Dystonic storm: a practical clinical and video review

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 2–4 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.

Rethinking status dystonicus

“…So far, no study has specifically addressed the pathophysiology of SD. It has been postulated that in patients with underlying dystonia, increased spasms severity may result from an increased pallidal output . A recent review has highlighted the similarities between epilepsy and paroxysmal dyskinesias, including their episodic nature, triggering factors, and the therapeutic response to the same drugs .…”

“…Overall, neurosurgery is the most successful strategy (effective in 33.7% of episodes) . The most recent proposed treatment algorithm suggested a management that divides decision making into the following 2 periods: the first 24 hours, when supportive therapy should be initiated and appropriate patients should be identified early as candidates for pallidal DBS or IBT, and the subsequent 2 to 4 weeks, when the aim is the symptomatic control of dystonia and supportive therapies …”

Deep brain stimulation treated dystonia‐trajectory via status dystonicus

“…Our series is the experience of a single center reporting on SD, with the longest follow‐up of a cohort, after SD. This study shows that SD is a lifetime threat . The spectrum of disorders that produce SD is large.…”

“…This study shows that SD is a lifetime threat. 6,9,10 The spectrum of disorders that produce SD is large. Frequently associated neurological signs and significant morphological alterations support the concept of network-level impairment, including cortical involvement, 11 and explain the complexity of dystonic conditions and the risk of progression into SD.…”

Off‐period status dystonicus in Parkinson’s disease treated with deep brain stimulation: A case report

“…The limited therapeutic efficacy of anti‐dystonia drugs and the potential risk of mortality necessitate immediate implementation of emergency procedures for status dystonicus . Although the reported cases of status dystonicus are limited, the efficacy of early, aggressive treatment with pallidal DBS has been suggested .…”