J Pediatr Neurol 2015; 13(01): 011-015
DOI: 10.1055/s-0035-1555146
Review Article
Georg Thieme Verlag KG Stuttgart · New York

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

Vincenzo Salpietro
Department of Pediatric Neurology, Chelsea and Westminster Hospital, Imperial College, London, United Kingdom
Department of Pediatrics, University of Messina, Messina, Italy
Martino Ruggieri
Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
Daniela Concolino
Department of Pediatrics, Magna Graecia University, Catanzaro, Italy
Conrad E. Johanson
Department of Neurosurgery, Brown University, Providence, Rhode Island, United States
Karl T. Weber
Division of Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis, Tennessee, United States
› Author Affiliations
Further Information

Publication History

12 October 2014

02 January 2015

Publication Date:
13 July 2015 (online)


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.