Multivariate analysis was performed by ordinal logistic regression

Multivariate analysis was performed by ordinal logistic regression. == Discussion == This study investigated the outcomes of patients with AE caused by the three most antibodies (anti-NMDAR, anti-LGI1 and anti-CASPR2) with respect to IVIG treatment in a large cohort. treatment groups over time, and the factors influencing the reduction in mRS at discharge were analyzed. Furthermore, a specific investigation was conducted to determine the differences in outcomes between TMA-DPH patients treated with ivMP + IVIG and ivMP + PE, each split by antibody subtype. == Results TMA-DPH == In all treatment groups analyzed, significant improvements were observed at the time of discharge and after 12 months compared to TMA-DPH disease onset, regardless of the type of first-line treatment. In untreated patients a significant improvement was not observed. The choice of IVIG or PE as an additional treatment to ivMP for anti-NMDAR encephalitis did not affect the primary outcome. In anti-LGI1 or anti-CASPR2 encephalitis, no influence on the primary outcome was observed when IVIG or PE was administered in addition to ivMP, too. However, a direct comparison of the individual antibody subgroups mRS reductions, depending on the treatment approach (ivMP + IVIG vs. ivMP + PE), revealed that a more significant mRS reduction was observed with ivMP + PE in anti-NMDAR encephalitis. == Discussion == The retrospective data give evidence that there is no difference in outcome for the use of ivMP + PE over ivMP + IVIG Rabbit Polyclonal to p38 MAPK (phospho-Thr179+Tyr181) or vice versa in the treatment of encephalitis caused by antibodies against NMDAR, LGI1 TMA-DPH or CASPR2. Furthermore, the specific method of plasma exchange, whether plasmapheresis or immunoadsorption, did not affect the mRS at discharge. == Supplementary Information == The online version contains supplementary material available at 10.1007/s00415-025-13032-0. Keywords:Autoimmune encephalitis, NMDA, LGI-1, CASPR2, IVIG, Immunotherapy == Introduction == The majority of antibodies that have been identified as targeting cell surface-expressed molecules in the central nervous system (CNS) have been shown to be directly associated with the development of autoimmune encephalitis (AE) [1]. Along with the N-methyl-D-aspartate receptor (NMDAR), leucine-rich-glioma-inactivated-1 (LGI1) and contactin-associated-protein-like-2 (CASPR2) are the three most common target antigens in antibody-mediated encephalitis. The age of onset for anti-NMDAR encephalitis ranges from 0.6 to 85 years, for anti-LGI1 encephalitis from 30 to 80 years, and for anti-CASPR2 encephalitis from 46 to 77 years [2]. The clinical presentation of anti-NMDAR encephalitis is typically characterized by the subacute onset of psychotic symptoms, which may include hallucinations, paranoia or behavioural disturbances, frequently followed by additional neurological manifestations such as movement disorders or seizures. Patients with anti-LGI1 encephalitis often present with focal seizures, particularly of the faciobrachial dystonic type, in addition to complex focal seizures accompanied by cognitive or autonomic features. Anti-CASPR2 encephalitis features cognitive impairment, memory disorders, seizures, cerebellar symptoms and sleep disturbances [3].The autonomic nervous system as well as the peripheral nervous system can also be affected by antibodies against CASPR2. The treatment of autoimmune encephalitis is typically a multi-step process that aims to regulate the autoimmune response. In the absence of randomised controlled trials, current treatment protocols are largely based on clinical experience and retrospective analyses. First-line treatment typically comprises high-dose corticosteroids such as intravenous methylprednisolone (ivMP), in conjunction with plasmapheresis (PE) or intravenous immunoglobulin (IVIG) TMA-DPH [4]. Corticosteroids are frequently the first choice due to their potent anti-inflammatory effects, while PE and IVIG are considered when rapid immunomodulation is necessary. In cases that are refractory to first-line treatment, second-line therapies such as rituximab and cyclophosphamide are employed. These agents aim to accomplish more serious and continuous suppression of the immune response, especially in instances that are severe or relapsing [5]..