Department of Neurology, Junwakai Memorial Hospital, Miyazaki, Japan.
Department of Radiology, Junwakai Memorial Hospital, Miyazaki, Japan.
Clin Neurol Neurosurg. 2021 May;204:106602. doi: 10.1016/j.clineuro.2021.106602. Epub 2021 Mar 20.
EEG findings in advanced Gerstmann-Sträussler-Scheinker syndrome (GSS) are shown. A 56-year-old woman developed GSS symptoms and was diagnosed as having GSS with the P102L mutation at age 58. During the early stage, there were no significant EEG findings. Her clinical condition worsened and she developed akinetic mutism at age 62. The patient died of pneumonia at age 65. EEGs were recorded annually from age 61 to 65. Bilateral independent periodic discharges (BIPDs) in both temporal areas appeared at age 64. No clinical seizures were noticed. MEG showed the sharp waves of BIPDs originated independently in each temporal lobe. Other causes of BIPDs were absent.
现将高级格斯特曼-施特劳斯勒-许斯勒综合征（GSS）的脑电图发现展示如下。一位 56 岁女性出现 GSS 症状，并于 58 岁时被诊断为 P102L 突变型 GSS。在早期阶段，脑电图无明显发现。她的临床状况恶化，62 岁时出现无动性缄默症。患者于 65 岁时因肺炎去世。从 61 岁到 65 岁，每年记录一次脑电图。64 岁时，双侧颞区出现独立周期性放电（BIPD）。未注意到临床癫痫发作。MEG 显示 BIPD 的尖波在每个颞叶中独立起源。不存在其他导致 BIPD 的原因。
