Abstract
Pseudotumor cerebri (PTC) is a syndrome of increased intracranial pressure without hydrocephalus or mass lesion and with normal cerebrospinal fluid (CSF) composition. Although often considered to be “idiopathic,” detailed investigation has revealed a high incidence of venous outflow abnormalities in PTC syndrome patients. The thought that elevated intracranial venous sinus pressure is a “universal mechanism” for PTC syndrome of varying etiologies has been called into question by a study indicating that the increased venous pressure in idiopathic PTC patients is caused by the elevated intracranial pressure and not the reverse, suggesting that “the chicken is the CSF pressure elevation and the egg is the venous sinus pressure elevation.” Vitamin A toxicity may play a role in the pathogenesis of idiopathic PTC. The treatment of PTC has two major goals: the alleviation of symptoms and preservation of visual function. When medical therapy fails or when visual dysfunction deteriorates, surgical therapies for PTC should be considered. The two main procedures performed include lumboperitoneal shunt and optic nerve sheath fenestration. Because of the association of PTC with venous sinus hypertension, some authors are considering venous sinus stenting for refractory cases of PTC. It is still unclear if primary treatment of the observed venous stenosis benefits patients with idiopathic PTC. This should be no surprise, as it is not certain whether the stenoses are the cause or the result of idiopathic PTC.
Similar content being viewed by others
References and Recommended Reading
Friedman DI, Jacobson DM: Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002, 59:1492–1495.
Van den Brink WA, Pieterman H, Avezaat CJ: Sagittal sinus occlusion, caused by an overlying depressed cranial fracture, presenting with late signs and symptoms of intracranial hypertension: case report. Neurosurgery 1996, 38:1044–1046.
Ansari I, Crichlow B, Gunton KB, et al.: A child with venous sinus thrombosis with initial examination findings of pseudotumor syndrome. Arch Ophthalmol 2002, 120:867–869.
Kiers L, King JO: Increased intracranial pressure following bilateral neck dissection and radiotherapy. Aust N Z J Surg 1991, 61:459–461.
Ageli SI, Sato Y, Gantz BJ: Glomus jugulare tumors masquerading as benign intracranial hypertension. Arch Otolaryngol 1994, 120:1277–1280.
Jicha GA, Suarez GA: Pseudotumor cerebri reversed by cardiac septal defect repair. Neurology 2003, 60:2016–2017.
Sareen D, Jain A, Paljor P: Pseudotumor syndrome associated with antiphospholipid antibodies and cerebral venous sinus thrombosis. J Assoc Physicians India 2002, 50:603–605.
Castillo IG, Foroozan R, Sergott RC: A sticky situation. Survey Ophthalmol 2002, 47:491–499.
Celebisoy N, Secil Y, Akyurekli O: Pseudotumor cerebri: etiological factors, presenting features and prognosis in the western part of Turkey. Acta Neurologica Scand 2002, 6:367–370.
Biousse V, Ameri A, Bousser MG: Isolated intracranial hypertension as the only sign of cerebral venous thrombosis. Neurology 1999, 53:1537–1542.
Tehindrazanarivelo A, Evard S, Schaison M, et al.: Prospective study of cerebral venous sinus thrombosis in patients presenting with benign intracranial hypertension. Cerebrovasc Dis 1992, 2:22–27.
Johnston I, Kollar C, Dunkley S, et al.: Cranial venous outflow obstruction in the pseudotumour syndrome: incidence, nature and relevance. J Clin Neurosci 2002, 9:273–278.
Farb RI, Vanek I, Scott JN, et al.: Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology 2003, 60:1418–1424. The authors determined the prevalence and nature of sinovenous structural abnormalities in idiopathic PTC using ATECO MRV. They prospectively studied 29 idiopathic PTC patients and 59 control patients. Substantial bilateral sinovenous stenoses were seen in 27 of 29 patients with idiopathic PTC and in only four of 59 control patients. Narrowed venous conduits appear to be a relatively sensitive and specific criterion for the diagnosis of idiopathic PTC, and over 90% of patients with idiopathic PTC have stenotic transverse sinuses.
