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帕金森病和自主神经功能障碍伴抗 CV2/CRMP5 相关副肿瘤性神经综合征,类似多系统萎缩:病例报告。

Parkinsonism and dysautonomia with anti-CV2/CRMP5 associated paraneoplastic neurological syndromes mimicking multiple system atrophy: a case report.

机构信息

Department of Neurology, Jilin University First Hospital, Xinmin Street 1, Changchun, Jilin, China.

出版信息

BMC Neurol. 2021 Oct 26;21(1):408. doi: 10.1186/s12883-021-02448-6.

Abstract

BACKGROUND

Paraneoplastic neurological syndromes (PNSs) are broad-spectrum disorders that can affect any part of the nervous system varying in core symptoms. Onconeural antibodies, including Hu, Yo, Ri, anti-CV2, amphiphysin, Ma2, and Tr are well-characterized and commonly used for the diagnosis of definite PNS. Generally, anti-CV2 antibodies have usually been associated with cerebellar ataxia, chorea, peripheral and autonomic neuropathies, myelopathy, optic neuritis, and retinitis. However, Parkinsonism has not been reported as the core symptom in patients with anti-CV2 antibodies.

CASE PRESENTATION

We report a patient with anti-CV2 antibody manifested as Parkinsonism and autonomic dysfunction, which may lead to the diagnosis of multiple system atrophy with predominant Parkinsonism (MSA-P). A lumbar puncture examination was undergone to find a positive anti-CV2 antibody in cerebrospinal fluid. PET-CT showed no tumor. Immunotherapy was adopted and the symptoms were relieved for 5 months. However, with no evidence of tumor, he died after 8 months.

CONCLUSIONS

Our findings indicate that PNS with anti-CV2 antibody can be shown as MSA-P mimic. Considering that MSA is a neurodegenerative disease with a poor prognosis, screening for other treatable or controllable factors like PNS presented in this case is necessary when encountering a rapid progressive MSA-mimic patient.

摘要

背景

副肿瘤神经系统综合征(PNS)是一种广泛的疾病谱,可影响神经系统的任何部位,核心症状各不相同。抗神经元抗体,包括 Hu、Yo、Ri、抗 CV2、 amphiphysin、Ma2 和 Tr 等,已经得到了很好的描述和广泛应用,可用于明确 PNS 的诊断。通常,抗 CV2 抗体与小脑共济失调、舞蹈病、周围和自主神经病、脊髓病、视神经炎和视网膜炎有关。然而,帕金森病并未被报道为抗 CV2 抗体患者的核心症状。

病例介绍

我们报告了一例抗 CV2 抗体表现为帕金森病和自主神经功能障碍的患者,可能导致以帕金森病为主的多系统萎缩(MSA-P)的诊断。进行了腰椎穿刺检查,发现脑脊液中存在阳性抗 CV2 抗体。PET-CT 未发现肿瘤。采用免疫疗法,症状缓解了 5 个月。然而,由于没有肿瘤的证据,他在 8 个月后死亡。

结论

我们的发现表明,抗 CV2 抗体的 PNS 可表现为 MSA-P 样表现。鉴于 MSA 是一种预后不良的神经退行性疾病,当遇到快速进展的 MSA 样患者时,有必要筛查其他可治疗或可控制的因素,如本例中出现的 PNS。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/08f5/8547084/3859c2db1145/12883_2021_2448_Fig1_HTML.jpg

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