Clinical characteristics of movement disorders in patients with anti-N-methyl-D-aspartate acid receptor encephalitis / 中华神经科杂志

ABSTRACT

Objective:To explore the clinical characteristics, therapeutic effect and prognosis of movement disorders in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods:The prospectively collected data of hospitalized 163 patients with anti-NMDAR encephalitis admitted to Xuanwu Hospital, Capital Medical University from June 2012 to October 2019 were analyzed. According to the presence of movement disorders, the patients were divided into movement disorders group (75 cases, 46.0%) and non-movement disorders group (88 cases, 54.0%). Patients were followed up for six months and 12 months after immunotherapy. The clinical manifestations, auxiliary examinations, treatment and prognosis of the two groups were compared.Results:Among 163 patients with anti-NMDAR encephalitis, 91 patients (55.8%) were male and 72 patients (44.2%) were female, with an age of 26(19, 34) years. In the 75 patients of the movement disorders group, 50 patients (66.7%) presented with orofacial dyskinesia, 45 patients (60%) with limb stereotypies, 28 patients (37.3%) with choreoathetosis, nine patients (12.0%) with ballism, seven patients (9.3%) with bradykinesia, five patients (6.7%) with tremor, and 13 patients (17.3%) with status dystonicus. Compared with the non-movement disorders group, the movement disorders group had a higher proportion of ovarian teratoma (14.7% vs 3.4%), modified Rankin Scale score of 3-5 before immunotherapy (76.0% vs 33.0%), abnormal electroencephalogram (89.3% vs 77.3%), increased lumbar puncture pressure (53.3% vs 34.1%), cerebrospinal fluid (CSF) pleocytosis (73.3% vs 51.1%), strong positive NMDAR antibody of CSF (44.0% vs 25.0%), admitting to intensive care unit (60.0% vs 9.1%), treated with intravenous immunoglobulin (80.0% vs 40.9%), plasma exchange (36.0% vs 3.4%), and immunosuppressive therapy (37.2% vs 17.0%); had shorter days from the onset to the beginning of immunotherapy [20(10, 33) d vs 35(15, 77) d]; had longer days from the beginning of immunotherapy to the improvement [34(20, 60) d vs 20(15, 35) d]; and there were significant differences of above items between the two groups ( P<0.05). There was no significant difference in the prognosis and relaps between the two groups at six and 12 months after immunotherapy. Conclusions:Nearly a half of patients with anti-NMDAR encephalitis had movement disorders with multiple phenotypes. The severity of movement disorders was related to the severity of the disease. After active immunotherapy and symptomatic treatment, movement disorders improved with the improvement of primary disease in majority of patients.