BACKGROUND: Status dystonicus (SD) is a rare, life-threatening disorder characterized by acute worsening of generalized dystonia. METHODS: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. RESULTS: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). CONCLUSIONS: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.
BACKGROUND:Status dystonicus (SD) is a rare, life-threatening disorder characterized by acute worsening of generalized dystonia. METHODS: This study was conducted to characterize the pathogenesis, clinical course, and prognosis of SD. We reviewed the records of six centers and analyzed them together with all the cases previously reported in the literature. RESULTS: Eighty-nine episodes occurring in 68 patients were studied. The majority of patients were males (64.7%), were <15 years of age (58.8%), and had secondary dystonia as the underlying condition (37.8%). The episodes were mainly characterized by tonic muscle spasms (68.5%), with phasic forms more common in secondary forms and among females. Almost all cases needed a multistaged approach, with surgery being the most successful strategy. Neurological conditions preceding the episode worsened in 16.2% of cases (ending in death in 10.3%). CONCLUSIONS: The course and outcome of SD is highly variable; male gender and prevalent tonic phenotype predict a poor outcome.
Authors: Laura Cif; Manju A Kurian; Victoria Gonzalez; Sara Garcia-Ptacek; Thomas Roujeau; Philippe Gelisse; Ana Maria Moura de Ribeiro; Arielle Crespel; Lesley MacPherson; Philippe Coubes Journal: Mov Disord Clin Pract Date: 2014-05-23