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与实体癌相关的副肿瘤性亚急性下运动神经元综合征

Paraneoplastic subacute lower motor neuron syndrome associated with solid cancer.

作者信息

Verschueren Annie, Gallard Julien, Boucraut José, Honnorat Jerome, Pouget Jean, Attarian Shahram

机构信息

Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France.

Reference Center for Neuromuscular Diseases and ALS, Hôpital de la Timone, Marseille, France.

出版信息

J Neurol Sci. 2015 Nov 15;358(1-2):413-6. doi: 10.1016/j.jns.2015.08.014. Epub 2015 Aug 12.

Abstract

We retrospectively analyzed three patients with pure motor neuronopathy followed for more than four years in our center. The patients presented a rapidly progressive lower motor neuron syndrome (LMNS) over the course of a few weeks leading to a severe functional impairment. The neurological symptoms preceded the diagnosis of a breast adenocarcinoma and a thymoma in the first two patients, one of them with anti-CV2/CRMP5 antibodies. Cancer was not detected in the third patient who had circulating anti-Hu antibodies. A final diagnosis of paraneoplastic syndrome was made after investigations for alternative causes of lower motor neuron syndrome. Early diagnosis, combined treatment of the underlying cancer, and immunomodulatory treatment led to neurological improvement of the disease in two out of the three cases in which the cancer was diagnosed. Cases of subacute LMNS with rapid progression may occur as an expression of a paraneoplastic neurological syndrome. Identification of these syndromes is important, as the treatment of underlying malignancy along with immunomodulatory treatment may result in a favorable long-term outcome of these potentially fatal diseases.

摘要

我们回顾性分析了在本中心随访超过四年的三名纯运动神经元病患者。这些患者在数周内呈现出快速进展的下运动神经元综合征(LMNS),导致严重的功能障碍。在前两名患者中,神经症状先于乳腺腺癌和胸腺瘤的诊断出现,其中一名患者存在抗CV2/CRMP5抗体。在第三名有循环抗Hu抗体的患者中未检测到癌症。在对下运动神经元综合征的其他病因进行调查后,最终诊断为副肿瘤综合征。早期诊断、对潜在癌症的联合治疗以及免疫调节治疗,使得在确诊癌症的三例患者中有两例病情得到神经功能改善。快速进展的亚急性LMNS病例可能是副肿瘤性神经综合征的一种表现。识别这些综合征很重要,因为对潜在恶性肿瘤的治疗以及免疫调节治疗可能会使这些潜在致命疾病获得良好的长期预后。

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