Skip to main content

Advertisement

SpringerLink
Log in

Headache Attributable to Nonvascular Intracranial Disorders

  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Headache attributable to nonvascular intracranial disorder is a basket of multiple, partly complex, and very diverse idiopathic or secondary disorders. By definition, the headache has to occur in a close temporal relationship to the intracranial disorder. Some of these headache disorders are caused by high or low cerebrospinal fluid pressure; noninfectious inflammatory diseases such as neurosarcoidosis, aseptic (noninfectious) meningitis, and lymphocytic hypophysitis; or intracranial neoplasm. Other nonvascular headaches, including hemicrania epileptica and postseizure headache, Chiari malformation type I, and the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis, are attributed to hypothalamic or pituitary hyper- or hyposecretion, intrathecal injection, or epileptic seizures. The clinical presentation of all these disorders can be diverse and often mimics the characteristics of primary headaches, which may delay the diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Headache Classification Subcommittee of the International Headache Society. The international classification of headache disorders: 2nd edition. Cephalalgia. 2004;24 Suppl 1:9–160.

    Google Scholar 

  2. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. 1988;45:875–7.

    PubMed  CAS  Google Scholar 

  3. Radhakrishnan K, Ahlskog JE, Cross SA, Kurland LT, O’Fallon WM. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol. 1993;50:78–80.

    PubMed  CAS  Google Scholar 

  4. Kesler A, Gadoth N. Epidemiology of idiopathic intracranial hypertension in Israel. J Neuroophthalmol. 2001;21:12–4.

    PubMed  CAS  Google Scholar 

  5. Karahalios DG, 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.

    PubMed  CAS  Google Scholar 

  6. Johnston I, Kollar C, Dunkley S, Assaad N, Parker G. Cranial venous outflow obstruction in the pseudotumour syndrome: incidence, nature and relevance. J Clin Neurosci. 2002;9:273–8.

    Article  PubMed  CAS  Google Scholar 

  7. Friedmann DI. Pseudotumor cerebri. Neurol Clin North Am. 2004;22:99–131.

    Google Scholar 

  8. • Volcy M, Tepper SJ. Cluster-like headache secondary to idiopathic intracranial hypertension. Cephalalgia. 2006;26:883–6. This interesting case report shows the diversity of the clinical picture in idiopathic intracranial hypertension.

    Article  PubMed  CAS  Google Scholar 

  9. Friedman DI, Rausch EA. Headache diagnoses in patients with treated idiopathic intracranial hypertension. Neurology. 2002;58:1551–3.

    PubMed  Google Scholar 

  10. Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia. 2006;26:384–99.

    Article  PubMed  CAS  Google Scholar 

  11. Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain. 1991;114(Pt 1A):155–80.

    PubMed  Google Scholar 

  12. Round R, Keane JR. The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology. 1988;38:1461–4.

    PubMed  CAS  Google Scholar 

  13. Johnston I, Paterson A. Benign intracranial hypertension. II. CSF pressure and circulation. Brain. 1974;97:301–12.

    Article  PubMed  CAS  Google Scholar 

  14. Said RR, Rosman NP. A negative cranial computed tomographic scan is not adequate to support a diagnosis of pseudotumor cerebri. J Child Neurol. 2004;19:609–13.

    Article  PubMed  Google Scholar 

  15. King JO, Mitchell PJ, Thomson KR, Tress BM. Manometry combined with cervical puncture in idiopathic intracranial hypertension. Neurology. 2002;58:26–30.

    PubMed  CAS  Google Scholar 

  16. Kupersmith MJ, Gamell L, Turbin R, Peck V, Spiegel P, Wall M. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology. 1998;50:1094–8.

    PubMed  CAS  Google Scholar 

  17. Sinclair AJ, Burdon MA, Nightingale PG, Ball AK, Good P, Matthews TD,et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ 341:c2701.

  18. Celebisoy N, Gokcay F, Sirin H, Akyurekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116:322–7.

    Article  PubMed  CAS  Google Scholar 

  19. • Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. 2008;8:87–93. This is an excellent review on the pathophysiology and therapeutic options of idiopathic intracranial hypertension.

    Article  PubMed  Google Scholar 

  20. Pagan FL, Restrepo L, Balish M, Patwa HS, Houff S. A new drug for an old condition? Headache. 2002;42:695–6.

    Article  PubMed  Google Scholar 

  21. Agarwal MR, Yoo JH. Optic nerve sheath fenestration for vision preservation in idiopathic intracranial hypertension. Neurosurg Focus. 2007;23:E7.

    Article  PubMed  Google Scholar 

  22. Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. 1997;49:734–9.

    PubMed  CAS  Google Scholar 

  23. Bynke G, Zemack G, Bynke H, Romner B. Ventriculoperitoneal shunting for idiopathic intracranial hypertension. Neurology. 2004;63:1314–6.

    PubMed  Google Scholar 

  24. McGirt MJ, Woodworth G, Thomas G, Miller N, Williams M, Rigamonti D. Cerebrospinal fluid shunt placement for pseudotumor cerebri-associated intractable headache: predictors of treatment response and an analysis of long-term outcomes. J Neurosurg. 2004;101:627–32.

    Article  PubMed  Google Scholar 

  25. Sorensen PS, Krogsaa B, Gjerris F. Clinical course and prognosis of pseudotumor cerebri. A prospective study of 24 patients. Acta Neurol Scand. 1988;77:164–72.

