Pseudotumor cerebri pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Pathophysiology
Physiology
- Cerebrospinal fluid lifecycle: 600 ml of CSF is produced daily. this amount will decrease with age. 2/3 of this fluid is produced by choroid plexus of brain ventricles and the other 1/3 by ependymal lining of ventricles. [1]
- CSF production is a energy consuming process and use Na/K ATPase to flow ions into lumen and attract water with it through aquaporins.[2] CSF flows out to subarachnoid granulation where it can be absorbed.[3] Some part of CSF flows into lymphatics of head and neck and some part absorbed through spine.[4][5]
Pathogenesis
- The exact pathogenesis of pseudotumor cerebri is not completely understood. Idiopathic intracranial hypertension or pseudotumor cerebri is defined by the symptoms of increased intracranial pressure which is the common final pathway of all IIH etiologies, without any evidence of Intracranial mass lesions.[6]
Any theory regarding the pathophysiology of this disease should explain the following statements:
- High incidence rate in women with childbearing age
- Reduced conductance to CSF outflow[7]
- Normal ventricle size and lack of hydrocephalus[8]
- No evidence of cerebral edema[9]
- The basic etiology behind every IIH pathogenesis theory is Increased production of CSF and reduced resorption.
- CSF Changes in Pseudotumor Cerebri: The production of CSF is constant so the main problem in IIH pathogenesis is related to CSF absorbtaion. Many studies suggested that increased resistant to CSF absorbtion in the main cause of IIH disease.[10][11][12][13]
- The venous sinuses: It has been suggested that primary or secondary venous outflow narrowing or can cause IIH.[14][15] One of the causes of venous sinuses narrowing is thrombotic event. In one of the IIH studies the amount of MTHFR was higher in IIH patients (38%) in comparison to control group (14%). This result suggests that thrombophilia-hypofibrinolysis can cause IIH. One other study demonstrated that fibrinogen level and RBC aggregation is higher in these patients.[16][17]
- Sodium and Water Regulation: Aldosterone and Vasopressin: In addition to kidneys, aldosterone also works on epithelial cells of choroid plexus inhancing the Na-K ATPase and CSF production. Based on this, primary or secondary hyperaldosteronism can cause IIH.[18][19] Like aldosterone, AVP also works on kidneys and CNS but it seems that the rise in AVP in IIH is the result of increase intracranial pressure not the cause.[20][21][22]
- Obesity: Some evidences suggest that obesity can increase intra abdominal and intracranial pressure and have a role in pathogenesis of IIH.[23] In a study on 7 obese women with IIH it was seen that weigh loss improved their symptoms.[24] In the other hand higher level of leptin (a protein released from adipose tissue) was found in IIH patients.[25] Obesity also cause increased plasminogen activator inhibitor activity which leads to hypofibrinolysis[26] In the other hand the amount of ANP and BNP decreases as BMI increases.[27]
- Vitamin A: both hypo and hypervitaminosis A are suggested to be related to IIH pathogenesis. Hypovitaminosis A can interfere with absorbtion of CSF and hypervitaminosis A can activate the mineralocorticoid receptor.[28][29]
- Corticosteroids: Corticosteroid withdrawal can decrease CSF absorbtion and increase intracranial pressure.[30]
- Sex hormones:Progesterone can increase CSF with its mineralocorticoid effect and estrogen can reduce cellular tight junctions and reduce CSF permeability.[16][31]
Genetics
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
References
- ↑ Redzic ZB, Preston JE, Duncan JA, Chodobski A, Szmydynger-Chodobska J (2005). "The choroid plexus-cerebrospinal fluid system: from development to aging". Curr. Top. Dev. Biol. 71: 1–52. doi:10.1016/S0070-2153(05)71001-2. PMID 16344101.
- ↑ Maren TH (1988). "The kinetics of HCO3- synthesis related to fluid secretion, pH control, and CO2 elimination". Annu. Rev. Physiol. 50: 695–717. doi:10.1146/annurev.ph.50.030188.003403. PMID 3132082.
