Clinical characteristics of neurological syndrome associated with anti-glutamic acid decarboxylase antibodies
10.3760/cma.j.cn115354-20230108-00013
- VernacularTitle:抗谷氨酸脱羧酶抗体相关神经系统综合征的临床特点分析
- Author:
Huiqin LIU
1
;
Zeqin DONG
;
Jiajia XU
;
Milan ZHANG
;
Rui PANG
;
Yushu JIANG
;
Lipin YUAN
;
Yue HUANG
;
Wei LI
Author Information
1. 河南省人民医院，河南大学人民医院神经内科，郑州 450003
- Keywords:
Glutamic acid decarboxylase antibody;
Stiff-person syndrome;
Encephalitis;
Cerebellar ataxia;
Paraneoplastic syndrome
- From:
Chinese Journal of Neuromedicine
2023;22(7):683-689
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical characteristics of neurological syndrome associated with anti-glutamic acid decarboxylase (GAD) antibodies (Abs).Methods:Six patients with neurological syndrome associated with anti-GAD-Abs admitted to Department of Neurology, Henan Provincial People's Hospital from January 2019 to October 2022 were chosen. The clinical manifestations, imaging and laboratory results, therapeutic schedules, and follow-up prognoses of these patients were collected and summarized.Results:Three females and 3 males were included, with onset age of (54.3±17.7) years. Three patients had stiff-person syndrome (SPS), 1 had limbic encephalitis+generalized epilepsy, 1 had extralimbic encephalitis+occipital epilepsy, and 1 had cerebellar ataxia who was diagnosed with paraneoplastic syndrome associated with small cell lung cancer. Four patients had elevated level of thyroid peroxidase antibodies, and 1 patient was positive for overlapping anti-gamma aminobutyric acid B receptor antibodies and Amphiphysin antibodies. Two patients with SPS had failed lumbar puncture; 1 had slightly increased white blood cells and proteins in cerebrospinal fluid (CSF); the remaining 3 patients were basically normal. Specific oligoclonal bands in CSF were observed in 2 patients. Brain MRI showed abnormal signals in the bilateral occipital lobes in 1 patient, and no specific inflammatory lesions in other patients. All patients accepted corticosteroids and intravenous immunoglobulin/plasma exchange therapies; except for the one with paraneoplastic syndrome associated with small cell lung cancer, the remaining 5 patients had improved modified Rankin scale (mRs) scores at discharge and received long-term immunotherapy. Two patients with SPS had gradually aggravated symptoms, and mRs scores reached 5 at the last follow-up (one for 3 years and the other one for 2 years).Conclusions:The clinical manifestations of patients with neurological syndrome associated with anti-GAD-Abs include SPS, limbic encephalitis, extralimbic encephalitis, epilepsy and cerebellar ataxia; some of these patients have paraneoplastic syndromes. Immunotherapies are effective except for these patients with paraneoplastic syndromes. Some patients with SPS tend to have a chronic course and a poor prognosis.
