Clinical Features and Early Recognition of 242 Cases of Autoimmune Encephalitis

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.

Case Report: Paroxysmal hyperhidrosis as an initial symptom in a patient with anti-LGI1 encephalitis

“…The clinical features of anti-LGI1 encephalitis, namely cognitive impairment, psychiatric disorders, faciobrachial dystonic seizures (FBDS), and refractory hyponatremia, have been welldocumented in the literature since LGI1 was first identified as the true antigen for AE in 2010 (3,4). The number of confirmed cases of anti-LGI1 encephalitis has been increasing annually in recent years, and the phenotypic spectrum of neurological presentations has been expanding accordingly (5,6). It is important to note that the diverse clinical manifestations of anti-LGI1 encephalitis hamper its early diagnosis and treatment.…”

“…It is important to note that the diverse clinical manifestations of anti-LGI1 encephalitis hamper its early diagnosis and treatment. Some anti-LGI1 encephalitis patients have prodromal symptoms, such as fever, headache, dizziness, fatigue, or drowsiness (6). Subacute onset of cognitive dysfunction and psychiatric symptoms has been demonstrated in most cases of anti-LGI1 encephalitis (7,8).…”