Clinical and physiological features of epilepsia partialis continua - Cases ascertained in the UK
Clinical and physiological features of epilepsia partialis continua - Cases ascertained in the UK
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DOI：
10.1093/brain/119.2.393
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发表时间：
1996-04-01
期刊：
影响因子：
14.5
通讯作者：
Shorvon, SD
中科院分区：
文献类型：
--
作者：
Cockerell, OC;Rothwell, J;Shorvon, SD
持续性部分性癫痫（Epilepsia partialis continua，EPC）是一种持续数小时、数天甚至数年的肢体局部抽搐综合征。EPC的解剖学和生理学起源一直是许多推测的主题。有人认为，EPC是一种形式的局灶性皮质肌阵挛，但也提出了皮质下机制。我们描述了一系列的36例患者确定在一个为期一年的英国使用英国神经监测单位。确定的最常见病因为Rasmussen综合征（n = 7; 19%）和脑血管疾病（n = 5; 14%）。Rasmussen综合征是16岁以下患者中最常见的诊断。7例患者的原因仍然不明。8例患者（22%）有局灶性癫痫样头皮EEG异常，56%有全身背景头皮EEG紊乱。MRI或CT检查发现病变20例（56%），其中半数以皮质受累为主。4名患者的肌肉抽搐消退（1名患者未接受治疗），7名（19%）患者对治疗有部分反应。在13例（36%）患者（以及所有Rasmussen综合征患者）中回顾性观察到认知或神经功能下降。我们亲自观察了16名患者，他们接受了详细的临床和神经生理学评估。只有6名患者的脑电图和肌电图证据表明，他们的抽搐起源于皮层;其他人则有间接证据表明，皮层起源于肌电图，磁刺激和其他调查。两名患者没有皮质起源的肌阵挛，但一些其他来源（脑干和基底节）。其余3例患者的病因尚不确定。尽管起源不同，但所有患者的肌肉痉挛的临床表现相似。我们建议，EPC的定义最好是限制在“连续肌肉抽搐的皮质起源”。来自神经系统其他部位的持续性肌肉抽搐应称为“连续性肌阵挛”。
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 I 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. Irt seven patients the cause remained unknown. Eight patients (22%) had focal epileptiform scalp EEC 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; Jive 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'.