[Non-convulsive epileptic status associated with Lafora disease: two case reports]. / Estado epiléptico no convulsivo asociado a enfermedad de Lafora: presentación de dos casos.

ABSTRACT

INTRODUCTION: Lafora s disease is a type of progressive myoclonic epilepsy with poor prognosis, is characterized by myoclonic crisis, tonic clonic seizures, absence or partial complex seizures and other neurological manifestations with a progressive course and a poor response to the treatment. It has not been considered as a cause of epileptic status. CASE REPORTS Two women without important past medical history with normal psychomotor development before their suffering, with manifestations of 2 years of evolution the first one and 8 years on the second case characterized by myoclonic generalized, partial complex seizures and progressive deterioration of the mental functions that joined to our institution in a non convulsive epileptic status and they featured with a different evolution. The first patient with favorable control of the event with a single medication and functionality recover later, the second one with torpid evolution complicated with an epileptic status convulsive widespread condition and a prolonged permanency in the unit of intensive therapy. In both patients the diagnosis of Lafora s disease was established based in the findings of the skin axilar biopsy. DISCUSSION AND CONCLUSION: We believe that Lafora s disease must be suspected as a probable cause of non convulsive epileptic status in patients with myoclonic epilepsy associated with other neurological manifestations and a refractary response to the medical treatment. The evolution and clinical response will depend on the evolutionary stage of the disease.