Idiopathic Intracranial Hypertension Etiology

Idiopathic Intracranial Hypertension Etiology

Patients in whom a syndrome of Intracranial hypertension secondary to certain medications develops or who are found to have cerebral lateral sinus thrombosis are nonetheless still classified as having idiopathic intracranial hypertension (IIH).

Idiopathic intracranial hypertension is the preferred term for this condition, replacing pseudotumor cerebri, which often includes cerebral venous sinus thrombosis and other etiologies of Intracranial hypertension, and benign intracranial hypertension, which does not take into account that some IIH patients do not have a “benign” course and go on to irreversibly vision loss 1).

Idiopathic intracranial hypertension (IIH) is an aetiologically unknown disorder that associates with endocrinological disturbances, including dysfunction of the hypothalamic-pituitary-adrenal-axis.

Although there are multiple hypotheses for the etiology of Idiopathic intracranial hypertension (IIH) mainly focused on obesity and metabolic dysfunction, there is no known cause of the development of IIH. IIH occurs most frequently among obese women of childbearing age 2).

Transverse sinus (TS) stenosis has been associated with IIH and some authors have suggested a possible pathogenic role of reduced venous outflow in the development of IIH 3).

Risk factors for development of IIH include high body mass index, recent weight gain, and obstructive sleep apnea 4).

IIH accounts for a considerable part of the causes of intractable headache in systemic lupus erythematosus SLE patients and steroids should be considered as a first-line treatment 5).

Young women are more frequently involved with in half of cases a diffuse proliferative glomerulonephritis. Predisposing factors, like anaemia, must be associated. IH allows SLE diagnose in more than the third of the cases. Then, SLE has to be searched as an etiology of IH, in particular in non-obese patients and when nephritis is associated 6).

Raggi et al reported for the first time the presence of Binge eating disorder (BED) among patients with idiopathic intracranial hypertension (IIH) and showed that BED is associated to IIH, ICP and history of abuse or neglect 7).

Ahmed et al report a case of a young female who presented with signs and symptoms of IIH and was subsequently diagnosed with IgA nephropathy and end-stage renal disease. This is the first report of IgA nephropathy presenting as end-stage renal disease in a patient who presented with IIH 8).

see Idiopathic intracranial hypertension after minocycline.

Avoidant Restrictive Food Intake Disorder


Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2012 May;83(5):488-94. doi: 10.1136/jnnp-2011-302029. Review. PubMed PMID: 22423118; PubMed Central PMCID: PMC3544160.
2) , 4)

Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010 Aug;28(3):593-617. doi: 10.1016/j.ncl.2010.03.003. PubMed PMID: 20637991; PubMed Central PMCID: PMC2908600.

Higgins JNP et al. J Neurol Neurosurg Psychiatry 2004; 75:621 – 625

Kim JM, Kwok SK, Ju JH, Kim HY, Park SH. Idiopathic intracranial hypertension as a significant cause of intractable headache in patients with systemic lupus erythematosus: a 15-year experience. Lupus. 2012 Apr;21(5):542-7. doi: 10.1177/0961203311435267. Epub 2012 Feb 2. PubMed PMID: 22300830.

Maillart E, Gueguen A, Obadia M, Moulignier A, Vignal-Clermont C, Gout O. [Intracranial hypertension and lupus]. Rev Neurol (Paris). 2011 Jun-Jul;167(6-7):505-10. doi: 10.1016/j.neurol.2010.10.014. Epub 2011 Mar 21. French. PubMed PMID: 21420703.

Raggi A, Curone M, Bianchi Marzoli S, Chiapparini L, Ciasca P, Ciceri EF, Erbetta A, Faragò G, Leonardi M, D’Amico D. Impact of obesity and binge eating disorder on patients with idiopathic intracranial hypertension. Cephalalgia. 2016 Mar 18. pii: 0333102416640514. [Epub ahead of print] PubMed PMID: 26994301.

Ahmed US, Bacaj P, Iqbal HI, Onder S. IgA Nephropathy in a Patient Presenting with Pseudotumor Cerebri. Case Rep Nephrol. 2016;2016:5273207. doi: 10.1155/2016/5273207. Epub 2016 Feb 16. PubMed PMID: 26989531; PubMed Central PMCID: PMC4771878.

Arterial hypertension treatment

Arterial hypertension treatment

There are anatomical and physiological evidences that the ventrolateral (VL) region of the medulla plays an important role in blood pressure regulation and that dysfunction at this level may generate hypertension (HT). Vascular compression by a megadolicho-artery from the vertebrobasilar arterial system at the root entry/exit zone (REZ) of the glossopharyngeal nerve (IXth) and vagal nerve(Xth) cranial nerves (CNs) and the adjacent VL aspect of the medulla has been postulated as a causal factor for HT from neurogenic origin. The first attempts at microvascular decompression (MVD) of the IX-Xth CNs together with the neighbouring VL brainstem was revealed promising. Established criteria for an indication of MVD as an aetiological treatment of apparent essential HT are still needed 1).

Angiotensin-converting enzyme inhibitor.

Angiotensin II receptor blocker.

To treat hypertension patients with COVID-19 caused pneumonia, anti-hypertensive drugs (ACEs and ARBs) may be used according to the relative guidelines. The treatment regimen with these drugs does not need to be altered for the COVID-19 patients 2).










Sindou M. Is There a Place for Microsurgical Vascular Decompression of the Brainstem for Apparent Essential Blood Hypertension? A Review. Adv Tech Stand Neurosurg. 2015;42:69-76. PubMed PMID: 25411145.

