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Idiopathic Intracranial Hypertension Without Papilledema

Idiopathic Intracranial Hypertension Without Papilledema Abstract • We describe 10 patients with idiopathic intracranial hypertension who did not have papilledema. Idiopathic intracranial hypertension without papilledema, although rarely reported, may well be a clinically important headache syndrome. Historical and demographic features of patients with idiopathic intracranial hypertension without papilledema are similar to those of patients with papilledema. Obese women with chronic daily headache and symptoms of increased intracranial pressure, pulsatile tinnitus, history of head trauma or meningitis, an empty sella on imaging studies, or a headache that is unrelieved by standard therapy should have a diagnostic lumbar puncture. Findings from laboratory and neurologic investigations are normal in most patients with idiopathic intracranial hypertension without papilledema. Initial management should include removal of possible inciting agents, weight loss if applicable, and standard headache therapy. Lumbar puncture and diuretic therapy should precede a trial of corticosteroids. Surgery (lumboperitoneal or ventriculoperitoneal shunt or perhaps optic nerve sheath fenestration) may be indicated for prolonged incapacitating headache that is not responsive to medical management or lumbar puncture. References 1. Weisberg LA. Benign intracranial hypertension . Medicine (Baltimore) . 1975;54:197-207.Crossref 2. Repka MX, Savino PJ. Pseudotumor cerebri . Am J Ophthalmol . 1984;98:741-746. 3. Ahlskog JE, O'Neill BJ. Pseudotumor cerebri . Ann Intern Med . 1982;97:249-256.Crossref 4. Johnston I, Paterson A. Benign intracranial hypertension, diagnosis and prognosis . Brain . 1974;97:289-300.Crossref 5. Paterson R, Depasquale N, Mann S. Pseudotumor cerebri . Medicine (Baltimore) . 1961;40:85-99.Crossref 6. Foley J. Benign forms of intracranial hypertension—'toxic' and 'otitic' hydrocephalus . Brain . 1955;78:1-41.Crossref 7. Berg L, Rosomoff HL, Aronson N, Silberman M, Pool JL. The syndrome of increased intracranial pressure without localizing signs . Arch Neurol Psychiatry . 1955;74:498.Crossref 8. Langiftt TW. Increased intracranial pressure . Clin Neurosurg . 1969;16:436-468. 9. Quincke H. Uber Meningitis Serosa and verwandte Zustande . Dtsch Z Neruenheilkd . 1897;9:149.Crossref 10. Lipton HL, Michelson PE. Pseudotumor cerebri syndrome without papilledema . JAMA . 1972;220:1591-1592.Crossref 11. Sher NA, Wirtschaffer J, Shapiro SK, See C, Shapiro I. Unilateral papilledema in 'benign' intracranial hypertension (pseudotumor cerebri) . JAMA . 1983;250:2346-2347.Crossref 12. Maxner CE, Freedman MI, Corbett JJ. Asymmetric papilledema and visual loss in pseudotumor cerebri . Can J Neurol Sci . 1987;14:593-596. 13. Scanarini M, Mingrino S, d'Avella D, Della-Cort V. Benign intracranial hypertension without papilledema: a case report . Neurosurgery . 1979;5:376-377.Crossref 14. Spence JD, Amacher AL, Willis NR. Benign intracranial hypertension without papilledema: role of 24 hour cerebrospinal fluid pressure monitoring in diagnosis and management . Neurosurgery . 1980;7:326-336.Crossref 15. Corbett JJ. Diagnosis and management of idiopathic intracranial hypertension (pseudotumor cerebri) . In: Shults WT, ed. Focal Points 1989: Clinical Modules for Ophthalmologists . San Francisco, Calif: American Academy of Ophthalmology; 1989;7(module3):1-12. 