Idiopathic Intracranial Hypertension (IIH) Guidelines

1. Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014 Jun. 71 (6):693-701. [QxMD MEDLINE Link].

2. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee., Wall M, McDermott MP, Kieburtz KD, Corbett JJ, Feldon SE, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014 Apr 23-30. 311 (16):1641-51. [QxMD MEDLINE Link].

3. Kalyvas A, Neromyliotis E, Koutsarnakis C, Komaitis S, Drosos E, Skandalakis GP, et al. A systematic review of surgical treatments of idiopathic intracranial hypertension (IIH). Neurosurg Rev. 2021 Apr. 44 (2):773-792. [QxMD MEDLINE Link].

4. Smith JL. Whence pseudotumor cerebri?. J Clin Neuroophthalmol. 1985 Mar. 5 (1):55-6. [QxMD MEDLINE Link].

5. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002 Nov 26. 59 (10):1492-5. [QxMD MEDLINE Link].

6. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016 Jan. 15 (1):78-91. [QxMD MEDLINE Link].

7. Toscano S, Lo Fermo S, Reggio E, Chisari CG, Patti F, Zappia M. An update on idiopathic intracranial hypertension in adults: a look at pathophysiology, diagnostic approach and management. J Neurol. 2021 Sep. 268 (9):3249-3268. [QxMD MEDLINE Link].

8. Dinkin M, Oliveira C. Men Are from Mars, Idiopathic Intracranial Hypertension Is from Venous: The Role of Venous Sinus Stenosis and Stenting in Idiopathic Intracranial Hypertension. Semin Neurol. 2019 Dec. 39 (6):692-703. [QxMD MEDLINE Link].

9. Mondejar V, Patsalides A. The Role of Arachnoid Granulations and the Glymphatic System in the Pathophysiology of Idiopathic Intracranial Hypertension. Curr Neurol Neurosci Rep. 2020 May 22. 20 (7):20. [QxMD MEDLINE Link].

10. Nicholson P, Kedra A, Shotar E, Bonnin S, Boch AL, Shor N, et al. Idiopathic Intracranial Hypertension: Glymphedema of the Brain. J Neuroophthalmol. 2021 Mar 1. 41 (1):93-97. [QxMD MEDLINE Link].

11. Virdee J, Larcombe S, Vijay V, Sinclair AJ, Dayan M, Mollan SP. Reviewing the Recent Developments in Idiopathic Intracranial Hypertension. Ophthalmol Ther. 2020 Dec. 9 (4):767-781. [QxMD MEDLINE Link].

12. Agraz D, Morgan LA, Fouzdar Jain S, Suh DW. Clinical features of pediatric idiopathic intracranial hypertension. Clin Ophthalmol. 2019. 13:881-886. [QxMD MEDLINE Link].

13. Libien J, Kupersmith MJ, Blaner W, McDermott MP, Gao S, Liu Y, et al. Role of vitamin A metabolism in IIH: Results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci. 2017 Jan 15. 372:78-84. [QxMD MEDLINE Link].

14. Uddin AB. Drug-induced pseudotumor cerebri. Clin Neuropharmacol. 2003 Sep-Oct. 26 (5):236-8. [QxMD MEDLINE Link].

15. Radhakrishnan K, Ahlskog JE, Garrity JA, Kurland LT. Idiopathic intracranial hypertension. Mayo Clin Proc. 1994 Feb. 69 (2):169-80. [QxMD MEDLINE Link].

16. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. 1988 Aug. 45 (8):875-7. [QxMD MEDLINE Link].

17. Wall M, George D. Visual loss in pseudotumor cerebri. Incidence and defects related to visual field strategy. Arch Neurol. 1987 Feb. 44 (2):170-5. [QxMD MEDLINE Link].

18. Shah VA, Kardon RH, Lee AG, Corbett JJ, Wall M. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology. 2008 Feb 19. 70 (8):634-40. [QxMD MEDLINE Link].

19. Wall M, Falardeau J, Fletcher WA, Granadier RJ, Lam BL, Longmuir RA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology. 2015 Sep 1. 85 (9):799-805. [QxMD MEDLINE Link].

20. Subramaniam S, Fletcher WA. Obesity and Weight Loss in Idiopathic Intracranial Hypertension: A Narrative Review. J Neuroophthalmol. 2017 Jun. 37 (2):197-205. [QxMD MEDLINE Link].

