Abstract
Objective: To analyze the clinical features of common autoimmune encephalitis and evaluate the sensitivity of antibodies contributing to focal epilepsy signs and symptoms (ACES) score.Methods: Collecting and analyzing the data of 242 patients with autoimmune encephalitis (AE) diagnosed in the First Affiliated Hospital of Zhengzhou University from August 2015 to December 2020 in this retrospective study. The six items of the ACES score (cognitive symptoms, behavioral changes, autonomic symptoms, speech problems, autoimmune diseases, temporal MRI hyperintensities) were screened in patients with complete clinical data.Results: (1) In total, 242 patients were included, with 147 cases of anti-N-methyl-D-aspartate receptor encephalitis, 47 cases of anti-γ-aminobutyric acid type B (GABA-B) receptor encephalitis, and 48 cases of anti-leucine-rich glioma inactivating protein 1 (LGI1) encephalitis. The most common clinical symptoms are cognitive impairment (77%), behavioral changes (79%), and seizures (71%). In total, 129 cases (54%) combined with autonomic dysfunction, such as gastrointestinal dysmotility, sinus tachycardia, and central hypoventilation. Twelve patients had autoimmune diseases, most of which were of thyroid diseases. (2) One hundred and twenty-seven patients with complete clinical data evaluated ACES score, 126 cases of whom (126/127, 99.2%) were equal to or >2 points, 1 case (1/127, 0.8%) was of <2 points.Interpretation: (1) Cognitive impairment, abnormal behavior, and seizures are the most common manifestations of AE and autonomic symptoms. Thyroid disease is the most autoimmune disease in AE. Clinically, for patients of suspected AE, increasing the knowledge and testing of thyroid function and rheumatism is necessary. (2) ACES score is a simple, effective, and easy-to-operate score, with a certain screening value for most patients suspected of AE.
Highlights
Since the first case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was reported in 2007 [1], more and more autoimmune antibodies have been discovered, and autoimmune encephalitis (AE) has gradually become well-known
To identify AE at an early stage, Bruijn et al [2] conducted a prospective, multi-center, observational study in which 582 patients with unexplained focal epilepsy, and no obvious symptoms of encephalitis were tested for antibodies
Twenty patients were positive for autoimmune antibodies (GAD65 n = 13, leucine-rich glioma inactivating protein 1 (LGI1) n = 3, contactinassociated protein-2 (CASPR2) n = 3, NMDAR n = 1), and the clinical symptoms of the 20 patients were analyzed and the antibodies contributing to focal epilepsy signs and symptoms (ACES) score was obtained
Summary
Since the first case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was reported in 2007 [1], more and more autoimmune antibodies have been discovered, and autoimmune encephalitis (AE) has gradually become well-known. To identify AE at an early stage, Bruijn et al [2] conducted a prospective, multi-center, observational study in which 582 patients with unexplained focal epilepsy, and no obvious symptoms of encephalitis were tested for antibodies. Twenty patients were positive for autoimmune antibodies (GAD65 n = 13, LGI1 n = 3, CASPR2 n = 3, NMDAR n = 1), and the clinical symptoms of the 20 patients were analyzed and the antibodies contributing to focal epilepsy signs and symptoms (ACES) score was obtained. Antibody testing is recommended when the score is ≥2 points for which the sensitivity is 100%, specificity is 84.9%, and they are highly suspected as the autoimmune causes of epileptic seizures. To verify the sensitivity of the ACES score, 242 definite cases with AE were retrospectively collected, and clinical features of which were analyzed. 127 cases with complete data were selected to validate the score
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