16. Andres R. Mortality and obesity: the rationale for age-specific height-weight tables: Metropolitan Life Insurance Company-age specific recommendations . In: Andres R, Hazzard WR, Birman E, eds. Principles of Geriatric Medicine . New York, NY: McGraw-Hill International Book Co; 1985:1070. 17. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana . Arch Neurol . 1988;45:875-877.Crossref 18. Digre KB, Corbett JJ. Pseudotumor cerebri in men . Arch Neurol . 1988;45:866-872.Crossref 19. Greer M. Benign intracranial hypertension, VI: obesity . Neurology . 1965;15:382-388.Crossref 20. Foley KM, Posner JB. Does pseudotumor cerebri cause the empty sella syndrome? Neurology . 1975;25:565-569.Crossref 21. Donaldson JO, Binstock ML. Pseudotumor cerebri in an obese woman with Turner syndrome . Neurology . 1981;31:758-760.Crossref 22. Donaldson JO. Endocrinology of pseudotumor cerebri . Neurol Clin . 1986;4:919-927. 23. Giuseffi V, Wall M, Siegal PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study . Neurology . 1991;41:239-244.Crossref 24. Sismanis A. Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management . Laryngoscope . 1987;97:1-17.Crossref 25. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients . Brain . 1991;114:155-180.Crossref 26. Wall M. The headache profile of idiopathic intracranial hypertension . Cephalalgia . 1990;10:331-335.Crossref 27. Foley KM. Is benign intracranial hypertension a chronic disease? Neurology . 1977;27:388. 28. Walsh TJ, Garden JW, Gallagher B. Obliteration of retinal venous pulsations . Am J Ophthalmol . 1969;67:954-956. 29. Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri: follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss . Arch Neurol . 1982;39:461-474.Crossref 30. Corbett JJ, Jacobson DM, Mauer RC, Thompson HS. Enlargement of the blind spot caused by papilledema . Am J Ophthalmol . 1988;105:261-265. 31. Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension . Arch Neurol . 1989;46:1049-1051.Crossref 32. Corbett J, Nerad JA, Tse DT, Anderson RL. Results of optic nerve sheath fenestration for pseudotumor cerebri: the lateral orbitotomy approach . Arch Ophthalmol . 1988;106:1391-1397.Crossref 33. Bjerre P, Lindholm J, Gyldensted C. Pseudotumor cerebri: a theory on etiology and pathogenesis . Acta Neurol Scand . 1982;66:472-481.Crossref 34. Fishman RA. The pathophysiology of pseudotumor cerebri: an unsolved puzzle . Neurology . 1984;41:257-258. 35. Donaldson JO. Pathogenesis of pseudotumor cerebri syndromes . Neurology . 1981;31:877-880.Crossref 36. Janny P, Chazal J, Colnet G, Irthom B, Georget AM. Benign intracranial hypertension and disorders of CSF absorption . Surg Neurol . 1981;15:168-174.Crossref 37. Gjerris F, Sorenson S, Vorstrup S, Paulson OB. Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow in patients with benign intracranial hypertension (pseudotumor cerebri) . Ann Neurol . 1985;17:158-162.Crossref 38. Borgesen SE, Gjerris F. Relationships between intracranial pressure, ventricular size, and resistance to CSF outflow . J Neurosurg . 1987;67:535-539.Crossref 39. Johnston I. Reduced CSF absorption syndrome: reappraisal of benign intracranial hypertension and related conditions . Lancet . 1973;2:418-421.Crossref 40. Fishman RA. Pathophysiology of pseudotumor . Ann Neurol . 1979;5:496.Crossref 41. VanAlphen HAM. Migraine, a result of increased CSF pressure: a new pathophysiologic concept (preliminary report) . Neurosurg Rev . 1986;9:121-124.Crossref 42. Vassilouthis J, Uttley D. Benign intracranial hypertension: clinical features and diagnosis using computed tomography and treatment . Surg Neurol . 1979;12:389-392. 