Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: A pilot study

Vincenzo Salpietro, Kshitij Mankad, Maria Kinali, Ashok Adams, Mariella Valenzise, Gaetano Tortorella, Eloisa Gitto, Agata Polizzi, Valeria Chirico, Francesco Nicita, Emanuele David, Anna Claudia Romeo, Carlo Attilio Squeri, Salvatore Savasta, Gian Luigi Marseglia, Teresa Arrigo, Conrad Earl Johanson, Martino Ruggieri

Research output: Contribution to journalArticlepeer-review


Aim: To unravel the potential idiopathic intracranial hypertension (IIH) endocrine-metabolic comorbidities by studying the natural (and targeted drug-modified) history of disease in children. IIH is a disorder of unclear pathophysiology, characterized by raised intracranial pressure without hydrocephalus or space-occupying lesion coupled with normal cerebrospinal fluid (CSF) composition. Methods: Retrospective study (years 2001-2010) of clinical records and images and prospective follow-up (years 2010-2013) in 15 children (11 girls, 4 boys; aged 5-16 years) diagnosed previously as "IIH", according to the criteria for pediatric IIH proposed by Rangwala, at four university pediatric centers in northern, central, and southern Italy. Results: We identified six potential endocrine-metabolic comorbidities including, weight gain and obesity (n=5), recombinant growth hormone therapy (n=3), obesity and metabolic syndrome (n=1), secondary hyperaldosteronism (n=1), hypervitaminosis A (n=1), and corticosteroid therapy (n=1). Response to etiologically targeted treatments (e.g., spironolactone, octreotide) was documented. Conclusions: IIH is a protean syndrome caused by various potential (risk and) associative factors. Several conditions could influence the pressure regulation of CSF. An endocrine-metabolic altered homeostasis could be suggested in some IIH patients, and in this context, etiologically targeted therapies (spironolactone) should be considered.

Original languageEnglish
Pages (from-to)107-115
Number of pages9
JournalJournal of Pediatric Endocrinology and Metabolism
Issue number1-2
Publication statusPublished - Jan 2014


  • Aldosterone
  • Childhood obesity
  • Children
  • Idiopathic intracranial hypertension
  • Magnetic resonance imaging
  • Metabolic syndrome
  • Pseudotumor cerebri
  • Spironolactone

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Pediatrics, Perinatology, and Child Health


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