The first Jacobson Lecture. Familial idiopathic intracranial hypertension

J Neuroophthalmol. 2008 Dec;28(4):337-47. doi: 10.1097/WNO.0b013e31818f12a2.

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.

Publication types

  • Lecture

MeSH terms

  • Adolescent
  • Adult
  • Cerebrospinal Fluid Pressure / physiology
  • Cohort Studies
  • Comorbidity
  • Female
  • Genetic Predisposition to Disease
  • Genetic Testing
  • Headache / etiology
  • Humans
  • Inheritance Patterns / genetics
  • Male
  • Middle Aged
  • Obesity / epidemiology
  • Papilledema / etiology
  • Prevalence
  • Pseudotumor Cerebri / congenital
  • Pseudotumor Cerebri / diagnosis*
  • Pseudotumor Cerebri / epidemiology
  • Young Adult