Multimodal Assessment of the Origin of Myoclonus in Lance-Adams Syndrome

作者：Geoffroy Vellieux, Emmanuelle Apartis, Paul Baudin, Miguel A. Quiñones, Diane Villemonte de la Clergerie, Aurélie Kas, Vincent Navarro, for the Lance-Adams Coinvestigators, Laurent Bailly, Louis Cousyn, Ana Cristina Gales, Bastien Herlin, Nicolas Mezouar, Yulia Worbe · 发表于：Neurology · 年份：2024 · DOI：10.1212/wnl.0000000000209994 · 被引用次数：9 · 研究领域：Neurological disorders and treatments、Glycogen Storage Diseases and Myoclonus、Genetic Neurodegenerative Diseases

Background and Objectives Lance-Adams syndrome (LAS), or chronic posthypoxic myoclonus, is a long-term disabling neurologic disorder occurring in survivors of anoxia. The cortical or subcortical origin of this myoclonus is unclear. We aimed to identify the neuroanatomical origin of myoclonus in LAS. Methods We conducted a cross-sectional study and investigated patients diagnosed with LAS from the Department of Neurology of Pitié-Salpêtrière Hospital, using multimodal neurologic explorations: EEG with quantitative analyses, polygraphic EMG recording of myoclonus, coupled EEG-EMG analyses with jerk-locked back averaging, and 18 fluorodeoxyglucose PET/CT imaging. Results All 18 patients had action multifocal or generalized myoclonus. Eleven patients also presented seizures, mainly generalized tonic-clonic seizures. For 8 patients, myoclonus decreased after seizures for a variable duration, from 1 day to 2 weeks. Epileptiform discharges were identified over the central median region (n = 14), with a maximal amplitude on the Cz (65 ± 20 µV, n = 12) and Fz (107 µV, n = 1) electrodes, and a significantly increased frequency during non–rapid eye movement sleep stages 1 (12 ± 8.5 events/minute, p = 0.004, n = 9) and 2 (11 ± 8.8 events/minute, p = 0.016, n = 7) compared with wake (5.5 ± 5.4 events/minute). The duration of the cortical and muscular events was significantly and positively correlated (ρ = 0.58, p < 0.001, n = 9). Action myoclonic jerks with a duration of <50 ms were confi...