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以瘙痒为首发表现的伴抗 amphiphysin 抗体的副肿瘤性僵人综合征:1 例罕见病例

Paraneoplastic Stiff Person Syndrome With Anti-amphiphysin Antibodies Presenting With Pruritus as the Initial Manifestation: An Unusual Case.

作者信息

Raj Aiswarya, Alapatt Paul, Johny Paul, Vv Ashraf

机构信息

Department of Neurology, Aster Malabar Institute of Medical Sciences (MIMS), Kozhikode, IND.

出版信息

Cureus. 2023 Feb 21;15(2):e35249. doi: 10.7759/cureus.35249. eCollection 2023 Feb.

Abstract

Stiff person syndrome (SPS), also known as Stiff-man syndrome/Moersch-Woltman syndrome, is a rare disorder of the central nervous system, first described in 1956, characterized by rigidity and stimulus-triggered painful muscle spasms of predominantly axial and proximal limb muscles. There are many variants of SPS; these include the classical SPS, stiff leg syndrome, and paraneoplastic variant. The paraneoplastic variant of SPS is more common in patients with breast cancer with anti-amphiphysin antibodies, followed by colon carcinoma, lung carcinomas, thymoma, and Hodgkin's lymphoma. A possible autoimmune origin for the disease has been proposed, including antibodies against glutamic acid decarboxylase and amphiphysin. We thus describe a case of anti-amphiphysin antibody-positive SPS, which initially manifested with generalized pruritus. After extensive investigations and removing her underlying tumor, she reported complete recovery of her symptoms.

摘要

僵人综合征(SPS),也称为僵人综合征/莫尔施-沃尔特曼综合征,是一种罕见的中枢神经系统疾病,于1956年首次被描述,其特征是主要累及躯干和近端肢体肌肉的僵硬以及刺激引发的疼痛性肌肉痉挛。SPS有许多变体;这些包括经典型SPS、僵腿综合征和副肿瘤性变体。SPS的副肿瘤性变体在患有抗 amphiphysin 抗体的乳腺癌患者中更为常见,其次是结肠癌、肺癌、胸腺瘤和霍奇金淋巴瘤。该病可能存在自身免疫起源,包括针对谷氨酸脱羧酶和 amphiphysin 的抗体。我们在此描述一例抗 amphiphysin 抗体阳性的SPS病例,该病例最初表现为全身性瘙痒。经过广泛检查并切除其潜在肿瘤后,她报告症状完全恢复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cba3/10034866/a591aab75075/cureus-0015-00000035249-i01.jpg

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