Clinical analysis of patients with anti-SOX1 antibody-positive paraneoplastic neurologic syndrome
10.3760/cma.j.cn115354-20240417-00242
- VernacularTitle:抗SOX1抗体阳性神经系统副肿瘤综合征的临床研究
- Author:
Junhua LIANG
1
;
Wenjing ZHANG
;
Wei WANG
Author Information
1. 首都医科大学宣武医院神经内科，北京 100053
- Keywords:
Anti SOX1 antibody;
Paraneoplastic neurologic syndrome;
Small cell lung cancer
- From:
Chinese Journal of Neuromedicine
2024;23(6):560-570
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, diagnoses, treatments and prognoses of anti-sex-determining region of Y chromosome-related high mobility group box 1 (SOX1) antibody-positive paraneoplastic neurologic syndrome (PNS).Methods:Clinical data of 2 patients with anti-SOX1 antibody positive PNS admitted to Department of Neurology, Xuanwu Hospital, Capital Medical University in 2020 and 2021 were retrospectively analyzed. Eleven papers about 13 patients with anti-SOX1 antibody positive PNS published from January 1, 1999 to March 31, 2021 were retrieved from PubMed, Wanfang Data and CNKI. Clinical features, diagnoses, treatments and prognoses of these 15 patients were summarized.Results:Twelve males and 3 females were included, with age of (59.1±15.1) years (ranged 45-77 years); 7 patients had Lamber-Eaton myasthenic syndrome (LEMS), 4 had limbic encephalitis, 3 had paraneoplastic cerebellar degeneration, and 1 had autoimmune chorea. All 15 patients had tumors: 13 complicated with lung cancer (8 with small cell lung cancer), 1 had small cell carcinoma of esophagus, and 1 had nodular sclerosis Hodgkin lymphoma. Fourteen patients developed neurological symptoms before tumor diagnosis, and the longest delay of diagnosis was 1 year. Thirteen patients received treatments: 10 received tumor therapy and 11 received immunotherapy; 11 patients had improved neurological symptoms and 2 didn't respond to treatments.Conclusion:Clinical manifestations of anti SOX1 antibody-positive PNS are various, among which LEMS is the most common type, followed by limbic encephalitis and paraneoplastic cerebellar degeneration; small cell lung cancer is the most common complicated tumor; the overall prognosis is poor; early diagnosis and timely treatment of the primary tumors may be the key to improve the prognosis.
