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Keywords:

  • pseudotumor cerebri;
  • idiopathic intracranial hypertension;
  • pathophysiology;
  • cerebral venous system;
  • retinol

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.