Silberstein SD, McKinstry RC: The death of idiopathic intracranial hypertension? Neurology 2003, 60:1406–1407.
Vorstman EB, Niemann DB, Molyneux AJ, Pike MG: Benign intracranial hypertension associated with arteriovenous malformation. Dev Med Child Neurol 2002, 44:133–135.
Karahalios D, Rekate HL, Khayata MH, Apostolides PJ: Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies. Neurology 1996, 46:198–202.
King JO, Mitchell PJ, Thompson KR, Tress BM: Monometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology 2002, 58:26–30. These authors studied patients with idiopathic PTC and found that when transducer-measured intracranial venous pressure is high, reduction of CSF pressure by removal of CSF predictably lowers the venous sinus pressure. This study indicates that the increased venous pressure in idiopathic PTC patients is caused by the elevated ICP.
Corbett JJ, Digre K: Idiopathic intracranial hypertension. An answer to, "the chicken or the egg?" Neuorology 2002, 58:5–6.
Jacobson DM, Berg R, Wall M, et al.: Serum vitamin A concentration is elevated in idiopathic intracranial hypertension. Neurology 1999, 53:1114–1118.
Selhorst JB, Kulkantrakorn K, Corbett JJ, et al.: Retinol-binding protein in idiopathic intracranial hypertension (IIH). J Neuro-Ophthalmol 2000, 20:250–252.
Warner JE, Bernstein PS, Yemelyanov A, et al.: Vitamin A in the cerebrospinal fluid of patients with and without idiopathic intracranial hypertension. Ann Neurol 2002, 52:647–650.
Lampl Y, Eshel Y, Kessler A, et al.: Serum leptin level in women with idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2002, 72:642–643.
Lee AG, Brazis PW: Papilledema. In Clinical Pathways in Neuro-Ophthalmology. An Evidence-Based Approach, edn 2. Edited by Lee AG and Brazis PW. New York: Thieme; 2003:129–165.
Biousse V, Rucker JC, Vignal C, et al.: Anemia and papilledema. Am J Ophthalmol. 2003, 135:437–446.
Taylor JP, Galetta SL, Asbury AK, Volpe NJ: Hemolytic anemia presenting as idiopathic intracranial hypertension. Neurology 2002, 59:960–961.
Jeng MR, Rieman M, Bhakta M, et al.: Pseudotumor cerebri in two adolescents with acquired aplastic anemia. J Ped Hematol Oncol 2002, 24:765–768.
Weber KT, Singh KD, Hey JC: Idiopathic intracranial hypertension with primary aldosteronism: report of 2 cases. Am J Med Sci 2002, 324:45–50.
Serratrice J, Granel B, Conrath J, et al.: Benign intracranial hypertension and thyreostimulin suppression hormonotherapy. Am J Ophthalmol 2002, 134:910–911.
Villain MA, Pageaux GP, Veyrac M, et al.: Effect of acetazolamide on ocular hemodynamics in pseudotumor cerebri associated with inflammatory bowel disease. Am J Ophthalmol 2002, 134:778–780.
Tilman O, Kaiser HJ, Killer HE: Pseudotumor cerebri in a patient with Goldenhar’s and Duane’s syndromes. Ophthalmologica 2002, 216:296–299.
Mokri B: Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clinic Proc 2002, 77:1241–1246.
Mochizuki K, Takahashi T, Kano M, et al.: Pseudotumor cerebri induced by minocycline therapy for acne vulgaris. Jap J Ophthalmol 2002, 46:668–672.
Ang ER, Zimmerman JC, Malkin E: Pseudotumor cerebri secondary to minocycline intake. J Am Board Family Pract 2002, 15:229–233.