21. Wall M. Idiopathic intracranial hypertension and the idiopathic intracranial hypertension treatment trial. J Neuroophthalmol. 2013 Mar. 33 (1):1-3. [QxMD MEDLINE Link].

22. Digre KB, Bruce BB, McDermott MP, Galetta KM, Balcer LJ, Wall M, et al. Quality of life in idiopathic intracranial hypertension at diagnosis: IIH Treatment Trial results. Neurology. 2015 Jun 16. 84 (24):2449-56. [QxMD MEDLINE Link].

23. Wong B, Fraser CL. Obstructive Sleep Apnea in Neuro-Ophthalmology. J Neuroophthalmol. 2019 Sep. 39 (3):370-379. [QxMD MEDLINE Link].

24. Bruce BB, Kedar S, Van Stavern GP, Monaghan D, Acierno MD, Braswell RA, et al. Idiopathic intracranial hypertension in men. Neurology. 2009 Jan 27. 72 (4):304-9. [QxMD MEDLINE Link].

25. Daniels AB, Liu GT, Volpe NJ, Galetta SL, Moster ML, Newman NJ, et al. Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol. 2007 Apr. 143(4):635-41. [QxMD MEDLINE Link].

26. Friedman DI, Quiros PA, Subramanian PS, Mejico LJ, Gao S, McDermott M, et al. Headache in Idiopathic Intracranial Hypertension: Findings From the Idiopathic Intracranial Hypertension Treatment Trial. Headache. 2017 Sep. 57 (8):1195-1205. [QxMD MEDLINE Link].

27. Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014 Jun. 71 (6):693-701. [QxMD MEDLINE Link].

28. Wall M, Falardeau J, Fletcher WA, Granadier RJ, Lam BL, Longmuir RA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology. 2015 Sep 1. 85 (9):799-805. [QxMD MEDLINE Link].

29. Cello KE, Keltner JL, Johnson CA, Wall M, NORDIC Idiopathic Intracranial Hypertension Study Group. Factors Affecting Visual Field Outcomes in the Idiopathic Intracranial Hypertension Treatment Trial. J Neuroophthalmol. 2016 Mar. 36 (1):6-12. [QxMD MEDLINE Link].

30. Wall M, Johnson CA, Cello KE, Zamba KD, McDermott MP, Keltner JL, et al. Visual Field Outcomes for the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). Invest Ophthalmol Vis Sci. 2016 Mar. 57 (3):805-12. [QxMD MEDLINE Link].

31. Friedman DI. The pseudotumor cerebri syndrome. Neurol Clin. 2014 May. 32 (2):363-96. [QxMD MEDLINE Link].

32. Lin WV, Berry S, Nakawah MO, Sadaka A, Lee AG. Idiopathic Intracranial Hypertension and Anemia: A Matched Case-Control Study. J Neuroophthalmol. 2020 Jun. 40 (2):163-168. [QxMD MEDLINE Link]. [Full Text].

33. Rehder D. Idiopathic Intracranial Hypertension: Review of Clinical Syndrome, Imaging Findings, and Treatment. Curr Probl Diagn Radiol. 2020 May - Jun. 49 (3):205-214. [QxMD MEDLINE Link].

34. Margolis MS, DeBusk AA, Moster ML, Falardeau JM, Eggenberger ER, Sergott RC, et al. Lumbar Puncture for Diagnosis of Idiopathic Intracranial Hypertension in Typical Patients. J Neuroophthalmol. 2021 Sep 1. 41 (3):375-378. [QxMD MEDLINE Link].

35. Avery RA, Shah SS, Licht DJ, Seiden JA, Huh JW, Boswinkel J, et al. Reference range for cerebrospinal fluid opening pressure in children. N Engl J Med. 2010 Aug 26. 363 (9):891-3. [QxMD MEDLINE Link].

36. Avery RA. Interpretation of lumbar puncture opening pressure measurements in children. J Neuroophthalmol. 2014 Sep. 34 (3):284-7. [QxMD MEDLINE Link].

37. Cartwright C, Igbaseimokumo U. Lumbar puncture opening pressure is not a reliable measure of intracranial pressure in children. J Child Neurol. 2015 Feb. 30 (2):170-3. [QxMD MEDLINE Link].

