Year: 2024
Type: article
Study design: Case Report
Abstract: Objective To describe four cases of relapsing AE that showed improvement with maintenance immunotherapy. Background Autoimmune encephalitis (AE) is a subacute immune-mediated inflammatory brain disorder characterized by memory loss, altered mental status, seizures, and psychiatric symptoms due to autoantibodies against neuroglial proteins. Although typically monophasic, 10-30% of cases may relapse leading to cognitive decline, treatment-resistant epilepsy, and severe psychiatric issues. While factors contributing to relapse risk are not fully understood, female sex and delayed treatment have been associated with an increased risk. We report four cases of relapsing AE that improved with maintenance immunosuppressive treatment. Design/Methods Patients were retrospectively evaluated from August 1st to December 31st, 2023. Results Four patients were diagnosed with confirmed autoimmune encephalitis. Three of them were female with a median age of 61 years. The most common presenting symptoms were headache, memory problems and seizures. One patient had facial-brachial dystonic seizures, and one presented with status epilepticus. Three patients showed psychotic features at presentation while one had a history of psychosis. Two patients had elevated GAD-65 in CSF, one had TPO antibodies and one patient had LG-1 antibody positive in CSF with MRI showing T2 bilateral temporal lobe hyperintensities. None of the patients received high-dose steroids on admission, but two received IVIG for five days. One did not respond to IVIG and underwent plasmapheresis for 5 days. None of the patients were discharged on any maintenance immunotherapy. However, all patients experienced a relapse of AE within two weeks of discharge. On re-admission, two patients received IVIG, one IV steroids, and one IV steroids plus IVIG, all were discharged on steroids as maintenance therapy for 2 months. Three patients did not develop further relapses, while one did. Conclusions AE can have relapses despite early immunosuppressive therapy as observed in two of our patients, possibly related to the lack of immunosuppressive maintenance therapy.
Source: Neurology
Authors: Muhammad Khalid, Alexander Carvajal-Gonzalez, Prabandh Buchhanolla, Muhammad Ayub, Arvin Parvathaneni, Erik Burton
Language: English
Subfield: Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4403207085