title: Use of sugammadex in Strumpell-Lorrain disease: a report of two cases.
abstract: 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.
authors.affiliation: Resident of the Department of Anesthesiology and Resuscitation, Miguel Servet University Hospital, Zaragoz, Spain. Electronic address: jafh73@hotmail.com.
authors.forename: José Antonio
authors.initials: JA
authors.lastname: Franco-Hernández
authors.affiliation: Staff physician, Department of Anesthesiology and Resuscitation, Miguel Servet University Hospital, Zaragoza, Spain.
authors.forename: Luis
authors.initials: L
authors.lastname: Muñoz Rodríguez
authors.affiliation: Staff physician, Department of Anesthesiology and Resuscitation, Miguel Servet University Hospital, Zaragoza, Spain.
authors.forename: Pilar Jubera
authors.initials: PJ
authors.lastname: Ortiz de Landázuri
authors.affiliation: Staff physician, Department of Anesthesiology and Resuscitation, Miguel Servet University Hospital, Zaragoza, Spain.
authors.forename: Alejandra
authors.initials: A
authors.lastname: García Hernández
mesh_terms: D000328:Adult; D000732:Androstanols / Q000037:antagonists & inhibitors; D000768:Anesthesia, General; D002763:Cholecystectomy; D003082:Colectomy; D005260:Female; D006801:Humans; D008875:Middle Aged; D019148:Neuromuscular Blockade; D003473:Neuromuscular Nondepolarizing Agents / Q000037:antagonists & inhibitors; D000077123:Rocuronium; D015419:Spastic Paraplegia, Hereditary / Q000188:drug therapy*; D000077122:Sugammadex; D047408:gamma-Cyclodextrins / Q000627:therapeutic use*
chemical_list: D000732:Androstanols; D003473:Neuromuscular Nondepolarizing Agents; D047408:gamma-Cyclodextrins; D000077122:Sugammadex; D000077123:Rocuronium
keywords: Anesthesia; Hereditary; Neuromuscular blockade/sugammadex.; Spastic Paraplegia