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.

Leave a Reply