Rethinking status dystonicus: A welcome start to a challenging problem

Abstract

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Prestatus and status dystonicus in children and adolescents

“…anti‐N‐methyl‐D‐aspartate receptor encephalitis in presentations dominated by choreic features). Other open questions include the role of fever, rhabdomyolysis, and metabolic disturbances as supporting criteria to define status dystonicus—as included in the DSAP scoring system . Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way.…”

“…Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way. The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus …”

“…29 These authors suggested including all potentially lifethreatening hyperkinetic movement disorder emergencies in the broad category of status dyskineticus, regardless of the specific phenomenology or pre-existing condition (if any). However, Lumsden and Allen 30 questioned this definition, arguing that hyperkinetic crises without dystonia should be differentiated from status dystonicus, because of the possibility of a specific underlying condition whose recognition and appropriate management should be emphasized (e.g. anti-N-methyl-D-aspartate receptor encephalitis in presentations dominated by choreic features).…”

“…Other open questions include the role of fever, rhabdomyolysis, and metabolic disturbances as supporting criteria to define status dystonicus-as included in the DSAP scoring system. 29,30 Nevertheless, the main critical issue remains unsolved; namely the duration (or number of episodes) of dystonic exacerbations required to define status dystonicus in an objective and replicable way. The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus.…”

“…The current lack of a robust standardized definition is probable the main barrier to prompt recognition and optimal management of status dystonicus. 30 Further limitations mainly exist in the retrospective design and the small sample size. First, during the study period no routine protocol for dystonia exacerbation management was applied at our institution, making assessment and management of our cohort highly heterogeneous.…”