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小细胞肺癌相关序贯性波动性副肿瘤性眼球震颤-舞蹈手足徐动症-肌阵挛综合征和 Lambert-Eaton 肌无力综合征。

Sequential fluctuating paraneoplastic ocular flutter-opsoclonus-myoclonus syndrome and Lambert-Eaton myasthenic syndrome in small-cell lung cancer.

机构信息

Department of Clinical Neurosciences, UCL Institute of Neurology, Royal Free Campus, London, UK.

出版信息

J Neurol Neurosurg Psychiatry. 2011 Mar;82(3):344-6. doi: 10.1136/jnnp.2009.172684. Epub 2010 Apr 14.

Abstract

Paraneoplastic cerebellar degeneration may occur in association with Lambert-Eaton myasthenic syndrome (LEMS), but to our knowledge, the co-occurrence of paraneoplastic opsoclonus-myoclonus syndrome and LEMS has not been previously reported. A 67-year-old woman presented with a complex partial seizure and evolving ocular flutter, opsoclonus, myoclonus and 'cerebellar' signs, all of which improved spontaneously within 6 weeks. Approximately 8 weeks after symptom onset, the patient became encephalopathic, she had a further complex partial seizure, and she became areflexic with potentiation of deep tendon reflexes. Radiological, bronchoscopic and histological investigations revealed small-cell lung cancer, and neurophysiological investigations confirmed a diagnosis of LEMS. High-titre anti-P/Q-type voltage-gated calcium-channel antibodies were identified in the serum, which increased as the signs of opsoclonus and myoclonus resolved. The encephalopathy and clinical features of LEMS responded dramatically to chemotherapy and radiotherapy. Spontaneous improvement of paraneoplastic opsoclonus-myoclonus syndrome may occur, and this syndrome may occur in association with LEMS. Antivoltage-gated calcium-channel antibodies are not implicated in the pathogenesis of paraneoplastic opsoclonus-myoclonus syndrome.

摘要

副肿瘤性小脑变性可与 Lambert-Eaton 肌无力综合征(LEMS)相关发生,但据我们所知,副肿瘤性眼阵挛-肌阵挛综合征和 LEMS 的同时发生尚未被报道过。一位 67 岁女性以复杂部分性癫痫发作和进行性眼球震颤、眼阵挛、肌阵挛和“小脑”征起病,所有这些症状在 6 周内均自发改善。大约在症状出现 8 周后,患者出现脑病,又发生了一次复杂部分性癫痫发作,且反射消失,深腱反射亢进。影像学、支气管镜和组织学检查显示小细胞肺癌,神经生理学检查证实 LEMS 的诊断。血清中发现了高滴度的抗 P/Q 型电压门控钙通道抗体,随着眼阵挛和肌阵挛的消退,其滴度增加。脑病和 LEMS 的临床特征对化疗和放疗有显著反应。副肿瘤性眼阵挛-肌阵挛综合征可能会自发改善,并且该综合征可能与 LEMS 相关。电压门控钙通道抗体与副肿瘤性眼阵挛-肌阵挛综合征的发病机制无关。

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