Clinical characteristics of autoimmune encephalitis with co-existence of multiple anti-neuronal antibodies

Abstract

Abstract Background Autoimmune encephalitis (AE) usually referred to a single anti-neuronal antibody-mediated encephalopathy syndrome. AE with co-existence of multiple anti-neuronal antibodies was reported in a few case reports or single-center retrospective studies. Further studies might be helpful to elucidate the uncertainties of clinical features, treatment and prognosis. Methods AE patients with two or more anti-neuronal antibodies were retrospectively recruited between August 2019 and February 2022 in our center. Clinical and laboratory data were collected. A total of 83 AE cases (9 cases in this study and 74 cases in the literatures) with multiple anti-neuronal antibodies were summarized and re-analyzed. Results Nine AE patients with co-existence of multiple anti-neuronal antibodies were identified from our 85 AE patients. The combination of anti-neuronal antibodies included NMDAR plus CASPR2 in 3 cases, NMDAR plus GABABR in 2 cases, AMPAR2 plus Hu in one, LGI1 plus IGLON5 in one, LGI1 plus CASPR2 in one, NMDAR plus GABABR and GAD65 in one. All 9 patients presented with encephalitis symptoms, clinically characterized by disturbance of consciousness, seizures, cognitive impairment, and psychiatric disorder. All 83 cases revealed that 73 cases had co-existence of two types of antibodies, 8 cases had three types, and 2 cases had four types. Thirty-nine cases (39/83, 46.9%) were confirmed or suspected to be accompanied with tumors, of which the most common was lung cancer (28/83, 33.7%). Partial or complete recovery was achieved in 57 cases (57/83, 68.6%), and 26 cases (26/83 31.3%) died during treatment or follow-up. Conclusions AE with co-existence of multiple anti-neuronal antibodies was a specific subgroup, which was increasingly recognized in the clinical practice. The co-existence of multiple anti-neuronal antibodies had complicated impacts on clinical features, disease progression, and prognosis.