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STATUS DYSTONICUS IN CHILDREN. CASE REPORTS.
Last modified: 2014-04-03
Abstract
INTRODUCTION: Status dystonicus (SD) is a medical emergency leading to a significant morbidity and mortality. It can affect either patients with primary or secondary dystonia. SD is characterized by episodes of generalized muscle contraction sustained and refractory to medical treatment. Usually is possible to identify triggers as fever, infections or abrupt discontinuation of treatment. From a therapeutic point of view there are no evidence-based management guidelines in SD.
OBJECTIVE: we report on four patients with status dystonicus describing their clinical manifestations, treatments and evolution.
MATERIALS AND METHODS: we reviewed medical records of patients admitted to our department with a diagnosis of status dystonicus from January 2009 to June 2012.
RESULTS: we present four male patients with secondary dystonia with usual drug treatment. Range of age at diagnosis was 4-13 years. Fever was the most frequent trigger. Three patients required intravenous benzodiazepines without mechanical ventilation needed; two of which showed unfavorable response, and required implementation of baclofen intrathecal infusion pump, achieving the resolution of the SD and decline in basic medication. Only one patient was managed optimizing oral medication.
CONCLUSION: Status dystonicus is a very difficult therapeutic management condition. Early use of surgical techniques, such as baclofen intrathecal infusion pump should be considered before treatment failure or relapse with intravenous benzodiazepines. We emphasize the importance of preventing and controlling triggers. There is an urgent need to develop evidence-based treatment guidelines.
OBJECTIVE: we report on four patients with status dystonicus describing their clinical manifestations, treatments and evolution.
MATERIALS AND METHODS: we reviewed medical records of patients admitted to our department with a diagnosis of status dystonicus from January 2009 to June 2012.
RESULTS: we present four male patients with secondary dystonia with usual drug treatment. Range of age at diagnosis was 4-13 years. Fever was the most frequent trigger. Three patients required intravenous benzodiazepines without mechanical ventilation needed; two of which showed unfavorable response, and required implementation of baclofen intrathecal infusion pump, achieving the resolution of the SD and decline in basic medication. Only one patient was managed optimizing oral medication.
CONCLUSION: Status dystonicus is a very difficult therapeutic management condition. Early use of surgical techniques, such as baclofen intrathecal infusion pump should be considered before treatment failure or relapse with intravenous benzodiazepines. We emphasize the importance of preventing and controlling triggers. There is an urgent need to develop evidence-based treatment guidelines.
Keywords
status dystonicus
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