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非癌性患者伴不明原因神经系统受累时存在副肿瘤抗体。

Presence of paraneoplastic antibodies in non-carcinomatous patients with neurological involvements of unknown cause.

机构信息

Department of Neurology, Toho University Omori Medical Center, Tokyo, Japan.

出版信息

J Neurol Sci. 2013 Dec 15;335(1-2):197-200. doi: 10.1016/j.jns.2013.09.031. Epub 2013 Sep 29.

DOI:10.1016/j.jns.2013.09.031
PMID:24094858
Abstract

BACKGROUND

Paraneoplastic antibodies (PAs) play a crucial role in the diagnostic approach of paraneoplastic neurological syndrome (PNS). We clarified the frequency and the clinical profile of PA-positive non-carcinomatous patients with neurological involvements of unknown cause.

METHODS

PAs were analyzed in sera of 222 consecutive non-carcinomatous patients (122 men and 100 women) defined as acute or subacute onset of unknown-causative symptoms involving the neuromuscular junction, the central and/or the peripheral nervous system between 2006 and 2009. PAs contained antineuronal nuclear autoantibody type 1, 2, 3, Purkinje cell cytoplasmic autoantibody type 1, 2, anti-Tr, amphiphysin, CRMP-5, P/Q-type, N-type voltage-gated calcium channels (VGCC), voltage-gated potassium channel complex (VGKCC) and neuronal acetylcholine receptor (nAChR) antibodies. PA-seropositive patients received detailed examination of carcinoma in the whole body for the following 2 years.

RESULTS

Nine patients were PA-positive. VGKCC antibodies were found in four patients, P/Q-type VGCC antibodies in two, N-type VGCC antibodies in two and nAChR antibodies in one. Neurological features revealed limbic encephalitis in four patients, sensorimotor neuropathy in three and Lambert-Eaton myasthenic syndrome in two. One year later, 2 patients developed myelodysplastic syndrome and lung adenocarcinoma (one patient each).

CONCLUSION

We conclude that PA-seropositive frequency is 4.1% in non-carcinomatous neurological patients at examination. VGKCC, P/Q-type and N-type VGCC, and nAChR antibodies have benefits for screening non-carcinomatous PNS patients with acute or subacute neurological deficits of unknown cause.

摘要

背景

副肿瘤抗体(PAs)在副肿瘤神经系统综合征(PNS)的诊断方法中起着至关重要的作用。我们阐明了具有神经受累且病因不明的非癌性患者中 PA 阳性的频率和临床特征。

方法

在 2006 年至 2009 年间,我们分析了 222 例连续的非癌性患者(122 名男性和 100 名女性)的血清中的 PAs,这些患者具有神经肌肉接头、中枢和/或周围神经系统的急性或亚急性发作,病因不明。PAs 包括抗神经元核自身抗体 1、2、3、浦肯野细胞细胞质自身抗体 1、2、抗 Tr、抗 Amphiphysin、CRMP-5、P/Q 型、N 型电压门控钙通道(VGCC)、电压门控钾通道复合物(VGKCC)和神经元乙酰胆碱受体(nAChR)抗体。PA 阳性患者在接下来的 2 年内接受了全身癌肿的详细检查。

结果

9 例患者为 PA 阳性。4 例患者存在 VGKCC 抗体,2 例患者存在 P/Q 型 VGCC 抗体,2 例患者存在 N 型 VGCC 抗体,1 例患者存在 nAChR 抗体。神经学特征显示 4 例患者为边缘性脑炎,3 例患者为感觉运动性神经病,2 例患者为 Lambert-Eaton 肌无力综合征。1 年后,2 例患者分别发展为骨髓增生异常综合征和肺腺癌。

结论

我们的结论是,在检查时,非癌性神经疾病患者中 PA 阳性率为 4.1%。VGKCC、P/Q 型和 N 型 VGCC 以及 nAChR 抗体有助于筛选具有急性或亚急性神经功能缺损且病因不明的非癌性 PNS 患者。

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