Clinical Characteristics and Short-Term Prognosis of Autoimmune Encephalitis: A Single-Center Cohort Study in Changsha, China

Abstract

Background and Purpose: The incidence and prevalence of autoimmune encephalitis is gradually increasing. This retrospective observational study primarily aimed to analyze the clinical characteristics of autoimmune encephalitis patients in the Second Xiangya Hospital and report patient prognoses after immunotherapy. Methods: The clinical data of 86 patients who were diagnosed with autoimmune encephalitis from October 2014 to September 2018 were collected, and their corresponding clinical characteristics, laboratory examination, treatment, and outcome data analyzed. Results: In our study, 72 patients (83.7%) were positive for anti-NMDAR (N-methyl-D-aspartate receptor) antibody; 5 patients (6%) for anti-GABABR (γ-aminobutyric acid receptor-A); 4 patients (4.7%) for anti-LGI1 (leucine-rich, glioma inactivated 1); 3 patients (3.5%) for anti-Caspr2 (contactin-associated protein-like 2) (1 patient was positive for both anti-LGI1 and anti-Caspr2 antibodies); and 3 patients (3.5%) for onconeural antibodies. Among the 86 patients diagnosed as having autoimmune encephalitis, 50% showed acute disease onset (≤2 weeks). The most common inducing factor was fever or cold (17/86, 19.8%). The main clinical symptoms included, among others, psychiatric disturbances (82.5%), epilepsy (60.5%), autonomic dysfunction (58.1%), sleep disorders (45.3%), consciousness disorders (45.3%), and speech disorders (46.5%). No significant correlation between ICU admission rates and CSF or serum antibody scores was observed. However, CSF antibody scores of (+ + +) and (++) were associated with longer lengths of hospitalization ( p < 0.05) and a higher CSF WBC count when compared with CSF antibody scores of (+) in patients with anti-NMDAR encephalitis ( p < 0.05). Additionally, there was no significant correlation between mRS score difference on admission and discharge (after immunotherapy) and age, sex, and choice of immune treatment, while immune therapy taken within 15 days from onset was more inclined to be associated with an mRS score difference ≥2 after immunotherapy in patients with anti-NMDAR encephalitis ( p = 0.006). Conclusions: Autoimmune encephalitis has an acute or sub-acute onset and presents with psychotic symptoms, epilepsy, and autonomic dysfunction. The sex ratio in anti-NMDAR encephalitis was nearly balanced. Infection was a major factor inducing anti-NMDAR encephalitis, and the CSF antibody scores could be helpful in determining its prognosis since these scores showed associations with hospitalization duration and CSF WBC counts.

Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study

“…Our results are similar to previous studies. In our study, the most common clinical symptoms in patients were psychiatric symptoms and epileptic seizures, consistent with other Chinese cohorts and most Western cohorts ( 7 , 8 , 14 , 15 ). In addition, consciousness impairment, memory deficit, speech disturbance, movement disorder and sleep disorder are common symptoms worth noting ( 17 ).…”

“…To our knowledge, this is the largest Chinese AE cohort to date. We identified anti-NMDAR encephalitis, anti-LGI-1 encephalitis, anti-GABAbR encephalitis, and anti-CASPR encephalitis as the most common subtypes in our cohort, which is similar to previous studies in China and in Western countries ( 7 , 8 , 14 , 15 ).…”

“…Most of the EEG abnormalities were slow waves, which are indicative of brain structural lesions. Epileptiform discharges such as sharp waves were also observed in the present and previous studies, indicating clinical or subclinical seizure activity ( 7 , 14 , 15 ).…”

“…Brain MRI and EEG are the main auxiliary examinations significant for the diagnosis of antibody-mediated AE. Findings on brain MRI and EEG analyses provide evidence that AE is a “diffuse encephalopathy” ( 7 , 15 , 27 ). In this study, abnormal signals on brain MRI were detected in 61.2% of the patients, predominantly in the medial temporal lobe.…”

“…Several cohort studies have been described in Western countries, whereas a few systemic research and information have been reported in East Asia. Some centers have reported cases with neurological involvement in patients with autoimmune disease and information about treatment or outcomes ( 7 – 9 ). However, more detailed information from cohort studies regarding baseline information or whether neurological symptoms are attributable to secondary mechanisms is needed.…”