Status dystonicus: study of five cases Status dystonicus: estudo de cinco casos

Abstract

Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.O "Status Dystonicus" (SD) é uma situação clínica caracterizada por contratura muscular generalizada em pacientes com distonias. Relatamos cinco casos de SD, que ocorreram em dois pacientes com distonia associada à paralisia cerebral, um paciente com distonia segmentar primária, um com síndrome de Hallervorden-Spatz e um com doença de Wilson (DW). Três pacientes foram submetidos a tratamento em terapia intensiva com uso de propofol e midazolam e dois pacientes foram submetidos ao tratamento neurocirúrgico (um paciente com palidotomia bilateral e outro com estimulador cerebral profundo palidal bilateral). Em três casos foram identificados fatores desencadeantes: infecção, estresse acentuado e o uso de zinco no tratamento da DW. Em dois pacientes ocorreu melhora importante do quadro distônico, e outros três retornaram à situação clínica pré-SD.

Keywords

• status dystonicus
• tempestade distônica
• distonia
• status dystonicus
• dystonic storm
• dystonia