38. Wall M, McDermott MP, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014 Apr 23-30. 311(16):1641-51. [QxMD MEDLINE Link].

39. Optical Coherence Tomography Substudy Committee., NORDIC Idiopathic Intracranial Hypertension Study Group. Papilledema Outcomes from the Optical Coherence Tomography Substudy of the Idiopathic Intracranial Hypertension Treatment Trial. Ophthalmology. 2015 Sep. 122 (9):1939-45.e2. [QxMD MEDLINE Link].

40. Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD. Idiopathic intracranial hypertension: risk of recurrences. Neurology. 2004 Nov 9. 63 (9):1737-9. [QxMD MEDLINE Link].

41. ten Hove MW, Friedman DI, Patel AD, Irrcher I, Wall M, McDermott MP, et al. Safety and Tolerability of Acetazolamide in the Idiopathic Intracranial Hypertension Treatment Trial. J Neuroophthalmol. 2016 Mar. 36 (1):13-9. [QxMD MEDLINE Link].

42. Celebisoy N, Gökçay F, Sirin H, Akyürekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007 Nov. 116 (5):322-7. [QxMD MEDLINE Link].

43. Sobel RK, Syed NA, Carter KD, Allen RC. Optic Nerve Sheath Fenestration: Current Preferences in Surgical Approach and Biopsy. Ophthalmic Plast Reconstr Surg. 2015 Jul-Aug. 31 (4):310-2. [QxMD MEDLINE Link].

44. Malik AI, Xu J, Lee AG. Outcomes of optic nerve sheath fenestration from superomedial eyelid crease approach. Orbit. 2022 Aug. 41 (4):413-421. [QxMD MEDLINE Link].

45. Brazis PW. Clinical review: the surgical treatment of idiopathic pseudotumour cerebri (idiopathic intracranial hypertension). Cephalalgia. 2008 Dec. 28(12):1361-73. [QxMD MEDLINE Link].

46. Lee AG, Patrinely JR, Edmond JC. Optic nerve sheath decompression in pediatric pseudotumor cerebri. Ophthalmic Surg Lasers. 1998 Jun. 29 (6):514-7. [QxMD MEDLINE Link].

47. Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology. 2000 Oct. 107 (10):1907-12. [QxMD MEDLINE Link].

48. Gilbert AL, Chwalisz B, Mallery R. Complications of Optic Nerve Sheath Fenestration as a Treatment for Idiopathic Intracranial Hypertension. Semin Ophthalmol. 2018. 33 (1):36-41. [QxMD MEDLINE Link].

49. Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. 1993 May. 111(5):632-5. [QxMD MEDLINE Link].

50. Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997 Sep. 49(3):734-9. [QxMD MEDLINE Link].

51. Eggenberger ER, Miller NR, Vitale S. Lumboperitoneal shunt for the treatment of pseudotumor cerebri. Neurology. 1996 Jun. 46 (6):1524-30. [QxMD MEDLINE Link].

52. Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension. J Neurosurg. 1988 Aug. 69 (2):195-202. [QxMD MEDLINE Link].

53. Chumas PD, Kulkarni AV, Drake JM, Hoffman HJ, Humphreys RP, Rutka JT. Lumboperitoneal shunting: a retrospective study in the pediatric population. Neurosurgery. 1993 Mar. 32 (3):376-83; discussion 383. [QxMD MEDLINE Link].

54. Rosenberg ML, Corbett JJ, Smith C, Goodwin J, Sergott R, Savino P, et al. Cerebrospinal fluid diversion procedures in pseudotumor cerebri. Neurology. 1993 Jun. 43 (6):1071-2. [QxMD MEDLINE Link].

55. Lundar T, Nornes H. Pseudotumour cerebri-neurosurgical considerations. Acta Neurochir Suppl (Wien). 1990. 51:366-8. [QxMD MEDLINE Link].

56. McGirt MJ, Woodworth G, Thomas G, Miller N, Williams M, Rigamonti D. Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache: predictors of treatment response and an analysis of long-term outcomes. J Neurosurg. 2004 Oct. 101 (4):627-32. [QxMD MEDLINE Link].

57. Puffer RC, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J Neurointerv Surg. 2013 Sep 1. 5 (5):483-6. [QxMD MEDLINE Link].

58. Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus. 2007. 23(5):E6. [QxMD MEDLINE Link].