Collucciello M: Pseudotumor cerebri induced by all-trans retinoic acid treatment of acute promyelocytic leukemia. Arch Ophthalmol 2003, 121:1064–1065.
Rosa N, Giamundo A, Jura A, et al.: Mesalazine-associated benign intracranial hypertension in a patient with ulcerative colitis. Am J Ophthalmol 2003, 136:212–213.
Siniclair N, Assaad N, Johnston I: Pseudotumour cerebri occurring in association with the Chiari malformation. J Clin Neurosci 2002, 9:99–101.
Choudri KA, Cooke C, Tan MH, Gray WJ: Papilloedema as the sole presenting feature of Chiari I malformation. Br J Neurosurg 2002, 16:398–400.
Vaphiades MS, Eggenberger ER, Miller NR, et al.: Resolution of papilledema after neurosurgical decompression of primary Chiari I malformation. Am J Ophthalmol 2002, 133:673–678.
Lee AG, Golnik K, Kardon R, et al.: Sleep apnea and intracranial hypertension in men. Ophthalmology 2002, 109:482–485.
Giuseffi V, Wall M, Siegel PZ, Roojas PB: Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991, 41:239–244.
Wall M, George D: Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain 1991, 114:155–180.
Friedman DI, Rausch EA: Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology 2002, 58:1551–1553.
Straussberg R, Harel L, Amir J: Pseudotumor cerebri manifesting as stiff neck and torticollis. Pediatr Neurol 2002, 26:225–227.
Stanley TV: Idiopathic intracranial hypertension presenting as hemiplegic migraine. Acta Paediatr 2002, 91:980–982.
Jacks AS, Miller NR: Spontaneous retinal venous pulsation: aetiology and significance. J Neurol Neurosurg Psychiatry 2003, 74:7–9. This article presents an argument that retinal venous pulsations are caused by variation in the pressure gradient along the retinal vein as it traverses the lamina cribrosa. The pressure gradient varies because of the difference in the pulse pressure between the intraocular space and the cerebrospinal fluid. The importance of this is that as the ICP rises, the intracranial pulse pressure rises to equal the intraocular pulse pressure and the spontaneous venous pulsations cease. Thus, it is shown that cessation of the spontaneous venous pulsation is a sensitive marker of raised ICP.
McCulley TJ, Lam BL, Bose S, Feuer WJ: The effect of optic disk edema on spontaneous venous pulsations. Am J Ophthalmol 2003, 135:706–708.
Huna-Baron R, Kupersmith MJ: Idiopathic intracranial hypertension in pregnancy. J Neurol 2002, 249:1078–1081.
Bandyopadhyay S, Jacobson DM: Clinical features of lateonset pseudotumor cerebri fulfilling the modified Dandy criteria. J Neuro-Ophthalmol 2002, 22:9–11.
Kesler A, Fattal-Valevski A: Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002, 17:745–748.
Weig SG: Asymptomatic idiopathic intracranial hypertension in young children. J Child Neurol 2002, 17:239–241.
Higgins JN, Owler BK, Cousins C, Pickard JD: Venous sinus stenting for refractory benign intracranial hypertension. Lancet 2002, 359:228–230.
Owler BK, Allan R, Parker G, Besser M: Pseudotumour cerebri, CSF rhinorrhoea and the role of venous sinus stenting in treatment. Br J Neurosurg 2003, 17:79–83.
Karakalios DG, Rekate HL, Khayata MH, Apostolides PJ: Elevated intracranial venous pressure as a universal mechanism in pseudotumor cererbi of various etiologies. Neurology 1996, 46:198–202.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Brazis, P.W. Pseudotumor cerebri. Curr Neurol Neurosci Rep 4, 111–116 (2004). https://doi.org/10.1007/s11910-004-0024-6
Issue Date:
DOI: https://doi.org/10.1007/s11910-004-0024-6