Wang W, Zhao X, Wei W, Fan W, Gao K, He S, Zhuang X. Angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARBs) may be safe for COVID-19 patients. BMC Infect Dis. 2021 Jan 25;21(1):114. doi: 10.1186/s12879-021-05821-5. PMID: 33494713.

Idiopathic intracranial hypertension surgery

Idiopathic intracranial hypertension surgery

Systematic reviews

Prospective studies on the surgical options for Idiopathic intracranial hypertension (IIH) are scant and no evidence-based guidelines for the surgical management of medically refractory IIH have been established. A search in Cochrane LibraryMEDLINE and EMBASE from 1 January 1985 to 19 April 2019 for controlled or observational studies on the surgical treatment of IIH (defined in accordance with the modified Dandy or the modified Friedman criteria) in adults yielded 109 admissible studies. Venous sinus stenting (VSS) improved papilledemavisual fields and headaches in 87.1%, 72.7%, and 72.1% of the patients respectively, with a 2.3% severe complication rate and 11.3% failure rate. Cerebrospinal fluid diversion techniques diminished papilledema, visual field deterioration, and headaches in 78.9%, 66.8%, and 69.8% of the cases and are associated with a 9.4 severe complication rate and a 43.4% failure rate. Optic nerve sheath fenestration ameliorated papilledema, visual field defects and headaches in 90.5, 65.2%, and 49.3% of patients. The severe complication rate was 2.2% and the failure rate was 9.4%. This is currently the largest systematic review for the available operative modalities for IIH. VSS provided the best results in headache resolution and visual outcomes, with low failure rates and a very favorable complication profile. In light of this, Venous sinus stenting ought to be regarded as the first-line surgical modality for the treatment of medically refractory IIH 1).


Idiopathic intracranial hypertension patients may require surgical management when maximal medical treatment has failed.

Controversy still exists about which is the preferred initial surgical treatment for IIH. Emerging procedures include venous sinus stenting in cases with venous sinus stenosis, and bariatric surgery for weight loss. Cranial (suboccipital or subtemporal) decompression was a more popular surgical procedure in the past, but can still have a role in selected cases with impaired cerebrospinal flow dynamics (e.g. Chiari malformation) or after multiple failed conventional surgical procedures 2).

Venous sinus stenting ought to be regarded as the first-line surgical modality for the treatment of medically refractory IIH 3).

The election will likely be based on local expertise until well designed, multicentered clinical trials clarify which intervention best suits a particular patient 4).

The visual outcomes of these procedures are favorable, though they tend to be associated with a high rate of complication and failure. Recent trials suggest that venous sinus stenting offers both comparable rates of efficacy – with improved papilledema in 97% of patients, resolved headache in 83%, and improved visual acuity in 78% – and improved safety and reliability relative to older surgical techniques.

Patients whose sight is threatened by medically refractory IIH must often consider invasive procedures to control their disease. Venous sinus stenting may offer equal efficacy and lower failure and complication rates than traditional surgical approaches such as optic nerve sheath fenestration and cerebrospinal fluid diversion.

Several surgical treatment modalities, including lumboperitoneal shunt or ventriculoperitoneal shunt surgery, subtemporal decompression, endovascular venous sinus stenting, optic nerve decompression (OND), were used in the management of idiopathic intracranial hypertension (IIH). Each surgical technique has different advantages and disadvantages. Endoscopic OND is rarely used in the management of IIH. There are only forteen reported cases 5).


Cerebrospinal fluid diversion procedures

The most commonly performed surgical treatments for IIH are cerebrospinal fluid diversion procedures (e.g. ventriculo- and lumboperitoneal shunts).

Lumboperitoneal shunt

see Lumboperitoneal shunt for idiopathic intracranial hypertension

Ventriculoperitoneal shunt

see Ventriculoperitoneal shunt for pseudotumor cerebri

Transverse sinus stenting

see Transverse sinus stenting for idiopathic intracranial hypertension.

Optic nerve sheath fenestration

see Optic nerve sheath fenestration.

Subtemporal decompression

see Subtemporal decompression.


1) , 3)

Kalyvas A, Neromyliotis E, Koutsarnakis C, Komaitis S, Drosos E, Skandalakis GP, Pantazi M, Gobin YP, Stranjalis G, Patsalides A. A systematic review of surgical treatments of idiopathic intracranial hypertension (IIH). Neurosurg Rev. 2020 Apr 25. doi: 10.1007/s10143-020-01288-1. [Epub ahead of print] Review. PubMed PMID: 32335853.

Spitze A, Malik A, Lee AG. Surgical and endovascular interventions in idiopathic intracranial hypertension. Curr Opin Neurol. 2014 Feb;27(1):69-74. doi: 10.1097/WCO.0000000000000049. PubMed PMID: 24296639.

Uretsky S. Surgical interventions for idiopathic intracranial hypertension. Curr Opin Ophthalmol. 2009 Nov;20(6):451-5. doi: 10.1097/ICU.0b013e3283313c1c. Review. PubMed PMID: 19687737.

Sencer A, Akcakaya MO, Basaran B, Yorukoglu AG, Aydoseli A, Aras Y, Sencan F, Satana B, Aslan I, Unal OF, Izgi N, Canbolat A. Unilateral endoscopic optic nerve decompression for idiopathic intracranial hypertension: a series of 10 patients. World Neurosurg. 2014 Nov;82(5):745-50. doi: 10.1016/j.wneu.2014.03.045. Epub 2014 Apr 2. PubMed PMID: 24704940.
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