Idiopathic intracranial hypertension (IIH), also historically known as pseudotumour cerebri, is a syndrome with signs and symptoms of increased intracranial pressure but where a causative mass or hydrocephalus is not identified.
The older term benign intracranial hypertension is generally frowned upon due to the fact that some patients with IIH have a fairly aggressive clinical picture with rapid visual loss.
Interestingly as it has become evident that at least some patients present with IIH due to identifiable venous stenosis, some authors now advocate reverting to the older term pseudotumour cerebri as in these patients the condition is not idiopathic 15. An alternative approach is to move these patients into a group termed secondary intracranial hypertension 15.
By far the most common affected demographic are middle-aged obese females, although the aetiological link between being female, overweight and developing IIH remains to be elucidated. Less commonly IIH can also be encountered in males, usually older, and less likely to be obese 15. IIH is rare in the paediatric population 15.
Papilloedema may not always be present or may be unilateral (including pseudo-Foster Kennedy syndrome), making the clinical diagnosis less than straightforward 6. Neurological examination is usually normal, with a sixth cranial nerve palsy sometimes encountered.
Additional clinical findings include normal CSF composition with elevated opening pressure (opening pressure varies, however, with a mean pressure of less than 35mmHg, with pressure waves as high as 80mmHg which last 5 to 20 minutes) 6.
Meningoceles with secondary CSF leak can present as rhinorrhea, otorrhea, intracranial hypotension, and recurrent bacterial meningitis 7,9. In such patients it is often only after dural repair that intracranial hypertension becomes evident; presumably the CSF leak from the meningocoele normalized pressure 9.
IIH is a poorly understood condition. Various mechanisms have been proposed to explain its pathogenesis, including decreased CSF absorption, increased CSF production, increased intravascular volume, increased intracranial venous pressure and hormonal changes 1, 15.
A variety of conditions are known to be associated with IIH including:
- doxycycline 2
- chronic renal failure
- systemic lupus erythematosus (SLE)
- hypervitaminosis A (in the paediatric population)
- dural venous sinus stenosis or web: rare but treatable cause of IIH
In the absence of a cause for intracranial hypertension, imaging features that support the diagnosis of IIH include 3,6-9,15:
- optic nerves
- prominent subarachnoid space around the optic nerves (~45%)
- vertical tortuosity of the optic nerves (~40%)
- flattening of the posterior sclera (~80%)
- intraocular protrusion of the optic nerve head
- enhancement of the prelaminar (intra-ocular) optic nerves (~50%)
- enlarged arachnoid out-pouchings
- bilateral venous sinus stenosis
- lateral segments of the transverse sinuses
- no evidence of current or remote thrombosis 8
- slitlike ventricles (relatively uncommon compared to other findings) 15
- acquired tonsillar ectopia (mimicking Chiari I malformation) 16
- increased subcutaneous fat thickness in the scalp and neck 17
Although bony changes are permanent, the rest are dynamic and may be reversible with treatment 3.
* It is important to take into account the age and gender of a specific patient in assessing the significance of this finding, as in older patients, and especially in males, a partially empty non-enlarged sella is not only common, but normal.
Treatment and prognosis
Treatment options include CSF letting, acetazolamide and lumboperitoneal shunts. In patients with progressive visual deterioration, optic nerve fenestration may be required to preserve vision.
Venous sinus stenting has been reported in case series 4,10, and also being trialled 13,14. The treatment is controversial as to whether apparent venous sinus stenosis is the cause or the effect of IIH 11,12. Spontaneous resolution of apparent stenosis is recognised 6.
History and etymology
Idiopathic intracranial hypertension was first reported in 1893 by Heinrich Quincke, and terms "meningitis serosa" . The term "pseudotumour cerebri" was later introduced in 1904, and later still "benign intracranial hypertension" in 1955 (not to be, but easily, confused with benign intracranial hypotension) 15.
Other causes of intracranial hypertension and papilloedema should be sought. Causes of venous obstruction (e.g. venous sinus thrombosis and venous outflow obstruction in the neck) can very closely mimic the intracranial findings.
