Síndrome De Kinsbourne: Papel De Rituximab / Kinsbourne Syndrome: Role Of Rituximab

RESUMEN

Resumen El Síndrome de Kinsbourne también conocido como "síndrome de opsoclonus-mioclonus" (SOM) o "síndrome de los ojos danzantes" se considera una enfermedad rara y poco frecuente. Se caracteriza por una triada clásica movimientos oculares rápidos, irregulares, multidireccionales (opsoclonus), movimientos mioclónicos en tronco, cara y/o extremidades y ataxia. La etiología es diversa pudiendo presentarse tanto de origen paraneoplásico como no paraneoplásico e idiopática. Fundamentalmente el tratamiento se realiza con inmunosupresores (corticoides), posteriormente inmunoglobulinas inespecifícas endovenosas y rituximab endovenoso. Procedemos a presentar un caso clínico de un niño de 4 años el cuál presentó dicho síndrome debido a una infección por Klebsiella pneumoniae.

ABSTRACT

Abstract Kinsbourne syndrome, also known as "Opsoclonus-myoclonus syndrome" or "Dancing eyes syndrome" is a rare condition characterized by rapid, irregular, multi-directional eye movements (opsoclonus), myoclonic movements in the trunk, face and/or limbs and ataxia. It occurs in children aged between 6 and 36 months. Its etiology may be paraneoplasic (neuroblastoma), non-paraneoplasic (infectious processes) or idiopathic. Regardless of its etiology, immunosuppressive drugs have been used in order to reduce the formation of antibodies possibly involved in the pathophysiology. We report the case of a four years-old boy with this syndrome secondary to an infectious urinary illness. He had ataxia, opsoclonus, upper limbs myoclonus, irritability and altered sleep pattern. Neuroblastoma was ruled out. Initial management was done with methylprednisolone pulses, followed by oral prednisolone and intravenous gammaglobulin. He started with rituximab with a good response. He had progressive clinical improvement, and is currently asymptomatic with no sequelae. In these patients a paraneoplasic syndrome should always be ruled out.