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The Pathophysiology of Pseudotumor Cerebri: An Unsolved Puzzle

The Pathophysiology of Pseudotumor Cerebri: An Unsolved Puzzle Abstract It has been a long-standing challenge to clinicians and investigators to explain the pathophysiology of the diverse syndromes collectively termed benign intracranial pressure (BIH), or pseudotumor cerebri. The diverse etiologic factors associated with BIH include lateral or superior longitudinal sinus thrombosis, menstrual irregularities with obesity, a heterogenous group of endocrine disorders including adrenal insufficiency, hyperadrenalism, and corticosteroid hormone withdrawal, hyperparathyroidism, hypothyroidism, pregnancy, menarche, lupus, intoxication with vitamin A or chlordecone, tetracycline therapy, spinal cord tumors, and the Guillain-Barré syndrome.1 In many cases the syndrome occurs de novo in otherwise healthy persons. Although papilledema and intracranial hypertension without focal signs are the initial clinical findings, it seems highly unlikely that the mechanism of the intracranial hypertension in the heterogenous conditions just listed would be the same. With the exception of those patients with venous occlusion, the pathophysiology of the intracranial hypertension in the various forms of the disorder are References 1. Fishman RA: Cerebrospinal Fluid in Diseases of the Nervous System . Philadelphia, WB Saunders Co, 1980, pp 128-139. 2. Ropper AH, Marmarou A: Mechanism of pseudotumor in Guillain-Barré syndrome . Arch Neurol 1984;41:259-261.Crossref 3. Britton C, Boxhill C, Brust JCM, et al: Pseudotumor cerebri, empty sella syndrome, and adrenal adenoma . Neurology 1980;30:292-296.Crossref 4. Shapiro K, Marmarou A, Shulman K: Characterization of clinical CSF dynamics and neural axis compliance using the pressure-volume index . Ann Neurol 1980;7:508-514.Crossref 5. Navab M, Smith JE, Goodman DS: Rat plasma prealbumin: Metabolic studies on effects of vitamin A status and on tissue distribution . J Biol Chem 1977;252:14:5107-5114. 6. Sahs AL, Joynt RJ: Brain swelling of unknown cause . Neurology 1956;6:791-802.Crossref 7. Reid AC, Matheson MS, Teasdale G: Volume of the ventricles in benign intracranial hypertension . Lancet 1980;2:7-8.Crossref 8. Raichle ME, Grubb RL, Phelps ME, et al: Cerebral hemodynamics and metabolism in pseudotumor cerebri . Ann Neurol 1978;4:104-111.Crossref 9. McComb JG: Recent research into the nature of cerebrospinal fluid formation and absorption . J Neurosurg 1983;58:369-383.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

The Pathophysiology of Pseudotumor Cerebri: An Unsolved Puzzle

Fishman, Robert A.
Archives of Neurology , Volume 41 (3) – Mar 1, 1984
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Publisher
American Medical Association
Copyright
Copyright © 1984 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1984.04050150035011
Publisher site
See Article on Publisher Site

Abstract

Abstract It has been a long-standing challenge to clinicians and investigators to explain the pathophysiology of the diverse syndromes collectively termed benign intracranial pressure (BIH), or pseudotumor cerebri. The diverse etiologic factors associated with BIH include lateral or superior longitudinal sinus thrombosis, menstrual irregularities with obesity, a heterogenous group of endocrine disorders including adrenal insufficiency, hyperadrenalism, and corticosteroid hormone withdrawal, hyperparathyroidism, hypothyroidism, pregnancy, menarche, lupus, intoxication with vitamin A or chlordecone, tetracycline therapy, spinal cord tumors, and the Guillain-Barré syndrome.1 In many cases the syndrome occurs de novo in otherwise healthy persons. Although papilledema and intracranial hypertension without focal signs are the initial clinical findings, it seems highly unlikely that the mechanism of the intracranial hypertension in the heterogenous conditions just listed would be the same. With the exception of those patients with venous occlusion, the pathophysiology of the intracranial hypertension in the various forms of the disorder are References 1. Fishman RA: Cerebrospinal Fluid in Diseases of the Nervous System . Philadelphia, WB Saunders Co, 1980, pp 128-139. 2. Ropper AH, Marmarou A: Mechanism of pseudotumor in Guillain-Barré syndrome . Arch Neurol 1984;41:259-261.Crossref 3. Britton C, Boxhill C, Brust JCM, et al: Pseudotumor cerebri, empty sella syndrome, and adrenal adenoma . Neurology 1980;30:292-296.Crossref 4. Shapiro K, Marmarou A, Shulman K: Characterization of clinical CSF dynamics and neural axis compliance using the pressure-volume index . Ann Neurol 1980;7:508-514.Crossref 5. Navab M, Smith JE, Goodman DS: Rat plasma prealbumin: Metabolic studies on effects of vitamin A status and on tissue distribution . J Biol Chem 1977;252:14:5107-5114. 6. Sahs AL, Joynt RJ: Brain swelling of unknown cause . Neurology 1956;6:791-802.Crossref 7. Reid AC, Matheson MS, Teasdale G: Volume of the ventricles in benign intracranial hypertension . Lancet 1980;2:7-8.Crossref 8. Raichle ME, Grubb RL, Phelps ME, et al: Cerebral hemodynamics and metabolism in pseudotumor cerebri . Ann Neurol 1978;4:104-111.Crossref 9. McComb JG: Recent research into the nature of cerebrospinal fluid formation and absorption . J Neurosurg 1983;58:369-383.Crossref

Journal

Archives of Neurology – American Medical Association

Published: Mar 1, 1984

References

  • Cerebrospinal Fluid in Diseases of the Nervous System
    Fishman RA
  • Mechanism of pseudotumor in Guillain-Barré syndrome
    Ropper AH, Marmarou A
  • Pseudotumor cerebri, empty sella syndrome, and adrenal adenoma
    Britton C, Boxhill C, Brust JCM, et al
  • Characterization of clinical CSF dynamics and neural axis compliance using the pressure-volume index
    Shapiro K, Marmarou A, Shulman K
  • Rat plasma prealbumin: Metabolic studies on effects of vitamin A status and on tissue distribution
    Navab M, Smith JE, Goodman DS
  • Brain swelling of unknown cause
    Sahs AL, Joynt RJ
  • Volume of the ventricles in benign intracranial hypertension
    Reid AC, Matheson MS, Teasdale G
  • Cerebral hemodynamics and metabolism in pseudotumor cerebri
    Raichle ME, Grubb RL, Phelps ME, et al
  • Recent research into the nature of cerebrospinal fluid formation and absorption
    McComb JG