The Use of Perampanel in the Treatment of Lance-Adams Syndrome

吡仑帕奈在兰斯-亚当斯综合征治疗中的应用

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Abstract

Lance Adams syndrome (LAS) is characterized by chronic action or intention myoclonus resulting from cerebral hypoxia. Perampanel, a non-competitive antagonist of aamino-3-hydroxy-5methyl-4 isooxazoleproprionic acid glutamate receptor, has demonstrated some efficacy in myoclonic epilepsy and other types of myoclonus. We report significant benefit in a patient with LAS treated with add on perampanel and provide a review of the relevant literature. In our case, a male patient in his 30s was found pulseless with unknown down time. The patient developed post anoxic myoclonus within 1 week from cardiac arrest. Patient continued to suffer from intractable myoclonus despite being treated with brivaracetam, valproic acid, and clonazepam. Perampanel was added to his medication regimen and up-titrated to 12 mg daily over 1-2 weeks. This resulted in significant improvement in frequency and severity of myoclonus for about 6 months. Growing evidence exists for perampanel as an adjunctive treatment in patients with post hypoxic myoclonus or LAS. A review of the available literature, comprised of case reports and case series, and suggests a potential role for perampanel in patients with LAS. Further study is warranted including controlled trials of perampanel use in post hypoxic myoclonus.

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