Objective: We present 20 cases of pediatric status dystonicus at a major pediatric center and describe their clinical course.

Background: Status dystonicus (SD) is a rare, life-threatening neurological emergency characterized by continuous, severe episodes of generalized dystonic spasms. Etiology, pathogenesis, diagnosis, and treatment for SD are poorly understood.

Method: Charts were reviewed retrospectively of 20 patients admitted to a single pediatric center for the diagnosis of SD (ICD9/10 codes) from 2015-2019.

Results: Majority of patients were male (60%,N=12). Dystonia age of onset ranged from 0-15 years and consisted of acquired (70%,N=14) and genetic (30%, N=6) etiologies. Most patients received medications for dystonia prior to admission, including benzodiazepines (75%,N=15) and/or antispasmodics (70%,N=14). Patients were an average of 8.7 years old at time of admission (range 3mo-20yr). Most patients (75%,N=15) were admitted to ICU and stayed an average of 19.7 days. Triggers for SD onset included infection (55%, N=11), medication withdrawal (20%,N=4), or none (20%,N=4).  3 patients (15%) diagnosed with SD met formal criteria of SD with metabolic decompensation while the rest were diagnosed as SD due to worsening and/or painful dystonia. All patients received IV and/or oral treatments, majority being benzodiazepines (95%,N=19) for both. 2 patients required neuromuscular blockade. Most patients required combination treatment (90%,N=18). Some patients required invasive treatments, including chemodenervation (15%,N=3) and intrathecal baclofen pump placement (5%,N=1). On average, any clinical improvement was noted after 0.8 days, while meaningful clinical improvement was seen on average 2.6 days after the definitive intervention. The definitive intervention along with treatment of underlying trigger was introduction/increase of benzodiazepines (60%,N=12), antispasmodics (20%,N=4), alpha-2 agonists (25%,N=5), chemodenervation (5%,N=1), and intrathecal baclofen pump (5%,N=1).

Conclusion: This study describes the clinical course of 20 pediatric SD cases. History of generalized dystonia, often in the setting of infection and even with concurrent ongoing dystonia management, may serve as increased risk of SD. Most patients require multiple treatment modalities for management of this complex condition; benzodiazepines seem to be the most definitive intervention within this cohort, representing a reasonable first line approach.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-characteristics-and-management-practices-of-pediatric-status-dystonicus/