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Dystonic Storm: a Practical Clinical and Video Review Pichet Termsarasab* and Steven J Termsarasab and Frucht Journal of Clinical Movement Disorders (2017) 4:10 DOI 10.1186/s40734-017-0057-z REVIEW Open Access Dystonic storm: a practical clinical and video review Pichet Termsarasab* and Steven J. Frucht Abstract Dystonic storm is a frightening hyperkinetic movement disorder emergency. Marked, rapid exacerbation of dystonia requires prompt intervention and admission to the intensive care unit. Clinical features of dystonic storm include fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failure. It is critical to recognize early and differentiate dystonic storm from other hyperkinetic movement disorder emergencies. Dystonic storm usually occurs in patients with known dystonia, such as DYT1 dystonia, Wilson’s disease and dystonic cerebral palsy. Triggers such as infection or medication adjustment are present in about one-third of all events. Due to the significant morbidity and mortality of this disorder, we propose a management algorithm that divides decision making into two periods: the first 24 h, and the next 2–4weeks.During the first 24 h, supportive therapy should be initiated, and appropriate patients should be identified early as candidates for pallidal deep brain stimulation or intrathecal baclofen. Management in the next 2–4 weeks aims at symptomatic dystonia control and supportive therapies. Keywords: Dystonic storm, Status dystonicus, Dystonia, Treatment, Early recognition, Deep brain stimulation Introduction Review Dystonic storm is a malignant hyperkinetic movement Recognition of dystonic storm disorder emergency in which rapid deterioration of dys- In addition to severe generalized dystonia, symptoms and tonia requires emergent intervention [1]. Jankovic and signs of dystonic storm include fever, tachycardia, tachyp- Penn first described the disorder in 1982 in an 8-year-old nea or respiratory change, hypertension, sweating and auto- boy with dystonia musculorum deformans who developed nomic instability. Dystonia can be tonic (i.e. sustained rapid marked worsening of dystonia, elevated serum creat- posturing) or phasic (i.e. irregular jerking). The largest ine kinase (CK) and myoglobinuria [2]. This entity was study of 89 episodes of storm revealed that most were tonic later referred to as “desperate dystonia” [3] or “dystonic (69%), and phasic storms occurred more commonly in fe- storm” [4]. In 1998, Manji described a series of 12 patients males and patients with secondary dystonia [6]. Other with “status dystonicus”, and their definition has been movement disorders such as chorea, ballism or myoclonus referenced in subsequent reports [5–7]. may accompany dystonia. Bulbar impairment such as dys- In this review, we summarize the clinical features of arthria, dysphagia and respiratory failure are common, and dystonic storm, review its differential diagnosis and these require close monitoring in the intensive care unit. pathophysiology, and present a practical guideline for Pain is also common, and often requires aggressive symp- the treating clinician, aimed at identifying patients who tomatic control. Laboratory findings include leukocytosis, are surgical candidates and managing those who are not elevated serum CK, high C-reactive protein, myoglobine- with medication and supportive care. mia, and myoglobinuria that may be severe enough to lead to renal failure and acidemia. Differential diagnosis of dystonic storm Several conditions may mimic dystonic storm, presenting * Correspondence: [email protected] Movement Disorder Division, Department of Neurology, Icahn School of with hyperthermia, elevated serum CK, and/or muscle ri- Medicine at Mount Sinai, 5 East 98th St, New York, NY 10029, USA gidity. It is important to differentiate dystonic storm from © The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Termsarasab and Frucht Journal of Clinical Movement Disorders (2017) 4:10 Page 2 of 8 other hyperkinetic movement disorder emergencies [8] symptoms including fever, hyperCKemia and leukocytosis. (Table 1 and Additional file 1: Video segment 1). The phe- Various neurotransmitter systems including dopaminergic, nomenology of the underlying movement disorder, associ- serotoninergic, GABAergic, glycinergic and glutamatergic ated neurological symptoms and signs, age of the patient, systems are likely involved in these disorders. history of triggers, and time course are helpful clues. Compared to other movement disorder