Immunotherapy Profile in the Rasmussen Encephalitis Treatment at a Brazilian Epilepsy Center
Abstract number :
3.441
Submission category :
8. Non-ASM/Non-Surgical Treatments (Hormonal, alternative, etc.)
Year :
2024
Submission ID :
470
Source :
www.aesnet.org
Presentation date :
12/9/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Tomásia Frezatti, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Úrsula Thomé, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Ricardo Saute, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Marina Dalio, MSc, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Frederico Nakano, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Veriano Alexandre Jr, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Tonicarlo Velasco, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Ana Paula Hamad, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Antônio Carlos Santos, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Vanessa Marques, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Américo Sakamoto, PhD, MD – Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP
Rationale: Rasmussen Encephalitis (RE) was first described after histological analysis from 3 children operated due to intractable focal seizures and progressive hemiparesis (RASMUSSEN, 1958). In 2005, a consensus proposed formal diagnostic criteria and a therapeutic pathway for RE. Despite epilepsy surgery being considered “the only ‘cure’ of the disease progression”, immunotherapy is considered in some cases (BIEN, 2005). RE evolves in 3 stages (prodromal, acute, and residual), and animal models showed that immunotherapy cannot reduce seizures once the disease is established. So, possible therapeutic windows were proposed (LAGARDE, 2022). As there are no standard immunotherapy protocols, data of small series remain relevant. We report a Brazilian Epilepsy Center experience with various immunomodulatory treatments in six RE patients.
Methods: We studied six patients with RE diagnosis, according to literature criteria (BIEN, 2005), who received immunotherapy. The treatment efficacy was classified as no response, reduction of seizures < 50%, and reduction of seizures > 50% or improvement of neurologic status.
Results: The data are summarized in Table 1. We evaluated five children and one adult. The time between epilepsy onset (EO) and clinical deterioration (status epilepticus or paresis) was seven days to 8 years. The admission at Epilepsy Center occurred six months to 7 years after EO. Patients received the first immunotherapy one month to 6 years after EO. The initial treatments in most patients were methylprednisolone and immunoglobulin. The other immunotherapies were azathioprine (3/6), rituximab (3/6), tacrolimus (2/6), cyclophosphamide (2/6), mycophenolate (1/6) and plasmapheresis (1/6). Patients used 3 to 5 immunotherapies over time, with variable treatment duration. Patient 1 had the longest immunotherapy trial, using azathioprine for over eight years. Figure 1 shows a schematic view of treatments over time. Patient 3 had the shorter time between EO and the immunotherapy, with the best initial response.
Conclusions: Considering a therapeutic window, the efficacy of immunotherapy might be affected by the time of disease. Furthermore, lacking solid evidence on the theme results in many different approaches. Our data are too limited to compare the efficacy of immunotherapies in RE patients. However, they are relevant in highlighting the importance of creating a treatment protocol and considering not just the diagnosis itself but also the time of progression and the mechanisms of available immunotherapies, besides the importance of referring and treating these patients as soon as possible.
Funding: This research received no specific grant from any funding agency.
Non-ASM