Bisulli Francesca, Volpi Lilia, Meletti Stefano, Rubboli Guido, Franzoni Emilio, Moscano Milena, d'Orsi Giuseppe, Tassinari Carlo Alberto
Department of Neurological Sciences, University of Bologna, Bellaria Hospital, Bologna, Italy.
Epilepsia. 2002 Dec;43(12):1559-63. doi: 10.1046/j.1528-1157.2002.15302.x.
To investigate ictal muscular phenomena characterizing symptomatic infantile spasms (ISs) and their relation to ictal EEG.
Four children with severe encephalopathy, neurologic impairment, and refractory ISs related to different dysplastic lesions, underwent videopolygraphic recordings collecting surface electromyogram (EMG) activity from several cranial and limb muscles to evaluate the pattern of muscular recruitment, duration, and side-to-side asymmetry of ISs. Acquired data were stored for off-line analysis by a computerized polygraphic system.
Spasms were characterized by a complex pattern of muscular activation. A constant or rostrocaudal propagation pattern was lacking in all patients. Intervals between the onset of EMG activity in different muscles in each spasm were very long: </=100-200 ms. Two patients, with hemispheric cortical dysplasia and agenesis of the corpus callosum, had asymmetric and asynchronous spasms in which the EMG onset of the muscles contralateral to the affected hemisphere constantly anticipated that of the ipsilateral ones. Backaveraging of EEG activity disclosed a high-amplitude EEG complex on the same side as the dysplastic lesion, preceding or succeeding the contralateral muscle activity onset. In the other two patients with diffuse cortical dysplasia, no EEG transient was related to EMG activity onset.
Despite clinical similarity between spasms in the same patient, our data demonstrate the complexity and heterogeneity of these motor phenomena. Our findings cannot be explained simply in terms of cortical or reticular generators like other motor phenomena such as cortical myoclonus or startle reflex.
研究症状性婴儿痉挛（ISs）的发作期肌肉现象及其与发作期脑电图的关系。
4例患有严重脑病、神经功能障碍且与不同发育异常病变相关的难治性ISs患儿，进行了视频多导记录，收集来自多个头颅和肢体肌肉的表面肌电图（EMG）活动，以评估ISs的肌肉募集模式、持续时间和左右不对称性。采集的数据由计算机多导系统存储以供离线分析。
痉挛的特点是肌肉激活模式复杂。所有患者均缺乏恒定或头尾向传播模式。每次痉挛中不同肌肉EMG活动开始之间的间隔非常长：≤100 - 200毫秒。2例患有半球皮质发育异常和胼胝体发育不全的患者出现不对称和不同步的痉挛，其中患侧半球对侧肌肉的EMG起始总是早于同侧肌肉。EEG活动的反向平均显示，在发育异常病变同侧有一个高幅EEG复合体，先于或后于对侧肌肉活动开始。在另外2例弥漫性皮质发育异常的患者中，没有EEG瞬变与EMG活动开始相关。
尽管同一患者的痉挛在临床上有相似性，但我们的数据表明这些运动现象具有复杂性和异质性。我们的发现不能像其他运动现象如皮质肌阵挛或惊吓反射那样简单地用皮质或网状发生器来解释。
