The Pseudotumor Cerebri Syndrome

Neurol Clin. 2024 May;42(2):433-471. doi: 10.1016/j.ncl.2024.02.001.


Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

Keywords: Idiopathic intracranial hypertension; Obesity; Optical coherence tomography; Papilledema; Pseudotumor cerebri.

Publication types

  • Review

MeSH terms

  • Headache / diagnosis
  • Headache / etiology
  • Headache / therapy
  • Humans
  • Intracranial Hypertension* / complications
  • Papilledema* / complications
  • Papilledema* / diagnosis
  • Pseudotumor Cerebri* / diagnosis
  • Pseudotumor Cerebri* / etiology
  • Pseudotumor Cerebri* / therapy
  • Syndrome
  • Vision Disorders / therapy