2017
DOI: 10.1002/mds.27207
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Rethinking status dystonicus

Abstract: Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme cli… Show more

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Cited by 47 publications
(56 citation statements)
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References 74 publications
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“…Ruiz‐Lopez and Fasano acknowledge that other elements of hyperkinetic movements (e.g., chorea/stereotypies) are sometimes seen in conjunction with dystonia. We agree with this observation, though raise the note of caution (as have other researchers), that presentations with strongly choreic features, for example, raise the possibility of specific diagnoses (e.g., anti‐NMDAR‐encephalitis or Sydenham's chorea) . The specific management of these conditions differs from SD, and their individual recognition should be promoted.…”
supporting
confidence: 79%
See 1 more Smart Citation
“…Ruiz‐Lopez and Fasano acknowledge that other elements of hyperkinetic movements (e.g., chorea/stereotypies) are sometimes seen in conjunction with dystonia. We agree with this observation, though raise the note of caution (as have other researchers), that presentations with strongly choreic features, for example, raise the possibility of specific diagnoses (e.g., anti‐NMDAR‐encephalitis or Sydenham's chorea) . The specific management of these conditions differs from SD, and their individual recognition should be promoted.…”
supporting
confidence: 79%
“…Having recently reviewed the management of status dystonicus (SD) in childhood, we applaud the review by Ruiz‐Lopez and Fasano and call to improve the precision of this term. We agree that the lack of a robust standardized definition is a barrier to establishing its epidemiology and optimal management.…”
mentioning
confidence: 99%
“…21 Our cases suggest that ATP1A3 mutations should be considered in early onset generalized dystonia especially when combined with intellectual disability and paroxysmal movement disorders.…”
Section: Discussionmentioning
confidence: 75%
“…• Generalized dystonia who may experience dystonic storm (status dystonicus). [10][11][12][13][14] • At this time, there is no evidence that other groups of patients are at risk for life-threatening symptoms from DBS cessation, but this could change with the course of time. • Though not life-threatening per se, severe symptoms upon DBS cessation due to battery depletion 15 should be evaluated for urgent surgery on a case-by-case basis, and in an effort to minimize hospitalizations or evaluations in the emergency department.…”
Section: Dbs For Movement Disorders Recommendationsmentioning
confidence: 99%
“…Warn the patient about a very rare side effect of status dystonicus (persistent severe spasms with difficulty in breathing/swallowing, metabolic dysfunction, pain), 12 and that if stimulation stops, they may need some oral medications (see below).…”
Section: Patients With Generalized Dystonia (Both Genetic/idopathic Amentioning
confidence: 99%