Status dystonicus: Predictors of outcome and progression patterns of underlying disease

Alfonso Fasano, Lucia Ricciardi, Anna Rita Bentivoglio, Carlotta Canavese, Giovanna Zorzi, Igor Petrovic, Nikola Kresojevic, Vladimir S. Kostić, Marina Svetel, Norbert Kovacs, Istvan Balas, Agathe Roubertie, Devendra Mishra, Paolo Mariotti, Teresa Temudo, Nardo Nardocci

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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

Original languageEnglish
Pages (from-to)783-788
Number of pages6
JournalMovement Disorders
Volume27
Issue number6
DOIs
Publication statusPublished - May 1 2012

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Keywords

  • Dystonia
  • Dystonic storm
  • Midazolam
  • Pantothenate-kinase-associated neurodegeneration
  • Status dystonicus

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Fasano, A., Ricciardi, L., Bentivoglio, A. R., Canavese, C., Zorzi, G., Petrovic, I., Kresojevic, N., Kostić, V. S., Svetel, M., Kovacs, N., Balas, I., Roubertie, A., Mishra, D., Mariotti, P., Temudo, T., & Nardocci, N. (2012). Status dystonicus: Predictors of outcome and progression patterns of underlying disease. Movement Disorders, 27(6), 783-788. https://doi.org/10.1002/mds.24981