Use of Sugammadex in Strumpell-Lorrain Disease: a Report of Two Cases

Abstract

Content: : Strumpell-Lorrain disease - or familial spastic paraplegia (FSP) - is a rare hereditary neurological disorder, mainly characterized by variable degrees of stiffness and weakening of the muscles, with cognitive impairment, deafness, and ataxia in the more severe cases. We describe two female siblings with FSP programmed for cholecystectomy and subtotal colectomy, respectively, and also how we dealt with the anesthetic management in both cases and review the literature on this disease in relation to anesthesia. Keywords: Spastic Paraplegia, Hereditary, Anesthesia, Neuromuscular blockade/sugammadex.