变，其中一半主要表现为皮质受累。4例患者的肌肉抽搐缓解（1例未接受治疗），7例（19%）患者治疗后有部分反应。回顾性观察发现13例（36%）患者有认知或神经功能衰退（拉斯穆森综合征患者全部如此）。我们亲自诊治了16例接受详细临床和神经生理学评估的患者。只有6例患者的脑电图和肌电图有证据表明其抽搐起源于皮质；另外5例从肌电图、磁刺激和其他检查中有间接证据表明起源于皮质。2例患者的肌阵挛并非起源于皮质，而是其他部位（脑干和基底节）。其余3例患者的起源尚不确定。尽管起源不同，但所有患者肌肉抽搐的临床表现相似。我们建议将EPC的定义最好限定为“起源于皮质的持续性肌肉抽搐”。起源于神经系统其他部位的持续性肌肉抽搐应称为“持续性肌阵挛”。
