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oai:recercat.cat:11351/40332025-10-24T10:32:35Zcom_2072_378070com_2072_378040col_2072_378092

Perampanel: A therapeutic alternative in refractory status epilepticus associated with MELAS syndrome Santamarina Pérez, Estevo Maisterra Santos, Olga Sueiras Gil, Maria Sarria Estrada, Silvana Isabel Guzmán García, Lorena Carolina Salas Puig, Xavier Toledo Argany, Manuel Abraira del Fresno, Laura Alpuente Ruiz, Alicia Institut Català de la Salut [Santamarina E, Alpuente A]Unitat d’Epilèpsia, Servei de Neurologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Maisterra O] Unitat Neurovascular, Servei de Neurologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Sueiras M] Unitat d’Electroencefalografia, Servei de Neurofisiologia Clínica, Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Sarria S] Unitat MRI, Servei de Neuroradiologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Guzman L] Unitat d’Electroencefalografia, Servei de Neurofisiologia Clínica, Hospital Universitari Vall d'Hebron, Barcelona, Spain. [Abraira L, Salas-Puig J, Toledo M] Unitat d’Epilèpsia, Servei de Neurologia, Hospital Universitari Vall d'Hebron, Barcelona, Spain. Vall d'Hebron Barcelona Hospital Campus Epilèpsia - Tractament Encefalitis - Tractament Piridina DISEASES::Nervous System Diseases::Neurologic Manifestations::Seizures::Status Epilepticus DISEASES::Musculoskeletal Diseases::Muscular Diseases::Mitochondrial Myopathies::Mitochondrial Encephalomyopathies::MELAS Syndrome Other subheadings::Other subheadings::Other subheadings::/drug therapy CHEMICALS AND DRUGS::Heterocyclic Compounds::Heterocyclic Compounds, 1-Ring::Pyridines::Pyridones ENFERMEDADES::enfermedades del sistema nervioso::manifestaciones neurológicas::convulsiones::estado epiléptico ENFERMEDADES::enfermedades musculoesqueléticas::enfermedades musculares::miopatías mitocondriales::encefalomiopatías mitocondriales::síndrome MELAS Otros calificadores::Otros calificadores::Otros calificadores::/tratamiento farmacológico COMPUESTOS QUÍMICOS Y DROGAS::compuestos heterocíclicos::compuestos heterocíclicos de 1 anillo::piridinas::piridonas MELAS; Perampanel; Status epilepticus MELAS; Perampanel; Estatus epilèptic MELAS; Perampanel; Status epiléptico To our knowledge, there are no reports of status epilepticus (SE) associated with mitochondrial diseases and treated with perampanel (PER). We present three cases of patients with refractory SE associated with MELAS syndrome who responded favorably to PER. All cases were diagnosed as non-convulsive SE (focal without impairment of level of consciousness). After an initial treatment with other anti-seizure drugs, PER was added in all cases (8, 16 and 12 mg) and cessation of SE was observed within the next 4-8 hours. All the cases involved a stroke-like lesion present on brain MRI. In our patients, PER was an effective option in SE associated with MELAS syndrome. 2019-04-09T11:22:06Z 2019-04-09T11:22:06Z 2019-02-07 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Santamarina E, Alpuente A, Maisterra O, Sueiras M, Sarria S, Guzman L, et al. Perampanel: A therapeutic alternative in refractory status epilepticus associated with MELAS syndrome. Epilepsy Behav Case Report. 2019;11:92–5. 2213-3232 https://hdl.handle.net/11351/4033 10.1016/j.ebcr.2019.01.008 30834194 http://hdl.handle.net/11351/4033 eng Epilepsy and Behavior Case Reports;11 https://www.sciencedirect.com/science/article/pii/S2213323218301695?via%3Dihub#! Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/ info:eu-repo/semantics/openAccess application/pdf Elsevier Scientia