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病例系列:三例莫旺综合征作为一种罕见的自身免疫性疾病伴抗 Caspr2 抗体。

A case series: Three cases of Morvan's syndrome as a rare autoimmune disorder with anti-Caspr2 antibody.

机构信息

Clinical Department of Traditional Chinese Medicine, Hubei University of Chinese Medicine, Wuhan, China.

Department of Traditional Chinese Medicine, Renmin Hospital of Wuhan University, Wuhan, China.

出版信息

Medicine (Baltimore). 2024 Oct 18;103(42):e40159. doi: 10.1097/MD.0000000000040159.

DOI:10.1097/MD.0000000000040159
PMID:39432629
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11495757/
Abstract

RATIONALE

Morvan syndrome (MoS) is an uncommon male-dominant autoimmune disorder marked by peripherally innervated hyperexcitability, autonomic disturbances, and encephalopathic encephalopathy, frequently with mass complaints manifesting as neuromyotonia (involuntary jerking, twitching, and stiffening of muscles), myotonia, neuropathic pain, hyperhidrosis, severe constipation, and severe sleep disturbances accompanied by dream reenactments, agrypnia agitation, and delusions, associated with autoantibodies to voltage-gated and potassium channel complexes such as anti-contactin-associated protein-like 2 (Caspr2) antibody. All this misery can be very disabling and even life-threatening. Reported cases show an unforeseeable outcome, with fatalities occurring even in those who initially responded. It has been reported that patients have reacted to immunologic therapies-corticosteroids, intravenous immunoglobulins, plasma exchanges, azathioprine, cyclophosphamide, rituximab, or carbamazepine, gabapentin, and clonazepam. However, no long-term effective cure has yet been found for this condition. Clinicians and researchers increasingly emphasize alternative and complementary medicine, with a growing trend toward traditional Chinese medicine (TCM).

PATIENT CONCERNS

Following glucocorticoid therapy, all 3 patients experienced a recurrence of the disease. Patients 1 and 2 observed symptomatic relief after intravenous immunoglobulin administration; however, upon discontinuation of the treatment, their conditions relapsed and worsened compared with the previous state.

DIAGNOSES

The 3 patients were definitively diagnosed with serum Caspr2-positive MoS, accompanied by a constellation of neurological manifestations.

INTERVENTIONS

The 3 patients were treated under the guidance of TCM theory. According to the principles of TCM, the patients were characterized by the deficiency of Yin, so the prescriptions were as follows: Shaoyao-Gancao decoction combined with Sanjia-Fumai decoction.

OUTCOMES

After the application of TCM, there was a reversal of neuropsychiatric manifestations such as unintentional rippling, jerking, muscle stiffness, myokymia, hyperhidrosis, and extreme constipation. Patients' quality of life improved significantly; to date, they have achieved Karnofsky Performance Status scores of 100, and the anti-Caspr2 antibody result in case 2 dropped from 1:32 to normal.

LESSONS

We first report the effective treatment of the MoS case series with TCM as complementary and alternative medicine.

摘要

背景

莫旺综合征(MoS)是一种罕见的男性主导的自身免疫性疾病,其特征为周围神经支配的过度兴奋、自主神经紊乱和脑病脑病,常伴有肿块表现为肌强直(不自主的抽搐、抽搐和肌肉僵硬)、肌强直、神经病性疼痛、多汗症、严重便秘和严重睡眠障碍,伴有梦境再现、睡眠不安和妄想,与电压门控和钾通道复合物的自身抗体有关,如抗接触蛋白相关蛋白样 2(Caspr2)抗体。所有这些痛苦都可能非常致残,甚至危及生命。报告的病例显示预后不可预测,即使在最初有反应的患者中也有死亡病例。据报道,患者对免疫疗法有反应-皮质类固醇、静脉注射免疫球蛋白、血浆置换、硫唑嘌呤、环磷酰胺、利妥昔单抗或卡马西平、加巴喷丁和氯硝西泮。然而,目前还没有找到治疗这种疾病的长期有效方法。临床医生和研究人员越来越强调替代和补充医学,对传统中医(TCM)的趋势越来越大。

患者关注

在糖皮质激素治疗后,所有 3 名患者的疾病均复发。患者 1 和 2 在静脉注射免疫球蛋白治疗后观察到症状缓解;然而,停止治疗后,他们的病情比以前恶化。

诊断

3 名患者均被明确诊断为血清 Caspr2 阳性 MoS,伴有一系列神经表现。

干预措施

3 名患者在中医理论的指导下进行治疗。根据中医的原则,患者的特点是阴虚,因此处方如下:芍药甘草汤合三甲复脉汤。

结果

应用中医后,神经精神表现如无意识的波动、抽搐、肌肉僵硬、肌强直、多汗症和极度便秘得到逆转。患者的生活质量显著改善;迄今为止,他们的卡诺夫斯基表现状态评分达到 100,病例 2 的抗 Caspr2 抗体从 1:32 降至正常。

经验教训

我们首次报告了使用中医作为补充和替代医学治疗莫旺综合征病例系列的有效治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2ba/11495757/ad98c464bdc9/medi-103-e40159-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2ba/11495757/ad98c464bdc9/medi-103-e40159-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a2ba/11495757/ad98c464bdc9/medi-103-e40159-g001.jpg

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