Brigo Francesco, Vogrig Alberto, Bratti Arianna, Tavernelli Veronica, Nardone Raffaele, Trinka Eugen
Department of Neurosciences, Biomedicine and Movement Sciences. University of Verona, Italy, Department of Neurology, Franz Tappeiner Hospital, Merano, Italy.
Department of Neurosciences, Santa Maria della Misericordia University Hospital, Udine, Italy.
Epileptic Disord. 2018 Aug 1;20(4):301-312. doi: 10.1684/epd.2018.0983.
Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. We describe a 22-year-old woman with EPC manifesting with isolated toe movements, prevalent over the left side and initially misdiagnosed as psychogenic, clinically almost indistinguishable from those observed in "painful legs and moving toes syndrome". The continuous involuntary movements with EMG correlates of twitches lasting <100 ms, the sharp waves over fronto-central regions on EEG, and the marked asymmetry in somatosensory evoked potentials with higher cortical amplitude over the right side following peripheral stimulation over the left foot confirmed the epileptic nature of the symptoms, leading to the diagnosis of EPC. The toe movements were markedly reduced following steroid therapy, whereas the infusion of immunoglobulins caused aseptic meningitis. Despite an extensive diagnostic work-up (including a search for antibodies for paraneoplastic and autoimmune encephalitis), an ultimate aetiological diagnosis was not reached, although the dramatic response to corticosteroids strongly supported an underlying dysimmune pathophysiology. Diagnosing EPC can be challenging, especially if movements are confined to a very small body region or strongly resemble movements encountered in other conditions. EEG-EMG monitoring should be performed in patients with continuous involuntary muscular jerks in order not to overlook a diagnosis of EPC. [Published with video sequences on www.epilepticdisorders.com].
部分性癫痫持续状态(EPC)是局灶性癫痫持续状态的一种罕见形式。我们描述了一名22岁患有EPC的女性,其表现为孤立的足趾运动,左侧更为常见,最初被误诊为精神性的,临床上与“疼痛性腿部和活动足趾综合征”中观察到的症状几乎无法区分。持续的不自主运动伴有肌电图显示抽搐持续时间<100毫秒,脑电图额中央区有尖波,以及在左侧足部进行外周刺激后体感诱发电位存在明显不对称,右侧皮质振幅更高,这些均证实了症状的癫痫性质,从而诊断为EPC。类固醇治疗后足趾运动明显减少,而输注免疫球蛋白导致无菌性脑膜炎。尽管进行了广泛的诊断检查(包括寻找副肿瘤性和自身免疫性脑炎的抗体),但最终未得出病因诊断,尽管对皮质类固醇的显著反应强烈支持潜在的免疫失调病理生理学。诊断EPC可能具有挑战性,特别是当运动局限于非常小的身体区域或与其他情况中遇到的运动非常相似时。对于持续不自主肌肉抽搐的患者应进行脑电图 - 肌电图监测,以免漏诊EPC。[在www.epilepticdisorders.com上发布了视频序列]