Appearance of bitemporal periodic EEG activity in the last stage of Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu): A case report

• Published in: Clinical neurology and neurosurgery Vol. 204; p. 106602
• Main Authors: Yazawa, Shogo, Tsuruta, Kazuhito, Sugimoto, Akiko, Suzuki, Yukiko, Yagi, Kazuhiro, Matsuhashi, Masao, Yoshimura, Michiyoshi, Takashima, Hiroshi
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.05.2021; Elsevier Limited
• Subjects: Age; Aged; Atrophy; Bilateral independent periodic discharge (BIPD); Brain - physiopathology; Case reports; Creutzfeldt-Jakob disease; Dysarthria; Editing; EEG; Electroencephalography; Electroencephalography (EEG); Female; Gerstmann-Straussler-Scheinker Disease - physiopathology; Humans; Magnetic resonance imaging; Magnetoencephalography (MEG); Middle Aged; Neurology; Patients; Pneumonia; Prion disease; Seizures; Supervision; Temporal lobe; Temporal spread imaging (TSI); Traumatic brain injury; Writing; Prion disease; Magnetoencephalography (MEG); Electroencephalography (EEG); Bilateral independent periodic discharge (BIPD); Temporal spread imaging (TSI)
• ISSN: 0303-8467; 1872-6968
• DOI: 10.1016/j.clineuro.2021.106602

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. •A patient with Gerstmann-Sträussler-Scheinker syndrome showed temporal periodic sharp waves on EEG 8 years after onset.•The periodic temporal activities were not synchronous across the both hemispheres.•MEG showed activation of the mesial and temporal lobes around the sharp wave peaks bilaterally [1].