Perampanel: A therapeutic alternative in refractory status epilepticus associated with MELAS syndrome

Estevo Santamarina; Alicia Alpuente; Olga Maisterra; María Sueiras; Silvana Sarria; Lorena Guzman; Laura Abraira; Javier Salas-Puig; Manuel Toledo

doi:10.1016/j.ebcr.2019.01.008

Perampanel: A therapeutic alternative in refractory status epilepticus associated with MELAS syndrome

Epilepsy Behav Case Rep. 2019 Feb 7:11:92-95. doi: 10.1016/j.ebcr.2019.01.008. eCollection 2019.

Authors

Estevo Santamarina¹, Alicia Alpuente¹, Olga Maisterra², María Sueiras³, Silvana Sarria⁴, Lorena Guzman³, Laura Abraira¹, Javier Salas-Puig¹, Manuel Toledo¹

Affiliations

¹ Epilepsy Unit, Department of Neurology, Vall d'Hebron University Hospital, Barcelona, Spain.
² Neurovascular Unit, Vall d'Hebron University Hospital, Barcelona, Spain.
³ EEG Unit, Department of Neurophysiology, Vall d'Hebron University Hospital, Barcelona, Spain.
⁴ MRI Unit, Department of Neuroradiology, Spain.

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.

Keywords: MELAS; Perampanel; Status epilepticus.

Publication types

Case Reports