Bakırcıoğlu-Duman Ezgi, Acar Zeynep, Benbir Gülçin, Yüceer Hande, Acar Hürtan, Baştan Birgül, Petek-Balcı Belgin, Karadeniz Derya, Çokar Özlem, Tüzün Erdem
Clinic of Neurology, Haseki Training and Research Hospital, Istanbul, Turkey.
Department of Neurology, Cerrahpaşa Faculty of Medicine, Istanbul University-Cerrahpaşa, Istanbul, Turkey.
Case Rep Neurol. 2019 Feb 28;11(1):80-86. doi: 10.1159/000497817. eCollection 2019 Jan-Apr.
Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history of constipation, hyperhidrosis, and insomnia. Neurologic examination revealed muscle twitching and needle electromyography showed myokymic discharges in all limbs. No clinical or electrophysiological features of neuromyotonia were present. Although the patient denied any cognitive symptoms, neuropsychological assessment revealed executive dysfunction, while other cognitive domains were preserved. Cranial and spinal MRIs were unrevealing and tumor investigation proved negative. Polysomnography examination revealed total insomnia, which was partially reversed upon immune-modulatory therapy. Investigation of a broad panel of antibodies revealed serum leucine-rich glioma inactivated protein 1 and contactin-associated protein 2 antibodies. The features of this case indicate that the presentation of PNH syndromes may show significant variability and that MoS patients may not necessarily exhibit full-scale PNH and encephalopathy symptoms.
莫旺综合征（MoS）的典型特征为神经肌强直、睡眠功能障碍、自主神经功能障碍和认知功能障碍。然而，也有文献报道过具有轻度周围神经兴奋性增高（PNH）或脑病特征的MoS患者。一名46岁女性，有2个月的便秘、多汗和失眠病史。神经系统检查发现肌肉抽搐，针极肌电图显示四肢均有肌束震颤放电。未发现神经肌强直的临床或电生理特征。尽管患者否认有任何认知症状，但神经心理学评估显示存在执行功能障碍，而其他认知领域未受影响。头颅和脊髓磁共振成像未发现异常，肿瘤检查结果为阴性。多导睡眠图检查显示完全失眠，免疫调节治疗后部分症状得到缓解。对一系列抗体的检测发现血清富含亮氨酸的胶质瘤失活蛋白1和接触蛋白相关蛋白2抗体。该病例的特征表明，PNH综合征的表现可能存在显著差异，且MoS患者不一定会出现全面的PNH和脑病症状。
