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Título

REFRACTORY STATUS DYSTONICUS: CASE REPORT

Apresentação do caso único

Case report: A male child aged 2 years and 6 months, cerebral palsy, microcephaly secondary by Cytomegalovirus and refractory epilepsy, was hospitalized to investigate seizures and fever associated with hepatomegaly and elevated transaminases, was diagnosed with non-infectious hepatitis and received antibiotic therapy. His anticonvulsants were suspended due to hepatitis, but it worsened his epileptic seizures. The patient developed dystonic posture with a significant increase of creatine phosphokinase (CPK), the first CPK was 2,322 IU/L (reference up to 190 IU/L), being diagnosed with dystonic status (DS) and initially treated with continuous midazolam, baclofen and biperiden, and transferred to a pediatric intensive care unit. He was refractory to treatment, presented a worsening CPK (10,759 IU/L), and it was necessary to associate neuromuscular blocker (rocuronium) and clonazepam. Also, dystonia specific drugs were elevated: biperiden at 20 mg/day, baclofen at 24 mg/day, trihexyphenidyl at 6 mg/day, and levodopa 1 mg/kg/day was associated as well. The patient needed mechanical ventilation due to the worsening respiratory pattern and neurological alteration. He evolved with progressive clinical improvement, gradual reduction in muscle damage enzymes, CPK coming to 296 IU/L, and was discharged after 19 days of hospitalization (remained on mechanical ventilation for 10 days) with a referral for outpatient follow-up and adjusted medication doses.

Discussão

Discussion: Dystonic status is a rare and potentially fatal movement disorder emergency characterized by generalized muscle contractions in dystonic patients and by increases in CPK of over 1,000 IU/L. It is considered refractory when, despite the specific drugs for dystonia being at optimized doses, weaning from sedative agents remains unsuccessful, making it necessary to consider early administration of baclofen and other associated agents for control. Severe generalized muscle spasms can cause respiratory impairment and severe metabolic disturbances such as rhabdomyolysis and acute renal failure. Treatment in refractory cases includes identification of the disease and its precipitants, supportive measures, sedation and early administration of optimized drugs and adjuvants.

Comentários finais

Final comments: Dystonic status is a rare pediatric emergency, a rapid recognition and stabilization of the patient can help minimize morbidity and mortality. When refractory, the optimization of control measures must be fundamental.

Referências

1. Sanger TD, Chen D, Fehlings DL, et al. Definition and classification of hyperkinetic movements in childhood. Mov Disord 2010; 25: 1538–49.
2. Monbaliu E, Ortibus E, De Cat J, et al. The Dyskinesia Impairment Scale: a new instrument to measure dystonia and choreoathetosis in dyskinetic cerebral palsy. Dev Med Child Neurol 2012; 54: 278–83.
3. Albanese A, Bhatia K, Bressman SB, et al. Phenomenology and classification of dystonia: a consensus update. Mov Disord 2013; 28: 863–73

Palavras Chave

Refractory status dystonicus; dystonic storm; dystonia