- ↑ Tripathi BJ, Tripathi RC (May 1974). "Vacuolar transcellular channels as a drainage pathway for cerebrospinal fluid". J. Physiol. (Lond.). 239 (1): 195–206. PMC 1330945. PMID 4369428.
- ↑ Weller RO, Djuanda E, Yow HY, Carare RO (January 2009). "Lymphatic drainage of the brain and the pathophysiology of neurological disease". Acta Neuropathol. 117 (1): 1–14. doi:10.1007/s00401-008-0457-0. PMID 19002474.
- ↑ Edsbagge M, Tisell M, Jacobsson L, Wikkelso C (December 2004). "Spinal CSF absorption in healthy individuals". Am. J. Physiol. Regul. Integr. Comp. Physiol. 287 (6): R1450–5. doi:10.1152/ajpregu.00215.2004. PMID 15308484.
- ↑ Friedman DI, Jacobson DM (November 2002). "Diagnostic criteria for idiopathic intracranial hypertension". Neurology. 59 (10): 1492–5. PMID 12455560.
- ↑ Bercaw BL, Greer M (August 1970). "Transport of intrathecal 131-I risa in benign intracranial hypertension". Neurology. 20 (8): 787–90. PMID 5468773.
- ↑ Jacobson DM, Karanjia PN, Olson KA, Warner JJ (September 1990). "Computed tomography ventricular size has no predictive value in diagnosing pseudotumor cerebri". Neurology. 40 (9): 1454–5. PMID 2392235.
- ↑ Wall M, Dollar JD, Sadun AA, Kardon R (February 1995). "Idiopathic intracranial hypertension. Lack of histologic evidence for cerebral edema". Arch. Neurol. 52 (2): 141–5. PMID 7848121.
- ↑ Sklar FH, Reisch J, Elashvili I, Smith T, Long DM (September 1980). "Effects of pressure on cerebrospinal fluid formation: nonsteady-state measurements in dogs". Am. J. Physiol. 239 (3): R277–84. doi:10.1152/ajpregu.1980.239.3.R277. PMID 7435599.
- ↑ Johnston I, Paterson A (June 1974). "Benign intracranial hypertension. II. CSF pressure and circulation". Brain. 97 (2): 301–12. PMID 4434179.
- ↑ Gjerris F, Soelberg Sørensen P, Vorstrup S, Paulson OB (February 1985). "Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow in patients with benign intracranial hypertension (pseudotumor cerebri)". Ann. Neurol. 17 (2): 158–62. doi:10.1002/ana.410170209. PMID 3872097.
- ↑ Janny P, Chazal J, Colnet G, Irthum B, Georget AM (March 1981). "Benign intracranial hypertension and disorders of CSF absorption". Surg Neurol. 15 (3): 168–74. PMID 7221863.
- ↑ Owler BK, Parker G, Halmagyi GM, Johnston IH, Besser M, Pickard JD, Higgins JN (2005). "Cranial venous outflow obstruction and pseudotumor Cerebri syndrome". Adv Tech Stand Neurosurg. 30: 107–74. PMID 16350454.
- ↑ Bateman GA (2004). "Idiopathic intracranial hypertension: priapism of the brain?". Med. Hypotheses. 63 (3): 549–52. doi:10.1016/j.mehy.2004.03.014. PMID 15288385.
- ↑ 16.0 16.1 Glueck CJ, Aregawi D, Goldenberg N, Golnik KC, Sieve L, Wang P (February 2005). "Idiopathic intracranial hypertension, polycystic-ovary syndrome, and thrombophilia". J. Lab. Clin. Med. 145 (2): 72–82. doi:10.1016/j.lab.2004.09.011. PMID 15746649.
- ↑ Kesler A, Yatziv Y, Shapira I, Berliner S, Assayag EB (October 2006). "Increased red blood cell aggregation in patients with idiopathic intracranial hypertension. A hitherto unexplored pathophysiological pathway". Thromb. Haemost. 96 (4): 483–7. PMID 17003926.
