The Pathophysiology of Pseudotumor Cerebri

Abstract

It has been a long-standing challenge to clinicians and investigators to explain the pathophysiology of the diverse syndromes collectively termedbenign intracranial pressure(BIH), orpseudotumor cerebri. The diverse etiologic factors associated with BIH include lateral or superior longitudinal sinus thrombosis, menstrual irregularities with obesity, a heterogenous group of endocrine disorders including adrenal insufficiency, hyperadrenalism, and corticosteroid hormone withdrawal, hyperparathyroidism, hypothyroidism, pregnancy, menarche, lupus, intoxication with vitamin A or chlordecone, tetracycline therapy, spinal cord tumors, and the Guillain-Barré syndrome.¹In many cases the syndrome occurs de novo in otherwise healthy persons. Although papilledema and intracranial hypertension without focal signs are the initial clinical findings, it seems highly unlikely that the mechanism of the intracranial hypertension in the heterogenous conditions just listed would be the same. With the exception of those patients with venous occlusion, the pathophysiology of the intracranial hypertension in the various forms of the disorder are