Lance-Adams Syndrome Treated by Perampanel in the Acute Term

Lance-Adams syndrome (LAS) is chronic post-hypoxic myoclonus after a hypoxic encephalopathy. Recently, the report on LAS in the chronic term treated by perampanel (PER) is increasing. However, PER's efficacy in the "acute term" has not been reported. Here, we report an LAS patient who markedly improved when PER was added to his existing treatment regime in the acute term. The 65-year-old patient presented with a return of spontaneous circulation after cardiopulmonary arrest. He developed myoclonus on the admission day, and it led to tonic-clonic convulsion. We started levetiracetam 3000 mg/day, lacosamide 400 mg/day, general anesthesia using midazolam 180 mg/day, dexmedetomidine 1000 μg/day, and fentanyl 1.2 mg/day. We could stop the convulsions after 18 h from the onset. We tried to reduce sedatives, but his convulsion recurred. We added PER 2 mg/day for three days, PER 4 mg/day for next four days, then used PER 8 mg/day and we could gradually reduce the sedatives. Single-photon emission computed tomography on day 40 showed cerebral blood flow (CBF) increase at the bilateral anterior lobes of the cerebellum, medial temporal lobes, and supplementary motor and premotor areas, while CBF decrease at the brain surface of the frontal, parietal, and temporal lobes. The myoclonus disappeared since day 12, and he was transferred to another rehabilitation hospital on day 56. The optimal treatment strategy has not been established for LAS, but our case suggested that PER could be one of the choices to treat LAS in the acute term.