- ↑ Weber KT (August 2003). "Aldosteronism revisited: perspectives on less well-recognized actions of aldosterone". J. Lab. Clin. Med. 142 (2): 71–82. doi:10.1016/S0022-2143(03)00062-3. PMID 12960953.
- ↑ Weber KT, Singh KD, Hey JC (July 2002). "Idiopathic intracranial hypertension with primary aldosteronism: report of 2 cases". Am. J. Med. Sci. 324 (1): 45–50. PMID 12120824.
- ↑ Seckl J, Lightman S (December 1988). "Cerebrospinal fluid neurohypophysial peptides in benign intracranial hypertension". J. Neurol. Neurosurg. Psychiatry. 51 (12): 1538–41. PMC 1032770. PMID 3221220.
- ↑ Seckl JR, Lightman SL (October 1987). "Intracerebroventricular arginine vasopressin causes intracranial pressure to rise in conscious goats". Brain Res. 423 (1–2): 279–85. PMID 3676810.
- ↑ Sørensen PS (August 1986). "Studies of vasopressin in the human cerebrospinal fluid". Acta Neurol. Scand. 74 (2): 81–102. PMID 3535347.
- ↑ Sugerman HJ, DeMaria EJ, Felton WL, Nakatsuka M, Sismanis A (August 1997). "Increased intra-abdominal pressure and cardiac filling pressures in obesity-associated pseudotumor cerebri". Neurology. 49 (2): 507–11. PMID 9270586.
- ↑ Sugerman HJ, Felton III WL, Sismanis A, Saggi BH, Doty JM, Blocher C, Marmarou A, Makhoul RG (April 2001). "Continuous negative abdominal pressure device to treat pseudotumor cerebri". Int. J. Obes. Relat. Metab. Disord. 25 (4): 486–90. PMID 11319651.
- ↑ Lampl Y, Eshel Y, Kessler A, Fux A, Gilad R, Boaz M, Matas Z, Sadeh M (May 2002). "Serum leptin level in women with idiopathic intracranial hypertension". J. Neurol. Neurosurg. Psychiatry. 72 (5): 642–3. PMC 1737898. PMID 11971053.
- ↑ Bowles LK, Cooper JA, Howarth DJ, Miller GJ, MacCallum PK (September 2003). "Associations of haemostatic variables with body mass index: a community-based study". Blood Coagul. Fibrinolysis. 14 (6): 569–73. doi:10.1097/01.mbc.0000061344.72909.44. PMID 12960611.
- ↑ Skau M, Goetze JP, Rehfeld JF, Jensen R (September 2010). "Natriuretic pro-peptides in idiopathic intracranial hypertension". Regul. Pept. 164 (2–3): 71–7. doi:10.1016/j.regpep.2010.05.009. PMID 20553977.
- ↑ Calhoun MC, Hurt HD, Eaton HD, Rousseau JE, Hall RC (September 1967). "Rates of formation and absorption of cerebrospinal fluid in bovine hypovitaminosis A". J. Dairy Sci. 50 (9): 1489–94. PMID 6070689.
- ↑ Hayes KC, McCombs HL, Faherty TP (1971). "The fine structure of vitamin A deficiency. II. Arachnoid granulations and CSF pressure". Brain. 94 (2): 213–24. PMID 5571041.
- ↑ Johnston I, Gilday DL, Hendrick EB (June 1975). "Experimental effects of steroids and steroid withdrawal on cerebrospinal fluid absorption". J. Neurosurg. 42 (6): 690–5. doi:10.3171/jns.1975.42.6.0690. PMID 1141965.
- ↑ Gorodeski GI (January 2007). "Estrogen decrease in tight junctional resistance involves matrix-metalloproteinase-7-mediated remodeling of occludin". Endocrinology. 148 (1): 218–31. doi:10.1210/en.2006-1120. PMC 2398688. PMID 17038551.