Ikeda Akio, Hirasawa Ken-ichi, Kinoshita Masako, Hitomi Takefumi, Matsumoto Riki, Mitsueda Takahiro, Taki Jun-ya, Inouch Morito, Mikuni Nobuhiro, Hori Tomokatsu, Fukuyama Hidenao, Hashimoto Nobuo, Shibasaki Hiroshi, Takahashi Ryosuke
Department of Neurology, Kyoto University School of Medicine, Shogoin, Sakyo-ku, Kyoto, Japan.
Epilepsia. 2009 Sep;50(9):2072-84. doi: 10.1111/j.1528-1167.2009.02097.x. Epub 2009 May 12.
Seizure manifesting motor arrest, that is, negative motor seizure (NMS), is a rare epileptic condition in which only inability to conduct voluntary movements or praxis is produced, although consciousness is preserved. The negative motor area (NMA) seems to be responsible, but its generator mechanism has not yet been clarified.
Three patients manifesting NMS were investigated. Two patients (ages 33 and 17) with intractable frontal lobe epilepsy had subdural grid implantation for epilepsy surgery, and one (age 77) had scalp electroencephalography (EEG) monitoring.
Ictal semiologies commonly observed, at least in the two patients, were found as follows; (1) indescribable or ill-localized aura, (2) repetitive involuntary vocalization, (3) inability to speak, (4) inability to move the extremities, and (5) subsequent evolution to positive motor seizures. Awareness and comprehension were preserved throughout the episode before generalized seizures. In two patients with epicortical EEG recording, ictal activity arose from the lateral NMA in one, and from the rostral supplementary motor area in the other. Cortical stimulation at NMA in one patient elicited symptoms identical to NMS. Another patient had scalp EEG and magnetic resonance imaging (MRI) abnormality, both suggesting the epileptogenic focus in the mesial frontal area.
We showed that (1) NMS was a rare condition in patients with seizure focus in the frontal lobe, and (2) that the NMA was responsible for the symptoms. The documented state in the present study may reflect ictal apraxia, but it requires further investigation.
以运动停止为表现的癫痫发作,即负性运动性癫痫发作(NMS),是一种罕见的癫痫病症,尽管意识保留,但仅表现为无法进行自主运动或运用。负性运动区(NMA)似乎与之相关,但其产生机制尚未阐明。
对3例表现为NMS的患者进行了研究。2例(年龄分别为33岁和17岁)难治性额叶癫痫患者接受了硬膜下网格电极植入术以进行癫痫手术,1例(77岁)接受了头皮脑电图(EEG)监测。
至少在2例患者中常见的发作期症状如下:(1)难以描述或定位不明确的先兆,(2)反复不自主发声,(3)无法说话,(4)肢体无法活动,以及(5)随后演变为正性运动性癫痫发作。在全身性发作前的整个发作过程中,意识和理解能力均得以保留。在2例进行皮质脑电图记录的患者中,1例的发作期活动起源于外侧NMA,另1例起源于嘴侧辅助运动区。对1例患者的NMA进行皮质刺激引发了与NMS相同的症状。另1例患者的头皮EEG和磁共振成像(MRI)异常,均提示癫痫病灶位于额叶内侧区域。
我们表明(1)NMS在额叶有癫痫病灶的患者中是一种罕见病症,(2)NMA是症状的起因。本研究中记录的状态可能反映了发作性失用症,但仍需进一步研究。