ランス−アダムス症候群(Lans-Adams syndrome)は、心肺停止などが原因で低酸素脳症を被ったのちに動作時ミオクローヌスなどをきたす病態です。そのほかに失調症、認知機能低下、てんかんなどを伴うこともあります。治療に苦慮する症例が多いのも特徴です。今回、当科の旭 雄士先生は、ランス−アダムス症候群の症例に対して両側淡蒼球に対する深部脳刺激療法にて著効を示した症例をJ Neurol誌に報告させていただきました。
Asahi T, Kashiwazaki D, Dougu N, Oyama G, Takashima S, Tanaka K, Kuroda S.
Alleviation of myoclonus after bilateral pallidal deep brain stimulation for Lance-Adams syndrome.
J Neurol. 2015 Jun;262(6):1581-3
http://www.ncbi.nlm.nih.gov/pubmed/25929661
A Voyage to Depth of Neuroscience Vol. 22
Alleviation of myoclonus after bilateral pallidal deep brain stimulation for Lance-Adams syndrome.
Lance–Adams syndrome (LAS), which can occur after cardiorespiratory arrest (CPA), is characterized by rest-, action-, and stimulus-sensitive myoclonus with or without other neurological signs such as ataxia, cognitive impair- ment, epilepsy, and pyramidal signs. In 1963, Lance and Adams first described four patients with post- hypoxic generalized myoclonic jerks [6]. Although case reports have been described sporadically, effective thera- pies have not been established. Deep brain stimulation (DBS) is widely used to treat movement disorders such as Parkinson’s disease, dystonia, and myoclonic diseases. Although functional neurosurgery, including intrathecal baclofen therapy (ITB) and DBS, has been performed to treat LAS, evidence for the success of these treatments is scarce. Here, we report a case of LAS treated successfully with bilateral globus pallidus interna (GPi) DBS.
