Idiopathic intracranial hypertension: Diagnosis, monitoring and treatment.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) is a disorder typically affecting young, obese women, producing a syndrome of increased intracranial pressure without identifiable cause.

STATE OF THE ART: Despite a large number of hypotheses and publications over the past decade, the etiology of IIH is still unknown. There continues to be no evidence-based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, many questions remain, regarding the efficacy of acetazolamide, cerebrospinal fluid (CSF) shunting procedures, optic nerve sheath fenestration, and cerebral transverse venous sinus stenting. Identification of subgroups of patients at high-risk for irreversible visual loss, such as black patients, men, morbidly obese patients, and patients with fulminant IIH, helps determine management approaches and refine follow-up strategies.

PERSPECTIVE: Better understanding of the pathophysiology and ongoing clinical trials will hopefully help inform treatment strategies over the next few years.