Síndrome de Cogan: manifestaciones neurológicas. Reporte de caso y revisión de la bibliografía

Cogan’s syndrome is a rare entity of inflammatory and autoimmune origin apparently triggered through molecular mimetism by infectious agent. Immunodominant peptides similar to autoantigens SSA/Ro and Lambda protein1 of Reovirus III as well as sequences of tyrosin phosphatase-1 (DEP-1/CO 148) that are capable as well to transfect laboratory animals, are expressed in endothelial cells, sensory epithelium, neurons and glia. The clinical result is a multi-system complex with constitutional symptoms, uveitis, interstitial keratitis, acute deafness and vasculitic manifestation in central and peripheral nervous system. We describe here the first observations of brain lesions by MR. The syndrome responds to therapy with steroids and limited immunosuppression.