Idiopathic intracranial hypertension (IIH) is defined as a syndrome of raised intracranial pressure with normal imaging of the brain and cerebrospinal fluid (CSF) composition. There is a rising incidence and prevalence of this disease related to the increased prevalence of obesity. It typically affects women of working age, and headache is the predominant morbidity in over 90%. The disease is also more prevalent in young males. (Rohit et al, 2021)
Venous sinus stenosis has been recognized as a contributing factor in the majority of patients with idiopathic intracranial hypertension. (Fargen et al, 2023)
Although the majority of patients with IIH present classically with headache and papilledema, some patients may have unusual presentations or manifestations. (Chen et al, 2023)
Visual alterations and headache are the two main symptoms of idiopathic intracranial hypertension, although additional features including cranial nerve palsies, cognitive deficits, olfactory deficits and tinnitus are not uncommon. The headache associated with idiopathic intracranial hypertension frequently has a migrainous phenotype. (Raoof and Hoffmann, 2021)
The typical patient symptom profile is the presence of daily headache, but also pulse synchronous tinnitus, transient visual obscurations, and papilledema with its associated visual loss. (Wall, 2017)
Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a disorder of elevated intracranial pressure of unknown cause. Patients present with daily headache, pulse-synchronous tinnitus, transient visual obscurations, papilledema with its associated visual loss, and diplopia from sixth nerve paresis. (Wall, 2008)
The Idiopathic Intracranial Hypertension Treatment has proven that acetazolamide should be the first line therapy, but other treatment options exist in patients who cannot tolerate acetazolamide, which requires surgical intervention. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse. (Burkett and Ailani, 2018)
Rohit W, Rajesh A, Mridula R, Jabeen SA. Idiopathic Intracranial Hypertension - Challenges and Pearls. Neurol India. 2021 Nov-Dec;69(Supplement):S434-S442. doi: 10.4103/0028-3886.332276. PMID: 35103000.
Fargen KM, Coffman S, Torosian T, Brinjikji W, Nye BL, Hui F. "Idiopathic" intracranial hypertension: An update from neurointerventional research for clinicians. Cephalalgia. 2023 Apr;43(4):3331024231161323. doi: 10.1177/03331024231161323. PMID: 36924237.
Chen BS, Britton JOT. Expanding the clinical spectrum of idiopathic intracranial hypertension. Curr Opin Neurol. 2023 Feb 1;36(1):43-50. doi: 10.1097/WCO.0000000000001131. Epub 2022 Nov 28. PMID: 36444979; PMCID: PMC9835678.
Raoof N, Hoffmann J. Diagnosis and treatment of idiopathic intracranial hypertension. Cephalalgia. 2021 Apr;41(4):472-478. doi: 10.1177/0333102421997093. Epub 2021 Feb 25. PMID: 33631966; PMCID: PMC8020303.
Wall M. Update on Idiopathic Intracranial Hypertension. Neurol Clin. 2017 Feb;35(1):45-57. doi: 10.1016/j.ncl.2016.08.004. PMID: 27886895; PMCID: PMC5125521.
Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. 2008 Mar;8(2):87-93. PMID: 18460275.
Burkett JG, Ailani J. An Up to Date Review of Pseudotumor Cerebri Syndrome. Curr Neurol Neurosci Rep. 2018 May 2;18(6):33. doi: 10.1007/s11910-018-0839-1. PMID: 29721718.