Familial occurrence of idiopathic intracranial hypertension

Ainat Klein*, Gad Dotan, Anat Kesler

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


Background: Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology. Its occurrence in the general population is 1/100,000, and 20/100,000 among overweight women of childbearing age. Familial occurrence is reportedly uncommon and not well-characterized. Objectives: To describe a familial association with IIH. Methods: We conducted a retrospective chart review of all familial cases of IIH examined in the neuro-ophthalmology clinic of our medical center between January 2006 and June 2013. Results: Of a total of 520 patients with IIH, 15 had other family members with IIH (from seven different families). The family relation was a mother and daughter in two families, a brother and sister in four families, and an aunt and two first-degree cousins in the seventh family. Symptoms, course of disease, and risk factors were similar among the relatives of all seven families, except for the age at diagnosis, which was different in one family. All of the adult patients of six families were obese (body mass index 25–35 kg/m2), and all of the members of the other family were morbidly obese. There was no association between other systemic risk factors and IIH. Conclusions: IIH occurrence within a family is more common than previously believed, and its incidence in families is more common than in the general population. The clinical course appears to be similar in family members. Our findings suggest a genetic predisposition. Further investigation of familial cases may yield useful information on the pathogenesis and genetic nature of this condition.

Original languageEnglish
Pages (from-to)557-560
Number of pages4
JournalIsrael Medical Association Journal
Issue number9
StatePublished - Sep 2018


  • Familial occurrence
  • Genetic predisposition
  • Idiopathic intracranial hypertension (IIH)
  • Papilledema
  • Pseudotumor cerebri (PTC)


Dive into the research topics of 'Familial occurrence of idiopathic intracranial hypertension'. Together they form a unique fingerprint.

Cite this