Recent Insights on Pediatric Pseudotumor Cerebri Syndrome Pathophysiology: From the “Unifying Neuroendocrine Perspective” to the “Integrated Bioenergetic–Hormonal Mechanism”

Abstract

Pseudotumor cerebri syndrome (PTCS) is a condition characterized by raised cerebrospinal fluid pressure in the setting of evidently normal brain parenchyma and stable composition of the cerebrospinal fluid. The pathophysiology of this syndrome is complex and not yet fully understood. Idiopathic intracranial hypertension (IIH), which is the primary (idiopathic) form of PTCS, generally affects adult obese females in their childbearing age. In children, PTCS is more commonly (compared with adults) associated with concomitant comorbidities (e.g., chronic medical conditions, endocrinopathies, treatment with tetracyclines, recombinant growth hormone, and thyroid supplementation). Together, these likely etiologic factors appear to lack a unifying pathophysiologic mechanism; however, the most recent research in this field is aiming to identify a common clue among most (if not all) secondary associations of pediatric PTCS. This has led in the last few years to a deeper understanding of the interplay among metabolic and endocrine derangements in PTCS, providing new insights into its pathophysiology.