Status dystonicus in childhood
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Abstract
SUMMARY
Background: Status Dystonicus is a rare complication of dystonia. It is a life threatening disorder that needs urgent treatment.The aim of this study is to describe clinical features, management and follow up of children with Status Dystonicus.
Methods: - We conducted a retrospective study over an 8-year period including all patients diagnosed with Status Dystonicus. Clinical characteristics, etiologies and management were analyzed.
Results: - Ten patients were included. Main features of Status Dystonicus were a severe generalized dystonia with vegetative signs. Laryngeal spasm and swallowing disorders were observed in 4 cases. Several treatments such as Levodopa, Anticholinergics, Baclofen, Benzodiazepines and Neuroleptics were tried. Mechanical ventilation was required in 4 cases. Two patients died due to rhabdomyolysis and respiratory failure. Others returned to their pre-Status Dystonicus.
Conclusion : - Status Dystonicus is a life threatening condition that needs an urgent management on an intensive care unit. In fact, patients with Status Dystonicus can develop respiratory failure and metabolic complications.On the basis of our experience, we delineated a therapeutic approach in which the patient with Status Dystonicus needs supportive care, specific therapy of dystonia and intravenous sedative treatment.
Background: Status Dystonicus is a rare complication of dystonia. It is a life threatening disorder that needs urgent treatment.The aim of this study is to describe clinical features, management and follow up of children with Status Dystonicus.
Methods: - We conducted a retrospective study over an 8-year period including all patients diagnosed with Status Dystonicus. Clinical characteristics, etiologies and management were analyzed.
Results: - Ten patients were included. Main features of Status Dystonicus were a severe generalized dystonia with vegetative signs. Laryngeal spasm and swallowing disorders were observed in 4 cases. Several treatments such as Levodopa, Anticholinergics, Baclofen, Benzodiazepines and Neuroleptics were tried. Mechanical ventilation was required in 4 cases. Two patients died due to rhabdomyolysis and respiratory failure. Others returned to their pre-Status Dystonicus.
Conclusion : - Status Dystonicus is a life threatening condition that needs an urgent management on an intensive care unit. In fact, patients with Status Dystonicus can develop respiratory failure and metabolic complications.On the basis of our experience, we delineated a therapeutic approach in which the patient with Status Dystonicus needs supportive care, specific therapy of dystonia and intravenous sedative treatment.
Keywords:
Dystonic storm, childhood, treatment##plugins.themes.academic_pro.article.details##
References
- Vaamonde J, Narbona J, Weiser R,et al. Dystonic storms: a practical management problem. Clin Neuropharmacol 1994;17:344 -347.
- Opal P, Tintner R, Jankovic J, et al. Intrafamilial phenotypic variability of the DYT1 dystonia: from asymptomatic TOR1A gene carrier status to dystonic storm. Mov Disord 2002;17:339-45.
- Mariotti P, Fasano A, Contarino MF, et al. Management of status dystonicus: our experience and review of the literature. Mov Disord 2007; 22:963-8.
- Manji H, Howard RS, Miller DH, et al. Status dystonicus: the syndrome and its management. Brain 1998; 121:243-52.
- Jankovic J, Penn AS. Severe dystonia and myoglobinuria. Neurology 1982; 32:1195-7.
- Allen NM1, Lin JP, Lynch T, King MD. Status dystonicus: a practice guide. Dev Med Child Neurol. 2014 Feb;56(2):105-12.
- Grosso S, Verrotti A, Messina M, et al. Management of status dystonicus in children. Cases report and review. Eur. J. Paediatr. Neurol 2012; 16:390-5.
- Fasano A, Ricciardi L, Bentivoglio AR, et al. Status dystonicus: predictors of outcome and progression patterns of underlying disease. Mov Disord 2012; 27: 783-8.
- Baxter P. Status dystonicus: under-recognized and under-treated. Dev Med Child Neurol. 2013 Feb;55(2):99.
- Teive HA, Munhoz RP, Souza MM, et al. Status Dystonicus: study of five cases. Arq Neuropsiquiatr 2005; 63:26-9.
- Marras CE, Rizzi M, Cantonetti L, Rebessi E, et al. Pallidotomy for medically refractory status dystonicus in childhood. Dev Med Child Neurol. 2014;56(7):649-56.