The first Jacobson Lecture. Familial idiopathic intracranial hypertension

Abstract

Background: Case reports of familial idiopathic intracranial hypertension (IIH) have appeared sporadically and infrequently.

Methods: We reviewed the medical records of all patients with IIH seen at our institution to identify the number of familial cases.

Results: Out of a cohort of 237 patients with IIH, we identified 27 members (25 women and 2 men) from 11 families, with IIH usually self-reported or reported by the index case. In 7 of the 11 families, the relationship was parent to child; in 4, it was sibling. Obesity was present in 85% of the family members.

Conclusions: Familial IIH appears to be more common than reported previously. A systematic evaluation of first-degree relatives may help to identify more cases. A study of the patterns of inheritance and associated co-morbidities may result in better understanding of the genetic issues with this disorder.