Status Dystonicus in Children: A Cross-Sectional Study and Review of Literature.

Background

Status dystonicus is a life-threatening, underrecognized movement disorder emergency. We aimed to ascertain the etiology, clinical presentation, complications, and outcomes of status dystonicus in children and reviewed the literature for similar studies.

Methods

Records of all children aged <14 years admitted to a single center with status dystonicus between 2014 and 2018 were reviewed.

Results

Twenty-four children (75% male) were identified with status dystonicus. The annual incidence rate was 0.05 per 1000 new admissions <12 years of age. The mean age at presentation was 6.3 ± 3.6 years. Median duration of hospital stay was 10.5 days (interquartile range 5-21.7). The severity of dystonia at presentation was grade 3 (n = 9; 37.5%) and 4 (n = 9; 37.5%). The most common triggering factor was intercurrent illness/infection (n = 18; 75%). The most common underlying etiologies were cerebral palsy (n = 8; 33.3%), complicated tubercular meningitis (n = 3; 12.5%), and mitochondrial disorders (n = 3; 12.5%). Basal ganglia involvement was seen in 15 cases (62.5%). Respiratory and/or bulbar compromise (n = 20; 83.3%) and rhabdomyolysis (n = 15; 62.5%) were most commonly seen. Oral trihexyphenidyl (96%) followed by oral or intravenous diazepam (71%), oral baclofen (67%), and midazolam infusion (54%) were the most common drugs used. Clonidine was used in 33% cases, without any significant side effects. Three children died owing to refractory status dystonicus and its complications; the mortality rate was 12.5%. Conclusion Status dystonicus is a neurologic emergency in children with severe dystonia, with significant complications and a high mortality rate. Static and acquired disorders are more common than heredo-familial causes. Identification and treatment of infection in children is important as the majority of cases are triggered by an intercurrent infection.