Wadi Lara, Khweileh Mays, Agashe Shruti, Southwell Derek, Parikh Prachi, Frauscher Birgit
Department of Neurology, Duke Comprehensive Epilepsy Center, Durham, North Carolina, USA.
Department of Neurosurgery, Duke School of Medicine, Durham, North Carolina, USA.
Epileptic Disord. 2024 Dec;26(6):868-874. doi: 10.1002/epd2.20285. Epub 2024 Sep 21.
We present two unique cases of sleep-related hypermotor epilepsy (SHE) originating from the occipital lobe. Patients with sleep-related seizures and drug-resistant occipital lobe epilepsy were identified from the ANPHY lab stereo-electroencephalography (SEEG) research database at the Duke Comprehensive Epilepsy Center. We identified two young females with frequent sleep-related focal seizures and occasional focal to bilateral tonic clonic seizures characterized by hypermotor movements. During wakefulness, the semiology also involved an elementary visual aura. They meet the 2016 diagnostic criteria for SHE, and SEEG monitoring with cortical stimulation mapping identified an epileptogenic zone (EZ) within the occipital lobe, with most seizures occurring out of NREM 2 sleep. Responsive neurostimulation devices were implanted, which indicated a trend for event detections in nocturnal periods. Extrafrontal SHE has characteristically been described in the temporal, insular-opercular, and parietal lobes. Here, we demonstrate using SEEG-confirmed EZ identification, that SHE can also originate in the occipital lobe. In patients with sleep-related seizures and hypermotor behavior, occipital lobe seizures thus should not be excluded from the differential diagnosis. Key in identifying this rare localization is non-frontal aura semiology and delay to motor symptoms, which may be supported by a visual field deficit and structural MRI abnormality.
我们报告了两例起源于枕叶的与睡眠相关的运动过多型癫痫(SHE)的独特病例。从杜克综合癫痫中心的ANPHY实验室立体定向脑电图(SEEG)研究数据库中识别出患有与睡眠相关癫痫发作和耐药性枕叶癫痫的患者。我们确定了两名年轻女性,她们频繁出现与睡眠相关的局灶性癫痫发作,偶尔出现从局灶性发作发展为双侧强直阵挛发作,其特征为运动过多。在清醒时,症状学还包括基本的视觉先兆。她们符合2016年SHE的诊断标准,SEEG监测结合皮质刺激图谱确定枕叶内存在癫痫ogenic区(EZ),大多数癫痫发作发生在非快速眼动睡眠2期之外。植入了反应性神经刺激装置,显示夜间有事件检测的趋势。额外SHE的特征性描述多见于颞叶、岛盖和顶叶。在此,我们通过SEEG确认的EZ识别证明,SHE也可起源于枕叶。因此,在患有与睡眠相关癫痫发作和运动过多行为的患者中,枕叶癫痫不应被排除在鉴别诊断之外。识别这种罕见定位的关键在于非额叶先兆症状学和运动症状出现延迟,这可能得到视野缺损和结构MRI异常的支持。