title: Status dystonicus: predictors of outcome and progression patterns of underlying disease.
abstract: 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.affiliation: Istituto di Neurologia, Università Cattolica del Sacro Cuore, Rome, Italy. alfonso.fasano@rm.unicatt.it
authors.forename: Alfonso
authors.initials: A
authors.lastname: Fasano
authors.forename: Lucia
authors.initials: L
authors.lastname: Ricciardi
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Bentivoglio
authors.forename: Carlotta
authors.initials: C
authors.lastname: Canavese
authors.forename: Giovanna
authors.initials: G
authors.lastname: Zorzi
authors.forename: Igor
authors.initials: I
authors.lastname: Petrovic
authors.forename: Nikola
authors.initials: N
authors.lastname: Kresojevic
authors.forename: Vladimir S
authors.initials: VS
authors.lastname: Kostić
authors.forename: Marina
authors.initials: M
authors.lastname: Svetel
authors.forename: Norbert
authors.initials: N
authors.lastname: Kovacs
authors.forename: Istvan
authors.initials: I
authors.lastname: Balas
authors.forename: Agathe
authors.initials: A
authors.lastname: Roubertie
authors.forename: Devendra
authors.initials: D
authors.lastname: Mishra
authors.forename: Paolo
authors.initials: P
authors.lastname: Mariotti
authors.forename: Teresa
authors.initials: T
authors.lastname: Temudo
authors.forename: Nardo
authors.initials: N
authors.lastname: Nardocci
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D020821:Dystonic Disorders / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D009125:Muscle Relaxants, Central / Q000627:therapeutic use; D011379:Prognosis; D012737:Sex Factors; D016896:Treatment Outcome
chemical_list: D009125:Muscle Relaxants, Central