Contemporary management of the pseudotumor cerebri syndrome

ABSTRACT

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.

KEYWORDS:

• Carbonic anhydrase inhibitor
• cerebrospinal fluid shunting
• idiopathic intracranial hypertension
• headache treatment
• optic nerve sheath fenestration
• optical coherence tomography
• papilledema
• pseudotumor cerebri

Article highlights

• Make the correct diagnosis using the most recent (2013) diagnostic criteria.

• Management requires a multi-disciplinary team with a captain (usually a neurologist or neuro-ophthalmologist)

• Evaluate patients for a secondary cause and discontinue potentially causative medications. CSF pressure-lowering treatments will probably still be needed.

• Obtain a detailed visual assessment including perimetry at diagnosis and throughout the course of the disorder.

• Acetazolamide is the only medication with level A evidence for the treatment of idiopathic intracranial hypertension associated with mild visual loss (Humphrey perimetric mean deviation of −2 to −7 dB). Other medical options which have not been rigorously studied include methazolamide, loop diuretics, topiramate, and octreotide.

• Headache is a main contributing factor to poor quality of life with IIH. Headaches often need separate treatment beyond efforts to lower the cerebrospinal fluid pressure. They are treated based on their phenotype while avoiding medications that cause weight gain or fluid retention.

• An initial weight loss goal of 6% of body weight is associated with improvements in vision and papilledema. Bariatric surgery data are limited but this may be a necessary option for some patients.

• Shunting and venous sinus stenting may improve or stabilize vision loss; they do not reliably improve headache and are not recommended for treatment of headache. Shunts have a high failure rate over time and may produce shunt dependency. Stenting requires an experienced neuro-interventionalist. A clinical trial is underway in the U.S. comparing shunting, optic nerve sheath fenestration and medical treatment for patients with moderate to severe vision less.

Declarations of interests

DI Friedman has declared previous participation on advisory boards for: Alder BioPharmaceuticals, Allergan, Amgen/Novartis, Biohaven Pharmaceuticals, electroCore, Eli Lilly, Revance, Supernus, Teva, Theranica and Zosano. She has also acted as a speaker for: Allergan, Amgen/Novartis, electocore, Autonomic Technologies, Inc, Supernus and Teva. She has received grant support from: Autonomic Technologies, Inc., Eli Lilly, Merck and Zosano. She has also consulted for electroCore and Promius and have acted on medical advisory boards for the Spinal CSF Leak Foundation and HealthyWomen. Finally, She have serves on the board of directors for the American HeadacheSociety. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or conflict with the subject matter or materials discussed in this manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Declaration of interest

No potential conflict of interest was reported by the author.