43. Silbergleit R, Jonck L, Gebarski SS, Hat-fielkd MK. Idiopathic intracranial hypertension (pseudotumor cerebri): MR imaging . Radiology . 1989;170:207-209.Crossref 44. George AE. Idiopathic intracranial hypertension: pathogenesis and the role of MR imaging . Radiology . 1989:170:21-22.Crossref 45. Weisberg LA. Computed tomography in benign intracranial hypertension . Neurology . 1985;35:175-178. 46. Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri . Neurology . 1983:33:1386-1388.Crossref 47. Alksne JF. Headache and brain tumor . In: Dalessio DJ, ed. Wolffs Headache and Other Head Pain . 4th ed. New York. NY: Oxford University Press; 1980:287-300. 48. Sorensen PS, Krogsaa B, Gjerris F. Clinical course and prognosis of pseudotumor cerebri: a prospective study of 24 patients . Acta Neurol Scand . 1988;77:164-172.Crossref 49. Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension . J Neurosurg . 1988;69:195-202.Crossref 50. Gutgold-Glen H, Kattah JC, Chavis RM. Reversible visual loss in pseudotumor cerebri . Arch Ophthalmol .1984;102:403-406.Crossref 51. VanderArk GD, Kempe LH, Smith DR. Pseudotumor cerebri treated . JAMA . 1971;217:1832-1834.Crossref 52. Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri . Arch Ophthalmol . 1988;106:1384-1390.Crossref 53. Tse DT, Nerad JA, Anderson RL, Corbett JJ. Optic nerve sheath fenestration in pseudotumor cerebri: a lateral orbitotomy approach . Arch Ophthalmol . 1988;106:1458-1462.Crossref 54. Brourman ND, Spoor TC, Ramocki JM. Optic nerve sheath decompression for pseudotumor . Arch Ophthalmol . 1988;106:1378-1383.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Idiopathic Intracranial Hypertension Without Papilledema

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Publisher
American Medical Association
Copyright
Copyright © 1991 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1991.00530160060014
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Abstract

Abstract • We describe 10 patients with idiopathic intracranial hypertension who did not have papilledema. Idiopathic intracranial hypertension without papilledema, although rarely reported, may well be a clinically important headache syndrome. Historical and demographic features of patients with idiopathic intracranial hypertension without papilledema are similar to those of patients with papilledema. Obese women with chronic daily headache and symptoms of increased intracranial pressure, pulsatile tinnitus, history of head trauma or meningitis, an empty sella on imaging studies, or a headache that is unrelieved by standard therapy should have a diagnostic lumbar puncture. Findings from laboratory and neurologic investigations are normal in most patients with idiopathic intracranial hypertension without papilledema. Initial management should include removal of possible inciting agents, weight loss if applicable, and standard headache therapy. Lumbar puncture and diuretic therapy should precede a trial of corticosteroids. Surgery (lumboperitoneal or ventriculoperitoneal shunt or perhaps optic nerve sheath fenestration) may be indicated for prolonged incapacitating headache that is not responsive to medical management or lumbar puncture. References 1. Weisberg LA. Benign intracranial hypertension . Medicine (Baltimore) . 1975;54:197-207.Crossref 2. Repka MX, Savino PJ. Pseudotumor cerebri . Am J Ophthalmol . 1984;98:741-746. 3. Ahlskog JE, O'Neill BJ. Pseudotumor cerebri . Ann Intern Med . 1982;97:249-256.Crossref 4. Johnston I, Paterson A. Benign intracranial hypertension, diagnosis and prognosis . Brain . 1974;97:289-300.Crossref 5. Paterson R, Depasquale N, Mann S. Pseudotumor cerebri . Medicine (Baltimore) . 