Medication Summary

Medications used in the treatment of idiopathic intracranial hypertension (IIH) include acetazolamide and corticosteroids.

Class Summary

Carbonic anhydrase (CA) is an enzyme found in many tissues of the body, including the eye. CA inhibitors catalyze a reversible reaction in which carbon dioxide becomes hydrated and CA dehydrated.

Acetazolamide (Diamox sequels)

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Acetazolamide is a first-line drug for treatment of IIH.

Class Summary

Corticosteroids agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body’s immune response to diverse stimuli.

Prednisone

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If acetazolamide fails to relieve symptoms of IIH, corticosteroids may be tried. Experience with prednisone in the treatment of pediatric IIH has shown that short-term use (ie, ≤ 1 month) is safe and effective.

Prednisolone (Pediapred, Millipred, Orapred)

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Prednisolone decreases inflammation by suppressing the production of leukotrienes and migration of polymorphonuclear leukocytes and by reducing capillary permeability. Experience has shown that short-term corticosteroid use (ie, ≤1 month) is safe and effective.

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Media Gallery

For IIH to be diagnosed, brain scans (such as MRI) must be performed to ensure there is no underlying cause for the increased pressure around the brain
Left optic disc with moderate chronic papilledema in patient with idiopathic intracranial hypertension (pseudotumor cerebri). Paton lines (arc-shaped retinal wrinkles concentric with disc margin) are seen along temporal side of inferior pole of disc.
Right optic disc with postpapilledema optic atrophy in patient with idiopathic intracranial hypertension (pseudotumor cerebri). Diffuse pallor of disc and absence of small arterial vessels on surface are noted, with very little disc elevation. Disc margin at upper and lower poles and nasally is obscured by some residual edema in nerve fiber layer and gliosis that often persists even after all edema has resolved.
Most common early visual field defect in papilledema as optic nerve develops optic atrophy is inferior nasal defect, as shown in left eye field chart (left side of figure). Shaded area indicates defective portion of field. Note sharp line of demarcation between defective lower nasal quadrant and normal upper nasal quadrant along horizontal midline. This is characteristic of early papilledema optic atrophy and is referred to as nasal step or inferonasal step.

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Author

Jasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Professor of Neurology, Loyola University Medical Center

Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Additional Contributors

Raj D Sheth, MD Chief, Division of Pediatric Neurology, Nemours Children's Clinic; Professor of Neurology, Mayo Clinic Alix School of Medicine; Professor of Pediatrics, University of Florida College of Medicine

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, Child Neurology Society

Disclosure: Nothing to disclose.

Acknowledgements

William C Robertson Jr, MD Professor, Departments of Neurology, Pediatrics, and Family Practice, Clinical Title Series, University of Kentucky College of Medicine

William C Robertson Jr, MD is a member of the following medical societies: American Academy of Neurology and Child Neurology Society

Disclosure: Nothing to disclose.

Raj D Sheth, MD Professor, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment