title: Management of status dystonicus: our experience and review of the literature.
abstract: Status dystonicus (SD) is a life threatening disorder that develops in patients with both primary and secondary dystonia, characterized by acute worsening of symptoms with generalized and severe muscle contractions. To date, no information is available on the best way to treat this disorder. We review the previously described cases of SD and two new cases are reported, one of which occurring in a child with static encephalopathy, and the other one in a patient with pantothenate kinase-associated neurodegeneration. Both patients were admitted to an intensive care unit and treated with midazolam and propofol. This approach proved to be useful in the former while the progressive nature of the dystonia of the second patient required the combination of intrathecal baclofen infusion and bilateral pallidal deep brain stimulation. We believe that a rapid and aggressive approach is justified to avoid the great morbidity and mortality which characterize SD. Our experience, combined with the data available in the literature, might permit to establish the best strategies in managing this rare and severe condition.
authors.affiliation: Istituto di Neuropsichiatria Infantile, Università Cattolica del Sacro Cuore, Roma, Italia.
authors.forename: Paolo
authors.initials: P
authors.lastname: Mariotti
authors.forename: Alfonso
authors.initials: A
authors.lastname: Fasano
authors.forename: M Fiorella
authors.initials: MF
authors.lastname: Contarino
authors.forename: Giacomo
authors.initials: G
authors.lastname: Della Marca
authors.forename: Marco
authors.initials: M
authors.lastname: Piastra
authors.forename: Orazio
authors.initials: O
authors.lastname: Genovese
authors.forename: Silvia
authors.initials: S
authors.lastname: Pulitanò
authors.forename: Antonio
authors.initials: A
authors.lastname: Chiaretti
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Bentivoglio
mesh_terms: D000293:Adolescent; D006801:Humans; D008297:Male; D016135:Spinal Dysraphism / Q000628:therapy*