Conflict of Interest: None.
Pseudotumor Cerebri Pathophysiology
Article first published online: 16 JAN 2014
© 2014 American Headache Society
Headache: The Journal of Head and Face Pain
Volume 54, Issue 3, pages 445–458, March 2014
How to Cite
McGeeney, B. E. and Friedman, D. I. (2014), Pseudotumor Cerebri Pathophysiology. Headache: The Journal of Head and Face Pain, 54: 445–458. doi: 10.1111/head.12291
- Issue published online: 4 MAR 2014
- Article first published online: 16 JAN 2014
- Manuscript Accepted: 17 NOV 2013
- pseudotumor cerebri;
- idiopathic intracranial hypertension;
- cerebral venous system;
Pseudotumor cerebri syndrome (PTCS) is an uncommon disorder of raised intracranial pressure of unknown etiology. The signs and symptoms have been well described but the pathogenesis remains a mystery. Most of the evidence suggests increased resistance to cerebrospinal fluid outflow as being pivotal to the disorder. Any comprehensive theory on causation will have to explain the preponderance of obese women of childbearing age with primary PTCS and lack of ventriculomegaly in the disorder. It is possible that female sex hormones, along with endocrinologically active adipose tissue, directly result in the syndrome, in those genetically predisposed. Aldosterone has been proposed also as important in the development of PTCS. Vitamin A, in the form of retinoic acid, may also play a pivotal role, and is influenced by both estrogen and adipose tissue. This article reviews proposed mechanisms of PTCS.