Total corpus callosotomy for medically refractory status epilepticus due to Progressive Myoclonic Epilepsy: A clinical challenging case.

Abstract

BACKGROUND\nProgressive myoclonic epilepsy (PME) is a syndrome characterized by development of progressive myoclonus, cognitive impairment and other neurological deficits. Despite major advances in medical treatment of epilepsy, some PME patients remain refractory to antiepileptic drugs. This may further accentuate cognitive impairment and deteriorate functional capacity. Corpus callosotomy (CC) is used in patients with drug-resistant epilepsy who are neither candidates for excisional epilepsy surgery nor neuro-stimulation. We report application of the standard complete callosotomy to control medically refractory status epilepticus in a patient with PME.\n\n\nCASE DESCRIPTION\nA 16-year-old boy was referred to emergency department with generalized tonic-clonic seizure. He was known case of PME since five years ago with frequent generalized seizures requiring hospitalization and reloading of the drugs. The patient was discussed about at the epilepsy surgery working group, and corpus callosotomy was considered as the last resort to control the refractory status epilepticus. The patient experienced no generalized seizure during 3 months postoperative period (Engel class IIIB).\n\n\nCONCLUSIONS\nSince surgery was the last resort to control severe disabling status epilepticus and because most of epileptogenic discharges were originating from parieto-occipital regions and due to presence of profound cognitive impairment, we decided to perform a complete rather than just anterior callosotomy. CC may be considered to prevent secondary generalized seizures as the most disabling attacks in patients who suffer from certain epilepsy syndromes. Nevertheless, the impact of palliative surgical intervention on the overall disease course of the patients with an underlying diffuse pathology remains to be determined.