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Status Dystonicus

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

Abstract

Status dystonicus (SD) is a movement disorder emergency thought to be rare, although it is often underrecognized and undertreated. SD was first described in 1982 and defined as a rapidly deteriorating severe dystonia requiring urgent hospitalization. Since then, the definition and phenomenology of this entity, also known as “dystonic storm,” have been better characterized. Thus, a new definition has been proposed for SD which focuses on bulbar/respiratory dysfunction requiring hospital admission due to severe dystonia or other hyperkinetic movements, in the context of a pre-existing dystonia syndrome or in absence of any condition at baseline. This chapter provides a critical overview of SD and will specifically address the epidemiology, associated etiologies, pathophysiological mechanisms, and management of this life-threatening condition.

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Acknowledgments

We would like to thank Sara Breitbart, Carolina Gorodetsky, and George Ibrahim from The Hospital for Sick Children (SickKids), Toronto, ON, Canada, for having provided Fig. 10.1 and Ariane Belzile for editing references.

Funding Sources and Conflict of Interest

This work was partly funded by the Chair in Neuromodulation and Multidisciplinary care at the University of Toronto and University Health Network (A.F.)

Financial Disclosures

IAM has nothing to disclose.

AF received speaker and/or consulting honoraria and/or research support from Abbott, Boston Scientific, Brainlab, Ceregate, Ipsen, and Medtronic.

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Authors and Affiliations

  1. Department of Neurosciences, CHU Sainte Justine, Montreal, QC, Canada

    Inge A. Meijer

  2. Toronto Western Hospital, Movement Disorders Centre, Toronto, ON, Canada

    Alfonso Fasano

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  1. Inge A. Meijer
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  2. Alfonso Fasano
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Correspondence to Alfonso Fasano .

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Editors and Affiliations

  1. Department of Neurology, NYU Langone Health, New York, NY, USA

    Steven J. Frucht

Electronic Supplementary Material

Four children with dystonic storm are shown in this video segment. The first video shows a pediatric case of status dystonic triggered by DBS surgery in a 9-year-old boy with generalized hyperkinetic movements possibly caused by an ADCY-5 mutation. Three patients with DYT-1 dystonia and dystonic storm are shown next. The first young man developed acute marked worsening of his dystonia, with severe generalized spasms involving all four limbs that required hospitalization in the intensive care unit. Despite numerous oral and intravenous medications, his dystonic spasms could not be controlled, and he eventually underwent placement of a baclofen pump (a standard procedure in the late 1990s, but not one that is used today for this entity). The second patient developed paroxysms of unremitting dystonic movements lasting hours, associated with marked sweating, fever, and elevation of CK to 5000. He was subsequently treated with deep brain stimulation of the globus pallidus interna. The third patient is afflicted with severe generalized dystonia, which temporarily worsened after a DBS stimulator became infected. A spectacular improvement in his dystonia was achieved months after bilateral DBS of the GPi (MP4 334351 kb)

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Meijer, I.A., Fasano, A. (2022). Status Dystonicus. In: Frucht, S.J. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana, Cham. https://doi.org/10.1007/978-3-030-75898-1_10

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  • DOI: https://doi.org/10.1007/978-3-030-75898-1_10

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  • Publisher Name: Humana, Cham

  • Print ISBN: 978-3-030-75897-4

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