emergencies, Dystonic storm: clinical features dystonic storm typically occurs in the pediatric age group. Dystonic storm usually occurs in patients who are Triggers may or may not be present (see below), and already known to have dystonia, and are usually followed storm usually develops quickly over hours to several days. in movement disorders clinic. De novo presentation of a patient in dystonic storm is rare. Mean prior dystonia Mechanism of dystonic storm duration is 6 years, with an average age at presentation The mechanism and neurotransmitter systems involved of 14 years [6]. Dystonic storm tends to occur in patients in dystonic storm remain unknown. We present a hypo- with severe or poorly controlled dystonia at baseline thetical framework for conceptualizing possible mecha- [2, 9], and one-third of events are unprovoked [6, 10]. nisms of various storm conditions in Fig. 1. In patients Among provoked events, triggers include infection with underlying dystonia, increased dystonia severity (50%) and medication change (30%). Examples of the may result from increased pallidal output. Intrathecal latter include ITB withdrawal [11, 12], reduction or baclofen (ITB) withdrawal, autoimmune disorders such withdrawal of other dystonia medications or initiation as stiff person syndrome or progressive encephalomyeli- of D-penicillamine in Wilson’s disease [13, 14]. Other tis with rigidity and myoclonus, and toxic etiologies examples include introduction of clonazepam (para- (such as tetanus) may lead to spinal-mediated overactiv- doxically it can also be used as treatment of dystonic ity. Decoppering in Wilson’s disease (especially with storm) [1], initiation of zinc in Wilson’sdisease[15] D-penicillamine), crisis in biotin-thiamine-responsive or zolpidem discontinuation in Parkinson’sdisease basal ganglia disease, acute osmotic demyelinating syn- [16]. Other provoking factors include surgical proce- drome and serotonin syndrome may be mediated through dures and deep brain stimulation (DBS) failure [17, 18]. the brainstem. Neuroleptic malignant syndrome from As DBS has become more widespread, there have dopaminergic blockade may lead to hypothalamic storm. been more reports of dystonic storm from DBS fail- The mechanism of malignant hyperthermia likely origi- ure, especially due to battery issues or at the end of nates peripherally, as evidenced by associated muscle dis- battery life [19]. orders such as myopathies secondary to ryanodine In some patients a prodrome of dystonic storm may receptor (RYR1) mutations. Neuroleptic malignant syn- occur, where dystonia is worsened from baseline but not as drome, serotonin syndrome and malignant hyperthermia severe as in true storm (Fig. 2 and Additional file 2: Video may share a final common pathway, characterized by an segment 2). Once true storm begins, it usually lasts 2–4 increase in spinal-mediated tone and associated signs and weeks with gradual recovery. However even with treatment, Table 1 Differential diagnosis of dystonic storm Entity Age Trigger Time Movement disorder Other neuro Altered mental Autonomic course phenomenology signs status instability Dystonic storm P +/− Hours-Days Dystonia +/− chorea −− + Choreic storm P-YA +/− Hours-Days Chorea −− − Oculogyric crisis All +++ Acute Dystonia Oculogyria − +/− Neuroleptic malignant syndrome A +++ Days-Weeks Parkinsonism − ++ Serotonin syndrome All +++ Hours-Days Myoclonus − ++ Lethal catatonia All +/− Hours-Days Parkinsonism − ++ Malignant hyperthermia All +++ Acute −−−− Drug intoxication All +++ Acute − Psychosis + +/− Intrathecal baclofen withdrawal P-YA +++ Acute-Hours −−++ Delirium tremens A +++ Acute Myoclonus Psychosis + + Autoimmune encephalitis (e.g. anti-NMDA) P-YA − Days-Weeks Chorea Psychosis + + This table provides the list of differential diagnosis of dystonic storm. Useful distinguishing features include age group (pediatric, young adult, adult and all), presence or absence of triggering factors (number of “+” sign correlates with stronger relationship; “+/−” represents inconsistent correlation; “−” represents no correlation), time course, movement disorder phenomenology, associated neurological signs, altered mental status and autonomic instability Abbreviations: P pediatric, YA young adults, A adults, All all age groups Termsarasab and Frucht Journal of Clinical Movement Disorders (2017) 4:10 Page 3 of 8 Fig. 1 Pathophysiology of movement disorder emergencies (“storms”) including dystonic storm. This figure demonstrates
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