- 1. Silbergleit R, Junck L, Gebarski SS et-al. Idiopathic intracranial hypertension (pseudotumor cerebri): MR imaging. Radiology. 1989;170 (1): 207-9. Radiology (abstract) - Pubmed citation
- 2. Digre KB. Not so benign intracranial hypertension. BMJ. 2003;326 (7390): 613-4. doi:10.1136/bmj.326.7390.613 - Free text at pubmed - Pubmed citation
- 3. Zagardo MT, Cail WS, Kelman SE et-al. Reversible empty sella in idiopathic intracranial hypertension: an indicator of successful therapy? AJNR Am J Neuroradiol. 17 (10): 1953-6. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 4. Higgins JN, Cousins C, Owler BK et-al. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. J. Neurol. Neurosurg. Psychiatr. 2003;74 (12): 1662-6. doi:10.1136/jnnp.74.12.1662 - Free text at pubmed - Pubmed citation
- 5. De lucia D, Napolitano M, Di micco P et-al. Benign intracranial hypertension associated to blood coagulation derangements. Thromb J. 2006;4 : 21. doi:10.1186/1477-9560-4-21 - Free text at pubmed - Pubmed citation
- 6. Suzuki H, Takanashi J, Kobayashi K et-al. MR imaging of idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2001;22 (1): 196-9. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 7. Schuknecht B, Simmen D, Briner HR et-al. Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. AJNR Am J Neuroradiol. 2008;29 (3): 542-9. doi:10.3174/ajnr.A0840 - Pubmed citation
- 8. Leach J, Fortuna R, Jones B et-al. Imaging of Cerebral Venous Thrombosis: Current Techniques, Spectrum of Findings, and Diagnostic Pitfalls1. Radiographics. 2006;26 (suppl 1): S19-S41. Radiographics (full text) - doi:10.1148/rg.26si055174
- 9. Bialer OY, Rueda MP, Bruce BB et-al. Meningoceles in idiopathic intracranial hypertension. AJR Am J Roentgenol. 2014;202 (3): 608-13. doi:10.2214/AJR.13.10874 - Free text at pubmed - Pubmed citation
- 10. Ahmed RM, Wilkinson M, Parker GD et-al. Transverse sinus stenting for idiopathic intracranial hypertension: a review of 52 patients and of model predictions. AJNR Am J Neuroradiol. 2011;32 (8): 1408-14. doi:10.3174/ajnr.A2575 - Pubmed citation
- 11. Rohr A, Dörner L, Stingele R et-al. Reversibility of venous sinus obstruction in idiopathic intracranial hypertension. AJNR Am J Neuroradiol. 2007;28 (4): 656-9. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 12. Scoffings , Pickard and Higgins. Resolution of transverse sinus stenoses immediately after CSF withdrawal in idiopathic intracranial hypertension J Neurol Neurosurg Psychiatry 2007;78:8 911-912
- 13. Venous Sinus Stenting for Idiopathic Intracranial Hypertension Refractory to Medical Therapy (VSSIIH). https://clinicaltrials.gov/ct2/show/NCT01407809
- 14. Stenting of Venous Sinus Stenosis for Medically Refractory Idiopathic Intracranial Hypertension. https://clinicaltrials.gov/ct2/show/NCT02143258
- 15. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol. 2011;32 (11): 1986-93. doi:10.3174/ajnr.A2404 - Pubmed citation
- 16. Aiken AH, Hoots JA, Saindane AM et-al. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 2012;33 (10): 1901-6. doi:10.3174/ajnr.A3068 - Pubmed citation
- 17. Factors Determining the Clinical Significance of an “Empty” Sella Turcica Amit M. Saindane, Paolo P. Lim, Ashley Aiken, Zhengjia Chen, and Patricia A. Hudgins American Journal of Roentgenology 2013 200:5, 1125-1131
- 18. San Millán D, Kohler R. Enlarged CSF spaces in pseudotumor cerebri. AJR. American journal of roentgenology. 203 (4): W457-8. doi:10.2214/AJR.14.12787 - Pubmed