Contemporary management of the pseudotumor cerebri syndrome

Introduction: Diagnosis and appropriate management of patients with the pseudotumor cerebri syndrome are imperative to prevent or minimize permanent visual loss and headache-related disability. Areas covered: Steps in management, including making the correct diagnosis, techniques to assess the patient's visual status, medical treatment of intracranial hypertension and the associated headaches, weight management strategies, surgical treatments and stenting are reviewed incorporating the most recent medical evidence. Expert opinion: As the pathogenesis of the pseudotumor cerebri syndrome is still unknown, many of the currently employed management strategies incorporate a 'plumbing approach' to decrease cerebrospinal fluid (CSF) pressure. The Idiopathic Intracranial Hypertension Trial (IIHTT) taught us that the disorder markedly affects visual and overall quality of life, and that reducing pressure alone is not enough to make patients well, even those considered to have 'mild' vision loss. Other than the IIHTT, the evidence supporting the use of various treatments is meager. The course of the disorder can be unpredictable, and the clinician(s) managing these patients are often uncertain about which treatments to employ. Moreover, the desired modalities and specialists are not universally available in all locations. An individualized and detailed approach to the various manifestations and nuances of the disorder is essential.