Abstract

Idiopathic intracranial hypertension (IIH) or Benign intracranial hypertension (BIH) is an uncommon disorder characterized by signs and symptoms of raised intracranial pressure of unknown cause and usually without any evidence of intracranial pathology. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. The most common symptoms are headache, nausea, vomiting, pulsatile tinnitus, transient episodes of visual loss, diplopia and other visual symptoms. Papilledema is a major clinical sign. Visual impairment is a serious complication that may not be recognized by the patients. As its presenting symptoms mimic those of a brain tumour, it’s also called pseudotumor cerebri (PTC). This paper in brief reviews epidemiology, clinical manifestations, etiopathogenetic hypothesis, diagnostic challenges, and current treatments of IIH including medical, surgical, and interventional management of this condition. The efficacy and complications of surgical interventions like cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in recent two to three decades have been summarized in brief

• 1.   Binder DK, Horton JC, Lawton MT, McDermott MW (March 2004). “Idiopathic intracranial hypertension”. Neurosurgery 54 (3): 538–51; discussion5512. doi:10.1227/01. NEU.0000109042.87246.3C.PMID 15028127
• 2.   Almarzouqi SJ, Morgan ML, Lee AG. Idiopathic intracranial hypertension in the Middle East: A growing concern. Saudi J Ophthalmol. 2015;29(1):26–31. doi: 10.1016/j.sjopt.2014.09.013
• 3.   Gafoor VA, Smita B, Jose J. Obesity, Ethnicity and Idiopathic Intracranial Hypertension. Ann Indian Acad Neurol. 2019;22(3):352. doi: 10.4103/aian. AIAN_114_19
• 4.   Mollan, Susan P et al. “Evolving evidence in adult idiopathic intracranial hypertension: pathophysiology and management.” Journal of neurology, neurosurgery, and psychiatry vol. 87,9 (2016): 982-92. doi:10.1136/jnnp-2015-311302
• 5.   Cordenier, Ann et al. “Headache associated with cough: a review.” The journal of headache and pain vol. 14,1 42. 20 May. 2013, doi:10.1186/1129-2377- 14-42
• 6.   Azarmina, Mohsen, and Hossein Azarmina. “The six syndromes of the sixth cranial nerve.” Journal of ophthalmic & vision research vol. 8,2 (2013): 160-71.
• 7.   Rigi, Mohammed et al. “Papilledema: epidemiology, etiology, and clinical management.” Eye and brain vol. 7 47-57. 17 Aug. 2015, doi:10.2147/EB. S69174
• 8.   Biousse, Valérie et al. “Update on the pathophysiology and management of idiopathic intracranial hypertension.” Journal of neurology, neurosurgery, and psychiatry vol. 83,5 (2012): 488- 94. doi:10.1136/jnnp-2011-302029
• 9.   Sugerman HJ, DeMaria EJ, Felton WL 3rd, Nakatsuka M, Sismanis A.Increased intraabdominal pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri. Neurology. 1997 Aug;49(2):507-11.
• 10.   Tabassi A, Salmasi AH, Jalali M.Serum and CSF vitamin A concentrations in idiopathic intracranial hypertension. Neurology. 2005 Jun 14;64(11):1893-6 11. Miyajima, Masakazu, and Hajime Arai. “Evaluation of the Production and Absorption of Cerebrospinal Fluid.” Neurologia medico-chirurgica vol. 55,8 (2015): 647-56. doi:10.2176/nmc.ra.2015-0003
• 12.   Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. 2002 Nov 26;59(10):1492-5.
• 13.   Hayreh, Sohan Singh. “Pathogenesis of optic disc edema in raised intracranial pressure.” Progress in retinal and eye research vol. 50 (2016): 108-44. doi: 10.1016/j.preteyeres.2015.10.001
• 14.   Update Idiopathic intracranial hypertension April by neurosurgery. Directory J.Sales-Llopis
• 15.   Doherty, Carolynne M, and Raeburn B Forbes. “Diagnostic Lumbar Puncture.” The Ulster medical journal vol. 83,2 (2014): 93-102.
• 16.   Weight Management: State of the Science and Opportunities for Military Programs. Washington (DC): National Academies Press (US); 2004. 4, Weight-Loss and Maintenance Strategies.
• 17.   Scotton, William J et al. “Topiramate is more effective than acetazolamide at lowering intracranial pressure.” Cephalalgia: an international journal of headache vol. 39,2 (2019): 209-218. doi:10.1177/0333102418776455
• 18.   Yaqub, Muhammad Amer et al. “Efficacy and safety of optic nerve sheath fenestration in patients with raised intracranial pressure.” Pakistan journal of medical sciences vol. 33,2 (2017): 471-475. doi:10.12669/pjms.332.11937
• 19.   Quincke HI (1893). “Meningitis serosa”. Sammlung Klinischer Vorträge 67: 655.
• 20.   Corbett JJ, Thompson HS (October 1989). “The rational management of idiopathic intracranial hypertension”. Arch. Neurol. 46 (10): 1049 51. PMID 2679506.

Data Sharing Statement

Funding