Status dystonicus due to internal pulse generator depletion in a patient with primary generalized dystonia

Sobstyl, Michał; Ząbek, Mirosław; Kmieć, Tomasz; Sławek, Jarosław; Budohoski, Karol P.

doi:10.1002/mds.25553

Cited by 16 publications

(12 citation statements)

References 9 publications

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“…With the exception of decompensated calcium metabolism in hypoparathyroidism, the triggers recognized after 2012 have confirmed those previously reported, particularly the role of infection . With the expanding use of DBS for dystonia patients, more SD related to hardware failure have been reported: 3 children with DYT‐TOR1A and 1 case with tardive dystonia . Furthermore, SD can be rarely triggered by the DBS procedure in children with generalized dystonia (Supporting Information Video 1).…”

Section: Triggerssupporting

confidence: 73%

“…In 2012, the largest SD series was published: 68 patients undergoing 89 SD episodes allowed drawing some conclusion on the clinical expression, management strategies, and outcome of SD . During the past 5 years, isolated case reports and a small case series of SD have been published: a fatal refractory case in ataxia telangiectasia, 2 patients with Sex Determining Region Y‐box 2 (SOX2)‐Anophthalmia syndrome, a child with familial idiopathic hypoparathyroidism, 2 NBIA/DYT‐PANK2 patients, a chromosomopathy (deletion of the long arm of chromosome 22), a DYT/CHOR‐GCDH (glutaric aciduria type I), epileptic encephalopathy, idiopathic bilateral striatal necrosis, 3 idiopathic isolated dystonia, additional dyskinetic JCP patients, a 19‐year‐old boy affected by mental retardation and severe behavior disorder treated with high doses of haloperidol, tardive dystonia acutely worsened by the depletion of deep brain stimulation (DBS) implantable pulse generator, dystonia secondary to kernicterus, a series of 5 DYT‐TOR1A positive (DYT1) patients, and an isolated case report of a DYT‐TOR1A patient with SD as a result of implantable pulse generator depletion …”

Section: Historical Review and Current Definitionsmentioning

confidence: 99%

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Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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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.

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Section: Triggerssupporting

confidence: 73%

Section: Historical Review and Current Definitionsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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“…Longer duration of illness and longer exposure to DBS stimulation were postulated as risk factors in another recent report of DBS withdrawal syndrome . Though cessation of pallidal stimulation is known to precipitate dystonic storm in primary dystonia, we suspect that such a malignant akinetic state may not follow cessation of pallidal stimulation in PD, given that these patients continue to use l ‐dopa . Pallidal stimulation may thus be a more lenient approach in this aspect.…”

Section: Discussionmentioning

confidence: 99%

Malignant Subthalamic Nucleus‐Deep Brain Stimulation Withdrawal Syndrome in Parkinson's Disease

Rajan

Krishnan

Kesavapisharady

et al. 2016

Movement Disord Clin Pract

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Abrupt cessation of STN‐DBS is an under‐recognized cause of life‐threatening akinetic crisis in Parkinson's disease (PD) and can present as a movement disorder emergency. We report on 2 patients who survived severe and prolonged akinetic crisis after abrupt cessation of STN stimulation for PD (malignant STN‐DBS withdrawal syndrome). We discuss the clinical similarities and possible differences in pathophysiology from the akinetic crisis in medically‐treated PD. Although early implantable pulse generator (IPG) replacement is the definitive treatment, medical and economic considerations may preclude early surgery and strategies for medical management assume importance. We reflect upon the socioeconomic concerns surrounding DBS in countries lacking health care coverage and the need for user‐independent monitors and indicators of low IPG battery status.

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“…Other provoking factors include surgical procedures and deep brain stimulation (DBS) failure [17, 18]. As DBS has become more widespread, there have been more reports of dystonic storm from DBS failure, especially due to battery issues or at the end of battery life [19]. …”

Section: Dystonic Storm: Clinical Featuresmentioning

confidence: 99%

Dystonic storm: a practical clinical and video review

Termsarasab

Frucht

2017

J Clin Mov Disord

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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 2–4 weeks. 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s40734-017-0057-z) contains supplementary material, which is available to authorized users.

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Status dystonicus due to internal pulse generator depletion in a patient with primary generalized dystonia

Cited by 16 publications

References 9 publications

Rethinking status dystonicus

Rethinking status dystonicus

Malignant Subthalamic Nucleus‐Deep Brain Stimulation Withdrawal Syndrome in Parkinson's Disease

Dystonic storm: a practical clinical and video review

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