Deep Brain Stimulation in Pediatric Status Dystonicus: A Case Series Investigation on Rapid Activation & ICU Stays

Purpose: Status dystonicus (SD) is a life-threatening movement disorder characterized by a sudden and severe worsening of generalized dystonia, often complicated by rhabdomyolysis with associated metabolic and respiratory consequences, including death. Deep brain stimulation (DBS) is a potential treatment modality for pharmaco-resistant SD (PRSD) which, despite growing interest in its use, requires further research into ideal patient selection and optimum timing for device activation. Timely deployment of this therapy can lead to shorter ICU stays and better outcomes for patients by reducing the need for additional invasive procedures and escalating pharmaceutical therapy.

Methods: This is a retrospective case series of 6 consecutive pediatric SD patients who were admitted to the PICU at Cook Children’s Medical Center between October 2010 and March 2022. Following their admission, these patients were treated with DBS and were all subsequently discharged from the ICU. We examined ICU time between patients who underwent rapid activation of DBS systems and those who delayed the start of this intervention.

Results: Patients with acquired forms of dystonia demonstrated a mean ICU stay of 58 following DBS activation, and patients with genetic dystonias had a mean stay of 18 days after the procedure. Rapid deployment of DBS systems and two stage procedures were associated with more positive patient outcomes,

Conclusions: SD is a condition with a wide range of clinical presentations and responsiveness to treatment. This unpredictability and variation in disease outcomes emphasizes the need for further controlled studies to clarify optimal timing and patient selection for DBS. Ethical and parental concerns regarding the recruitment of children into clinical trials remains a major limiting factor to this goal, making meaningful clinical reports and data sharing between care centers essential in advancing the collective understanding of this condition and in optimizing patient care.