High Dose Clonidine as a Novel Therapy for Symptomatic Status Dystonicus in a Pediatric Patient
Objective:
To highlight the role of high dose clonidine in the treatment of refractory status dystonicus in children.
Background:
Status dystonicus (SD) is a movement disorder emergency characterized by involuntary sustained dystonia that can result from infections, traumatic brain injuries, hypoxia, or toxins. If under-treated, it can result in respiratory failure and end organ dysfunction. Therefore, it is crucial for SD to be identified and treated early with both supportive care and pharmacologic agents. Common pharmacologic agents include dopamine receptor blockers, baclofen, anticholinergics, and benzodiazepines. However, some patients remain refractory to standard treatments.
Design/Methods:
Case report and literature review.
Results:
A 16-month-old female presented to an outside facility in febrile status epilepticus of >90 minutes duration. She suffered a brief cardiorespiratory arrest, was quickly resuscitated, and then subsequently suffered a second cardiorespiratory arrest and was transferred to our facility. Due to concern for status dystonicus, she was treated with a combination of Baclofen, Diazepam, Clonazepam, Amantadine and Trihexyphenidyl with minimal improvement. MRI showed acute bilateral globus pallidus injury. Extensive infectious, autoimmune, and genetic testing was overall unremarkable. After several days of continued refractory SD with maximized medication therapy, high-dose enteral clonidine was started, increasing dosing over the course of the week from 6 mcg/kg/day to 33 mcg/kg/day, which correlated with significant improvement in symptoms after reaching maximal dose. As she continued to improve with high dose clonidine, she was able to be weaned off of several medications and discharged to acute inpatient rehab.
Conclusions:
Status dystonicus can be a difficult condition to treat, as there are limited pharmaceutical treatment options in pediatric patients. Studies in adult populations have shown effectiveness of high dose clonidine, yet this has not been well described in children. This case suggests that high dose oral clonidine may be a safe and effective treatment option for refractory SD in children.