Clinical characteristics and prognoses of 13 patients with Morvan syndrome with obvious electromyography characteristics
10.3760/cma.j.cn115354-20240403-00297
- VernacularTitle:具有明显肌电图特征的13例莫旺综合征患者临床特点与预后分析
- Author:
Zeqin DONG
1
;
Wenli MEI
;
Shaomin ZUO
;
Jiajia XU
;
Lipin YUAN
;
Wei LI
;
Huiqin LIU
Author Information
1. 河南大学人民医院（河南省人民医院）神经内科，郑州 450003
- Keywords:
Morvan syndrome;
Autoimmune disease;
Neuroelectrophysiology;
Contactin-associated protein-like 2;
Leucine-rich glioma inactivated 1;
Peripheral nerve hyper
- From:
Chinese Journal of Neuromedicine
2024;23(5):471-477
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical manifestations, neuroelectrophysiological characteristics and prognoses of Movan syndrome (MoS), and provide references for early diagnoses and prognoses.Methods:A retrospective analysis was performed. The clinical data, such as clinical symptoms, treatments and prognoses, laboratory test results and electrophysiological test results, of 13 patients with confirmed MoS in Department of Neurology, He'nan Provincial People's Hospital from January 2018 to October 2023 were collected.Results:Ten male MoS patients and 3 female ones were included. Main clinical manifestations of 13 patients with MoS included myokymia, pain, numbness of limbs, itching all over the body, hyperhidrosis, urinary and defecation disorder, tachycardia, insomnia, anxiety and depression. Ten patients completed the autoimmune encephalitis antibody detection: 3 only had positive anti-contactin-associated protein-like 2 (CASPR2) antibody, 2 only had positive anti-leucine-rich glioma-inactivated protein1 (LGI1) antibody, and 2 had both positive anti-CASPR2 antibody and anti-LGI1 antibody. Eleven patients completed tumor screening and 4 tumors (thymoma [ n=2], lung squamous cell carcinoma [ n=1] and adrenal non-Hodgkin's lymphoma [ n=1]) were noted. Ten patients completed electrocardiogram, including 3 patients with resting tachycardia and 2 patients with ST segment elevation. All patients completed the electromyographic examination; 12 patients showed abnormal motor unit potential, including myokymia potential, fasciculation potential and neuromyotonic potential; F-wave and/or M-wave post-discharge potentials were found in all patients. Follow up was performed for 1-12 months; in 9 non-tumor patients, 5 were improved in 6 patients accepted immunotherapy and one was improved in 3 patients received symptomatic treatment; in 4 tumor patients, only one was improved in 3 received immunotherapy. Conclusion:Myokymia, pain, urinary and defecation disorder, and severe insomnia are typical symptoms for MoS patients; serum anti-CASPR2/LGI1 antibody and electromyography results provide evidences for MoS diagnosis; early immunotherapy can improve the MoS prognosis, and MoS patients combined with tumors have poor clinical prognosis.
