Status dystonicus in childhood

Dystonia is a common paediatric neurological condition. At its most severe, dystonia may lead to life-threatening complications, a state termed status dystonicus. This review provides an update on the definition, causes, management and outcome of childhood status dystonicus.

High-quality studies in childhood status dystonicus are lacking, though an increasing number of case series have been published. Status dystonicus appears to occur more frequently in children compared with adults, with a clear precipitant identified in around two-thirds of cases. Although febrile illness remains the commonest trigger for status dystonicus, unplanned interruption to deep brain stimulation (DBS) is increasingly reported as a precipitant. In parallel with this, neurosurgical intervention for status dystonicus appears to have become more widely used, though optimum timing and patient selection remains unclear. In most cases, a multistaged approach is required; we propose an 'ABCD' approach - Addressing precipitants, Beginning supportive measures, Calibrating sedation and Dystonia specific medications. Outcomes following status dystonicus appear to have slightly improved in recent years, potentially as a consequence of increasing use of DBS, though mortality has remained around 10%.

Future work is needed to inform evidence-based guidelines for the management of status dystonicus. One of many pressing questions is the precise indication, and timing of interventions such as DBS.