Clinical characteristics, diagnostic challenges, and outcome of paroxysmal sympathetic hyperactivity in pediatric patients: a retrospective cohort study in a tertiary hospital setting.

Introduction: Paroxysmal Sympathetic Hyperactivity (PSH) is an under-recognized condition in pediatric patients, particularly those with non-traumatic brain injuries, often leading to delayed diagnosis and suboptimal management. The condition features episodic increases in sympathetic nervous system activity, which creates significant diagnostic and therapeutic challenges. This study aims to comprehensively characterize the clinical presentation, diagnostic challenges, and treatment outcomes of pediatric PSH in a tertiary care setting. Additionally, we investigate factors contributing to delayed diagnosis and assess the impact of various clinical and management variables on patient outcomes.

Methods: This retrospective cohort study was conducted at King Abdullah Specialist Children's Hospital (KASCH), Riyadh, Saudi Arabia, encompassing 42 pediatric patients diagnosed with PSH between 2016 and 2023. We extracted comprehensive data from patient records, including demographic profiles, clinical presentations, diagnostic findings, and treatment outcomes. Statistical analyses were employed to identify factors influencing mortality and clinical improvement, including univariate and multivariate regression.

Results: The cohort had a mean age of 6.53 years, with PSH onset typically around 4.19 years. The majority (88.1%) of PSH cases stemmed from non-traumatic causes, notably hypoxic-ischemic encephalopathy (31%). Key clinical features included fever, tachycardia, and dystonia, with a significant rate of initial misdiagnosis (69%). Healthcare providers frequently administer gabapentin as a preventive medication, while they commonly use benzodiazepines for abortive therapy. Clonidine use was associated with a statistically significant reduction in mortality (P < 0.05), whereas delayed diagnosis correlated with poorer clinical outcomes.

Conclusions: PSH in pediatric patients predominantly arises from non-traumatic brain injuries, presenting with nonspecific symptoms that often lead to misdiagnosis. This study underscores the importance of early and accurate diagnosis in improving patient outcomes. Clonidine shows potential as a life-saving intervention in this context. These findings highlight the need for further research to refine diagnostic criteria and optimize treatment strategies for pediatric PSH.