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 clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.

Rethinking status dystonicus: A welcome start to a challenging problem

“…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.…”

“…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.…”

Generalized Dystonia and Paroxysmal Dystonic Attacks due to a Novel ATP1A3 Variant

“…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.…”

Letter: Evaluation and Surgical Treatment of Functional Neurosurgery Patients With Implanted Deep Brain Stimulation and Vagus Nerve Stimulation Pulse Generators During the COVID-19 Pandemic

“…• 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.…”

“…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).…”