Introduction: Status Dystonicus (SD), the most severe expression of severe dystonias, is caused by abnormal signaling and neurotransmission between the globus pallidus internus (GPi), other brainstem structures, cerebellum and sensorimotor cortex. Primary SD occurs in monogenetic, neurometabolic or degenerative entities, while secondary SD is acquired. SD, which can recur, is precipitated by infection, pain, sleep disruption, discontinuation syndromes, including intrathecal baclofen (ITB) pump failure, and other stressors. Severe pain and organ dysfunction are observed. Mortality is high (~12%), due to complications of renal and other organ failure. Many children recover to a lower functional “baseline”. Methods: Meta analyses, topical reviews on medical and surgical aspects of care, clinical practice guidelines and case studies sourced using the PubMed and Embase search engines using the terms “dystonia” and “status dystonicus” between years 2020 and July 2025, were reviewed. Results: More than 300 articles have been devoted to severe dystonias in the last 5 years. There is broad agreement, with some regional variation, on the medical management, with antipyretics, analgesia, sedation (lorazepam), anticholinergics (trihexyphenidyl), muscle relaxants (primarily baclofen and benzodiazepines), alpha-2-adrenergic agonists (clonidine, tizanidine), and the vesicular monoamine transporter (VMAT2) tetrabenazine. Titratable infusions, including midazolam, dexmedetomidine, opioids, and pentobarbital may be needed. Dialysis and mechanical ventilation with neuromuscular blockade may be required in refractory SD associated with rhabdomyolysis or respiratory failure. Interventional approaches such as chemodenervation using botulinum toxin or phenol, ITB pump revision or placement, rhizotomy, and deep brain stimulator (DBS) installation and titration, and pallidotomy are safe and effective, even in young children with SD in PICU. Success with non-invasive lesioning of the GPi with MRI-guided high intensity focused ultrasound has been described. Conclusions: Prevention of SD episodes is key. A multidisciplinary, systematic approach to care is essential, with the input of medical, surgical and other subspecialists. Early consideration of interventional approaches in critically ill children with SD is warranted.
Okhuysen-Cawley et al. (Sun,) studied this question.