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首页> 外文期刊>Journal of neurology >Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome
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Motor neuron involvement in anti-Ma2-associated paraneoplastic neurological syndrome

机译：电机神经元参与抗MA2相关的肺状瓣膜神经综合症

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ObjectiveTo present clinical, radiological, and pathological features of a cohort of patients with motor neuron involvement in association with anti-Ma2 antibodies (Ma2-Ab).MethodsRetrospective case-series of patients with definite paraneoplastic neurological syndrome (PNS) and Ma2-Ab, and cases identified from a review of the literature.ResultsAmong 33 Ma2-Ab patients referred between 2002 and 2016, we retrospectively identified three patients (9.1%) with a motor neuron syndrome (MNS). Seven additional cases were retrieved among the 75 Ma2-patients reported in the literature (9.3%). A total of ten patients are, therefore, described herein. MNS was evident as combined upper and lower MNS in four patients, isolated upper MNS in two, and isolated lower MNS in one; three patients were diagnosed with myeloradiculopathy. The most common MNS signs/symptoms were: hyperreflexia (80%), proximal weakness (60%), proximal upper-limb fasciculations (50%), head drop (40%), and dysarthria/dysphagia (30%). Brain MRI abnormalities included bilateral pyramidal tract T2-weighted/FLAIR hyperintensities (three patients). Spine MRI found bilateral, symmetric, T2-weighted signal abnormalities in the anterior horn in two patients. CSF examination was abnormal in nine patients. Cancer was found in seven patients (four testicular, two lung, and one mesothelioma). Eight patients underwent first-line immunotherapy. Second-line immunotherapy was adopted in all our patients and in none of those identified in the literature. Motor improvement was observed in 33% of our patients, and 20% in the literature series.ConclusionsMotor neuron involvement could complicate Ma2-Ab-associated PNS in almost 10% of patients and must be carefully studied to adapt treatment. This disorder differs from amyotrophic lateral sclerosis.
机译：ObjectiveTo目前与抗MA2抗体（MA2-AB）相关联的运动神经元参与患者的临床，放射性和病理特征.Methodsrietrosepary-Series系列患者明确的平原神经综合症（PNS）和MA2-AB，和审查文献审查中确定的案件。关于2002年至2016年的33名MA2-AB患者，我们回顾性地确定了三名患者（9.1％），用电机神经元综合征（MNS）。在文献中报告的75名MA2患者中检出了七种额外案例（9.3％）。因此，在此描述了10名患者。在四名患者中，MNS在患者中占据上下MNS，孤立的上部MNS在两个中，较低的MNS在一个患者中。三名患者被诊断为骨髓癌病变。最常见的MNS迹象/症状是：超折叠（80％），近端弱点（60％），近端肢体坐着（50％），头部下降（40％）和休闲症/吞咽困难（30％）。脑MRI异常包括双侧金字塔型T2加权/发病性超萎缩（三名患者）。脊柱MRI发现两名患者前角的双侧，对称，T2加权信号异常。九名患者的CSF检查异常。在7名患者中发现癌症（四个睾丸，两个肺和一个间皮瘤）。八名患者接受一线免疫疗法。所有患者采用二线免疫疗法，并非在文献中确定的患者。在33％的患者中观察到电机改善，文献系列中的20％。结合MOTOR神经元受累可能在近10％的患者中使MA2-AB相关的PNS复杂化，必须仔细研究以适应治疗。这种疾病与肌营养的外侧硬化症不同。

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