59. Kupersmith MJ, Gamell L, Turbin R, Peck V, Spiegel P, Wall M. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology. 1998 Apr. 50(4):1094-8. [QxMD MEDLINE Link].

60. Fridley J, Foroozan R, Sherman V, Brandt ML, Yoshor D. Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg. 2011 Jan. 114(1):34-9. [QxMD MEDLINE Link].

61. Hermes SM, Bharadwaj M, Miller NR, Waslo C, Husain FA, Wolfe BM, et al. Long-Term Outcomes of Bariatric Surgery in Idiopathic Intracranial Hypertension Patients. Neurologist. 2022 May 19. [QxMD MEDLINE Link].

62. Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998 Dec. 105(12):2313-7. [QxMD MEDLINE Link].

63. Wall M, Falardeau J, Fletcher WA, Granadier RJ, Lam BL, Longmuir RA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology. 2015 Sep 1. 85 (9):799-805. [QxMD MEDLINE Link].

64. [Guideline] Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018 Oct. 89 (10):1088-1100. [QxMD MEDLINE Link].

65. Pascarella A, Manzo L, Bono F. Effect of mannitol bolus administration on cerebrospinal fluid pressure in patients with idiopathic intracranial hypertension: a pilot study. J Neurol. 2022 Jun 25. [QxMD MEDLINE Link].

Andrea Tham, MD Resident Physician, Department of Ophthalmology, University of California, Irvine, School of Medicine

Andrea Tham, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Physicians, American Medical Association

Disclosure: Nothing to disclose.

Donny W Suh, MD, MBA, FAAP, FACS Professor, Department of Ophthalmology, Chief of Pediatric Ophthalmology and Adult Strabismus, Medical Director of Eye-Mobile, Gavin Herbert Eye Institute, UC Irvine Health, University of California, Irvine, School of Medicine; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Chief Medical Officer, Suh Precision Syringe, LLC; Medical Staff, Children’s Hospital of Orange County

Donny W Suh, MD, MBA, FAAP, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American College of Healthcare Executives, American College of Surgeons, American Medical Association, Section on Ophthalmology, American Ophthalmological Society, International Strabismological Association, Iowa Medical Society, Metro Omaha Medical Society, National Eye Care Project, Nebraska Chapter of American Academy of Pediatrics, ORBIS, Surgical Eye Expeditions (SEE) International

Disclosure: Nothing to disclose.

Andrew G Lee, MD Chair, Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch School of Medicine; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Texas Ophthalmological Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: AstraZeneca; Bristol Myers Squibb; Horizon<br/>Serve(d) as a speaker or a member of a speakers bureau for: Horizon<br/>Received ownership interest from Credential Protection for other.

Mark S Gans, MD Associate Professor, Director of Neuro-Ophthalmology, Interim Chair, Department of Ophthalmology, McGill University Faculty of Medicine; Clinical Director, Department of Ophthalmology, Adult Sites, McGill University Hospital Center, Canada

Mark S Gans, MD is a member of the following medical societies: American Academy of Ophthalmology, Canadian Medical Association, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Ariel Chen Baylor College of Medicine

Disclosure: Nothing to disclose.

Ashwini Kini, MD, FRCS Clinical Fellow in Neuro-Ophthalmology, Department of Ophthalmology, Houston Methodist Hospital

Ashwini Kini, MD, FRCS is a member of the following medical societies: All India Ophthalmological Society, Indian Medical Association

Disclosure: Nothing to disclose.

Robert A Egan, MD Director of Neuro-Ophthalmology, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association

Disclosure: Nothing to disclose.

Eric R Eggenberger, DO, MS, FAAN Professor, Vice-Chairman, Department of Neurology and Ophthalmology, Colleges of Osteopathic Medicine and Human Medicine, Michigan State University; Director of Michigan State University Ocular Motility Laboratory; Director of National Multiple Sclerosis Society Clinic, Michigan State University

Eric R Eggenberger, DO, MS, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

James Goodwin, MD Associate Professor, Departments of Neurology and Ophthalmology, University of Illinois College of Medicine; Director, Neuro-Ophthalmology Service, University of Illinois Eye and Ear Infirmary

James Goodwin, MD is a member of the following medical societies: American Academy of Neurology, Illinois State Medical Society, North American Neuro-Ophthalmology Society, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Edsel Ing, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Toronto East General Hospital, Canada

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada

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

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article