1961;40:85-99.Crossref 6. Foley J. Benign forms of intracranial hypertension—'toxic' and 'otitic' hydrocephalus . Brain . 1955;78:1-41.Crossref 7. Berg L, Rosomoff HL, Aronson N, Silberman M, Pool JL. The syndrome of increased intracranial pressure without localizing signs . Arch Neurol Psychiatry . 1955;74:498.Crossref 8. Langiftt TW. Increased intracranial pressure . Clin Neurosurg . 1969;16:436-468. 9. Quincke H. Uber Meningitis Serosa and verwandte Zustande . Dtsch Z Neruenheilkd . 1897;9:149.Crossref 10. Lipton HL, Michelson PE. Pseudotumor cerebri syndrome without papilledema . JAMA . 1972;220:1591-1592.Crossref 11. Sher NA, Wirtschaffer J, Shapiro SK, See C, Shapiro I. Unilateral papilledema in 'benign' intracranial hypertension (pseudotumor cerebri) . JAMA . 1983;250:2346-2347.Crossref 12. Maxner CE, Freedman MI, Corbett JJ. Asymmetric papilledema and visual loss in pseudotumor cerebri . Can J Neurol Sci . 1987;14:593-596. 13. Scanarini M, Mingrino S, d'Avella D, Della-Cort V. Benign intracranial hypertension without papilledema: a case report . Neurosurgery . 1979;5:376-377.Crossref 14. Spence JD, Amacher AL, Willis NR. Benign intracranial hypertension without papilledema: role of 24 hour cerebrospinal fluid pressure monitoring in diagnosis and management . Neurosurgery . 1980;7:326-336.Crossref 15. Corbett JJ. Diagnosis and management of idiopathic intracranial hypertension (pseudotumor cerebri) . In: Shults WT, ed. Focal Points 1989: Clinical Modules for Ophthalmologists . San Francisco, Calif: American Academy of Ophthalmology; 1989;7(module3):1-12. 16. Andres R. Mortality and obesity: the rationale for age-specific height-weight tables: Metropolitan Life Insurance Company-age specific recommendations . In: Andres R, Hazzard WR, Birman E, eds. Principles of Geriatric Medicine . New York, NY: McGraw-Hill International Book Co; 1985:1070. 17. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri: population studies in Iowa and Louisiana . Arch Neurol . 1988;45:875-877.Crossref 18. Digre KB, Corbett JJ. Pseudotumor cerebri in men . Arch Neurol . 1988;45:866-872.Crossref 19. Greer M. Benign intracranial hypertension, VI: obesity . Neurology . 1965;15:382-388.Crossref 20. Foley KM, Posner JB. Does pseudotumor cerebri cause the empty sella syndrome? Neurology . 1975;25:565-569.Crossref 21. Donaldson JO, Binstock ML. Pseudotumor cerebri in an obese woman with Turner syndrome . Neurology . 1981;31:758-760.Crossref 22. Donaldson JO. Endocrinology of pseudotumor cerebri . Neurol Clin . 1986;4:919-927. 23. Giuseffi V, Wall M, Siegal PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study . Neurology . 1991;41:239-244.Crossref 24. Sismanis A. Otologic manifestations of benign intracranial hypertension syndrome: diagnosis and management . Laryngoscope . 1987;97:1-17.Crossref 25. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients . Brain . 1991;114:155-180.Crossref 26. Wall M. The headache profile of idiopathic intracranial hypertension . Cephalalgia . 1990;10:331-335.Crossref 27. Foley KM. Is benign intracranial hypertension a chronic disease? Neurology . 1977;27:388. 28. Walsh TJ, Garden JW, Gallagher B. Obliteration of retinal venous pulsations . Am J Ophthalmol . 1969;67:954-956. 29. Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri: follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss . Arch Neurol . 1982;39:461-474.Crossref 30. Corbett JJ, Jacobson DM, Mauer RC, Thompson HS. Enlargement of the blind spot caused by papilledema . Am J Ophthalmol . 1988;105:261-265. 31. Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension . Arch Neurol . 1989;46:1049-1051.Crossref 32. Corbett J, Nerad JA, Tse DT, Anderson RL. Results of optic nerve sheath fenestration for pseudotumor cerebri: the lateral orbitotomy approach . Arch Ophthalmol . 1988;106:1391-1397.Crossref 33. Bjerre P, Lindholm J, Gyldensted C. Pseudotumor cerebri: a theory on etiology and pathogenesis . Acta Neurol Scand . 1982;66:472-481.Crossref 34. Fishman RA. The pathophysiology of pseudotumor cerebri: an unsolved puzzle . Neurology . 1984;41:257-258. 35. Donaldson JO. Pathogenesis of pseudotumor cerebri syndromes . Neurology . 1981;31:877-880.Crossref 36. Janny P, Chazal J, Colnet G, Irthom B, Georget AM. Benign intracranial hypertension and disorders of CSF absorption . Surg Neurol . 1981;15:168-174.Crossref 37. Gjerris F, Sorenson S, Vorstrup S, Paulson OB. Intracranial pressure, conductance to cerebrospinal fluid outflow, and cerebral blood flow in patients with benign intracranial hypertension (pseudotumor cerebri) . Ann Neurol . 1985;17:158-162.Crossref 38. Borgesen SE, Gjerris F. Relationships between intracranial pressure, ventricular size, and resistance to CSF outflow . J Neurosurg . 1987;67:535-539.Crossref 39. Johnston I. Reduced CSF absorption syndrome: reappraisal of benign intracranial hypertension and related conditions . Lancet . 1973;2:418-421.Crossref 40. Fishman RA. Pathophysiology of pseudotumor . Ann Neurol . 1979;5:496.Crossref 41. VanAlphen HAM. Migraine, a result of increased CSF pressure: a new pathophysiologic concept (preliminary report) . Neurosurg Rev . 1986;9:121-124.Crossref 42. Vassilouthis J, Uttley D. Benign intracranial hypertension: clinical features and diagnosis using computed tomography and treatment . Surg Neurol . 1979;12:389-392. 43. Silbergleit R, Jonck L, Gebarski SS, Hat-fielkd MK. Idiopathic intracranial hypertension (pseudotumor cerebri): MR imaging . Radiology . 1989;170:207-209.Crossref 44. George AE. Idiopathic intracranial hypertension: pathogenesis and the role of MR imaging . Radiology . 1989:170:21-22.Crossref 45. Weisberg LA. Computed tomography in benign intracranial hypertension . Neurology . 1985;35:175-178. 46. Corbett JJ, Mehta MP. Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri . Neurology . 1983:33:1386-1388.Crossref 47. Alksne JF. Headache and brain tumor . In: Dalessio DJ, ed. Wolffs Headache and Other Head Pain . 4th ed. New York. NY: Oxford University Press; 1980:287-300. 48. Sorensen PS, Krogsaa B, Gjerris F. Clinical course and prognosis of pseudotumor cerebri: a prospective study of 24 patients . Acta Neurol Scand . 1988;77:164-172.Crossref 49. Johnston I, Besser M, Morgan MK. Cerebrospinal fluid diversion in the treatment of benign intracranial hypertension . J Neurosurg . 1988;69:195-202.Crossref 50. Gutgold-Glen H, Kattah JC, Chavis RM. Reversible visual loss in pseudotumor cerebri . Arch Ophthalmol .1984;102:403-406.Crossref 51. VanderArk GD, Kempe LH, Smith DR. Pseudotumor cerebri treated . JAMA . 1971;217:1832-1834.Crossref 52. Sergott RC, Savino PJ, Bosley TM. Modified optic nerve sheath decompression provides long-term visual improvement for pseudotumor cerebri . Arch Ophthalmol . 1988;106:1384-1390.Crossref 53. Tse DT, Nerad JA, Anderson RL, Corbett JJ. Optic nerve sheath fenestration in pseudotumor cerebri: a lateral orbitotomy approach . Arch Ophthalmol . 1988;106:1458-1462.Crossref 54. Brourman ND, Spoor TC, Ramocki JM. Optic nerve sheath decompression for pseudotumor . Arch Ophthalmol . 1988;106:1378-1383.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Apr 1, 1991

References