Clinical study of autonomic dysfunction in patients with autoimmune encephalitis

Abstract

Background: Autoimmune encephalitis (AE) is a collective name, covering an emerging spectrum of autoimmune-mediated neurological diseases related to antibodies and synaptic or intracellular proteins. Anti-NMDAR, anti-LGI1, and anti-GABABR are three types of neuronal cell surface antibodies. Autonomic dysfunction represents a frequently occurring clinical manifestation. This observational study purposes to investigate comparisons between two groups with or without autonomic dysfunction and detect the autonomic dysfunction and other indexes in anti-NMDAR, anti-LGI1, and anti-GABABR cohorts.

Methods: Patients with anti-NMDAR, anti-LGI1 and anti-GABABR encephalitis were recruited from the May 2017 to the April 2022. The following information was recorded: age, age at onset, tumor presence, gender, prodromal symptoms, clinical manifestations, cranial magnetic resonance imaging, cerebrospinal fluid and blood examinations, and immunotherapy.

Results: There were totally 161 patients enrolled in this study. Among these participants, 104 individuals (64.6%) presented autonomic dysfunction and the remaining 57 (35.4%) were free of autonomic dysfunction. Sinus tachycardia was the most common autonomic dysfunction, followed by pollakiuria/uroclepsia, feverscence, central hypoventilation, sinus bradycardia, constipation, uroschesis, hyperhidrosis, hypersalivation, hypotension, and early satiety/emesis. Compared to patients without autonomic dysfunction, those with autonomic dysfunction had a higher incidence of central hypoventilation and ICU admissions. Meanwhile, in both groups with or without autonomic dysfunction, meatal behavior disorder, cognitive impairment, and epileptic seizure were three most common clinical manifestations. There were no significant differences in cranial magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) examination, antibody titers and number of immunotherapy types. Further analysis of AE mediated by distinct neuronal surface antibodies demonstrated that there were 85 anti-NMDAR, 56 anti-LGI1, and 20 anti-GABABR encephalitis patients. The significant differences between these three cohorts appeared in age, tumor presence, fervescence presence and antibody titers.

Conclusion: This study demonstrated the comparisons between autonomic dysfunction group and autonomic dysfunction-free group and provided insights into better diagnosis and treatment.

Keywords: Anti-GABABR; Anti-LGI1; Anti-NMDAR; Autoimmune encephalitis; Autonomic dysfunction; Clinical study.