Idiopathic Intracranial Hypertension: An Unusual Cause of Headache in a Patient With Sickle Cell Disease

• Type of Material: Article
• Authors: Toby Terwilliger, Emory University; Mary Ann Kirkconnell Hall, Emory University; Eric Galante, Emory University
• Language: English
• Date: 2022-01-01
• Publisher: American Federation for Medical Research
• Publication Version: Final Published Version
• Copyright Statement: © 2022 American Federation for Medical Research
• License: Creative Commons Attribution-NonCommercial 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1177/23247096221140248
• Title of Journal or Parent Work: Journal of Investigative Medicine High Impact Case Reports
• Volume: 10
• Start Page: 23247096221140248
• End Page: 23247096221140248
• Grant/Funding Information: The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: No specific funding was received for the clinical conduct or writing of this work. The Emory University Division of Hospital Medicine Open Access Publishing Fund provided funding for open access publication.
• Keywords: headache; Pseudotumor Cerebri; sickle cell disease; case report; Magnetic Resonance Imaging; Humans; Hydroxyurea; idiopathic intracranial hypertension; Anemia, Sickle Cell; Adult; Headache; Female
• Research Categories: Health Sciences, Mental Health

Abstract

Idiopathic intracranial hypertension (IIH) is a rare cause of headache and visual disturbance with no known association with sickle cell disease (SCD). We present a patient with SCD with gradual onset of a severe headache and visual changes. Brain magnetic resonance imaging, angiography, and venography were nondiagnostic. Lumbar puncture established a diagnosis of IIH, and the patient had rapid improvement with large-volume lumbar puncture and acetazolamide. To our knowledge, this is the first case of IIH in a nonobese adult with SCD taking hydroxyurea, suggesting an association between SCD or hydroxyurea use and IIH. Furthermore, her clinical course demonstrates the complexities of treating IIH in patients with SCD.