Síndrome de Susac: actitud diagnóstica y terapéutica. Presentación de un nuevo caso

Abstract

The Susac syndrome is made up of the clinical triad: encephalopathy, visual and hearing defects. It is caused by microangiopathy of unknown origin affecting the small arteries of the brain, retina and cochlea. It is very uncommon. It mainly affects young women. The course of the illness is usually monophasic and self-limiting. The deficit of visual acuity is caused by occlusion of tributaries of the retinal artery. The auditory defect is bilateral and symmetrical, and particularly affects medium and low frequencies. NMR is of great diagnostic value, showing multiple lesions in the grey and white matter. Our case gives more data regarding the evolution of this condition and the contribution of cerebral SPECT to diagnosis, the results of systemic treatment, use of hyperbaric oxygen and reflections on the physiopathology of the process. We present the clinical case of a young woman who presented with psychiatric symptoms and migraine followed by clinical encephalopathy and acute/subacute coma. There were also visual and auditory deficits. Other types of systemic disease were ruled out. Findings on SPECT suggested the presence of a microangiopathic disorder of the brain. The patient responded to systemic treatment with cortico-steroids. The encephalopathy resolved in a few days and two months later she had resumed her former daily activities. Treatment with hyperbaric oxygen definitely reduced visual sequelae.