继发于抗胡副肿瘤性神经综合征的厣肌强直-无张力状态（OMASE）

IF 1.8 Q3 CLINICAL NEUROLOGY Epilepsy and Behavior Reports Pub Date : 2024-01-01 DOI:10.1016/j.ebr.2024.100703

César Romero, Alonso Quijada, Gabriel Abudinén, Catherine Céspedes, Ledda Aguilera

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引用次数: 0

摘要

局灶性肌阵挛-失神状态癫痫（OMASE）是一种罕见的局灶性运动性癫痫，由多种病因引起。其特征是波动性构音障碍和癫痫性肌阵挛累及双侧舌咽肌。本病例是一名 52 岁的女性患者，她的面部和球部肌肉逐渐出现进行性瘫痪和肌阵挛；其他检查显示她患有右乳腺导管腺癌，血清中抗 Hu 和抗 GAD65 抗体呈阳性。大剂量类固醇脉冲、抗癫痫治疗和利妥昔单抗部分控制了肌阵挛；肿瘤切除术改善了吞咽困难和构音障碍。

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Opercular myoclonic-anarthric status (OMASE) secondary to anti-Hu paraneoplastic neurological syndrome

Focal Opercular Myoclonic – Anarthric Status Epilepticus (OMASE) is a rare form of focal motor status epilepticus caused by several etiologies. It is characterized by fluctuating dysarthria and epileptic myoclonus involving the bilateral glossopharyngeal musculature. We present the case of a 52-year-old woman who experienced gradual and progressive paralysis and myoclonus of facial and bulbar muscles; additional tests revealed the presence of right breast ductal adenocarcinoma and positive serum anti-Hu and anti-GAD65 antibodies. High doses of steroid pulses, anti-seizure therapy, and rituximab partially controlled myoclonus; the tumor resection improved dysphagia and dysarthria.

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