Pseudotumor cerebri syndrome

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By Deborah I Friedman MD

Pseudotumor cerebri syndrome continues to be a diagnostic and therapeutic challenge, and the incidence is rising as obesity becomes more prevalent. Dr. Deborah I Friedman of the University of Texas Southwestern Medical Center updates the clinical features of pseudotumor cerebri syndrome in adults and children, secondary causes, current therapies, and the role of cerebral venous sinus disease.

Key points

  • Headaches, transient obscurations of vision and pulsatile tinnitus, are the most frequent early manifestations of pseudotumor cerebri syndrome in adults.
  • Obese women of childbearing age are most commonly affected by idiopathic intracranial hypertension, but the syndrome may occur from a secondary cause in children, nonobese patients, and those over 45 years of age.
  • The manifestations of pseudotumor cerebri syndrome in children differ from those in adults and include prominent neck or back pain, diplopia, torticollis, other focal neurologic signs, headache, visual loss, and asymptomatic papilledema.
  • Central visual loss at presentation is an ominous sign requiring aggressive intervention.
  • A team approach to management is ideal, and a team leader (generally a neurologist or neuro-ophthalmologist) is critical.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited