1. Perampanel: A therapeutic alternative in refractory status epilepticus associated with MELAS syndrome
- Author
-
Manuel Toledo, María Sueiras, Olga Maisterra, Silvana Sarria, Alicia Alpuente, Javier Salas-Puig, Lorena Guzmán, Laura Abraira, Estevo Santamarina, 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., and Vall d'Hebron Barcelona Hospital Campus
- Subjects
Pediatrics ,medicine.medical_specialty ,Status epilepticus ,MELAS syndrome ,Other subheadings::Other subheadings::/drug therapy [Other subheadings] ,Perampanel ,Article ,lcsh:RC321-571 ,Lesion ,03 medical and health sciences ,Behavioral Neuroscience ,chemistry.chemical_compound ,0302 clinical medicine ,Level of consciousness ,Refractory ,Heterocyclic Compounds::Heterocyclic Compounds, 1-Ring::Pyridines::Pyridones [CHEMICALS AND DRUGS] ,enfermedades musculoesqueléticas::enfermedades musculares::miopatías mitocondriales::encefalomiopatías mitocondriales::síndrome MELAS [ENFERMEDADES] ,Brain mri ,Medicine ,Initial treatment ,030212 general & internal medicine ,lcsh:Neurosciences. Biological psychiatry. Neuropsychiatry ,Otros calificadores::Otros calificadores::/tratamiento farmacológico [Otros calificadores] ,Nervous System Diseases::Neurologic Manifestations::Seizures::Status Epilepticus [DISEASES] ,business.industry ,Musculoskeletal Diseases::Muscular Diseases::Mitochondrial Myopathies::Mitochondrial Encephalomyopathies::MELAS Syndrome [DISEASES] ,Encefalitis - Tractament ,medicine.disease ,Epilèpsia - Tractament ,Piridina ,enfermedades del sistema nervioso::manifestaciones neurológicas::convulsiones::estado epiléptico [ENFERMEDADES] ,Neurology ,chemistry ,MELAS ,Neurology (clinical) ,medicine.symptom ,compuestos heterocíclicos::compuestos heterocíclicos de 1 anillo::piridinas::piridonas [COMPUESTOS QUÍMICOS Y DROGAS] ,business ,030217 neurology & neurosurgery - Abstract
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., Highlights • Status epilepticus (SE) in MELAS is associated with a stroke-lesion and it is usually refractory. • We present three cases of refractory SE and MELAS who responded favorably to Perampanel. • Perampanel (PER) may be an effective option in SE associated with MELAS syndrome.
- Published
- 2019