    Article  PubMed  CAS  Google Scholar 

  26. Kesler A, Goldhammer Y, Hadayer A, Pianka P. The outcome of pseudotumor cerebri induced by tetracycline therapy. Acta Neurol Scand. 2004;110:408–11.

    Article  PubMed  CAS  Google Scholar 

  27. Rowe FJ, Sarkies NJ. Assessment of visual function in idiopathic intracranial hypertension: a prospective study. Eye (Lond). 1998;12(Pt 1):111–8.

    Google Scholar 

  28. Schievink WI, Morreale VM, Atkinson JL, Meyer FB, Piepgras DG, Ebersold MJ. Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg. 1998;88:243–6.

    Article  PubMed  CAS  Google Scholar 

  29. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295:2286–96.

    Article  PubMed  CAS  Google Scholar 

  30. Albayram S. Intracranial hypotension syndrome following chiropratic manipulation of the cervical spine. J Headache Pain. 2006;7:369–70. author reply 371–362.

    Article  PubMed  Google Scholar 

  31. Prasad S, El-Haddad G, Zhuang H, Khella S. Intracranial hypotension following chiropractic spinal manipulation. Headache. 2006;46:1456–8.

    Article  PubMed  Google Scholar 

  32. Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia–evolution of a concept. Mayo Clin Proc. 1999;74:1113–23.

    Article  PubMed  CAS  Google Scholar 

  33. Schievink WI, Tourje J. Intracranial hypotension without meningeal enhancement on magnetic resonance imaging. Case report. J Neurosurg. 2000;92:475–7.

    Article  PubMed  CAS  Google Scholar 

  34. Pavlin DJ, McDonald JS, Child B, Rusch V. Acute subdural hematoma–an unusual sequela to lumbar puncture. Anesthesiology. 1979;51:338–40.

    Article  PubMed  CAS  Google Scholar 

  35. Schievink WI, Maya MM, Moser FG, Tourje J. Spectrum of subdural fluid collections in spontaneous intracranial hypotension. J Neurosurg. 2005;103:608–13.

    Article  PubMed  Google Scholar 

  36. Koss SA, Ulmer JL, Hacein-Bey L. Angiographic features of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2003;24:704–6.

    PubMed  Google Scholar 

  37. Roll JD, Larson 3rd TC, Soriano MM. Cerebral angiographic findings of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 2003;24:707–8.

    PubMed  Google Scholar 

  38. Savoiardo M, Armenise S, Spagnolo P, De Simone T, Mandelli ML, Marcone A, et al. Dural sinus thrombosis in spontaneous intracranial hypotension: hypotheses on possible mechanisms. J Neurol. 2006;253:1197–202.

    Article  PubMed  Google Scholar 

  39. Alvarez-Linera J, Escribano J, Benito-Leon J, Porta-Etessam J, Rovira A. Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology. 2000;55:1895–7.

    PubMed  CAS  Google Scholar 

  40. Mokri B, Posner JB. Spontaneous intracranial hypotension: the broadening clinical and imaging spectrum of CSF leaks. Neurology. 2000;55:1771–2.

    PubMed  CAS  Google Scholar 

  41. Chiapparini L, Farina L, D’Incerti L, Erbetta A, Pareyson D, Carriero MR, et al. Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology. 2002;44:143–50. discussion 151–142.

    Article  PubMed  CAS  Google Scholar 

  42. Rabin BM, Roychowdhury S, Meyer JR, Cohen BA, LaPat KD, Russell EJ. Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol. 1998;19:1034–9.

    PubMed  CAS  Google Scholar 

  43. Liong WC, Constantinescu CS, Jaspan T. Intrathecal gadolinium-enhanced magnetic resonance myelography in the detection of CSF leak. Neurology. 2006;67:1522.

    Article  PubMed  Google Scholar 

  44. Chung SJ, Kim JS, Lee MC. Syndrome of cerebral spinal fluid hypovolemia: clinical and imaging features and outcome. Neurology. 2000;55:1321–7.

    PubMed  CAS  Google Scholar 

  45. Moriyama E, Ogawa T, Nishida A, Ishikawa S, Beck H. Quantitative analysis of radioisotope cisternography in the diagnosis of intracranial hypotension. J Neurosurg. 2004;101:421–6.

    Article  PubMed  Google Scholar 

  46. Chung SJ, Lee JH, Im JH, Lee MC. Short- and long-term outcomes of spontaneous CSF hypovolemia. Eur Neurol. 2005;54:63–7.

    Article  PubMed  Google Scholar 

  47. Kong DS, Park K, Nam DH, Lee JI, Kim JS, Eoh W, et al. Clinical features and long-term results of spontaneous intracranial hypotension. Neurosurgery. 2005;57:91–6. discussion 91–96.

    Article  PubMed  Google Scholar 

  48. Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001;57:1921–3.

    PubMed  CAS  Google Scholar 

  49. Udommongkol C, Rajchadara S, Choedamphai E. Successful epidural blood patch treatment for spontaneous low cerebrospinal pressure headache with multiple leakages in lumbar spinal matter. J Med Assoc Thai. 2005;88 Suppl 3:S271–4.

    PubMed  Google Scholar 

  50. Halpern S, Preston R. Postdural puncture headache and spinal needle design. Metaanalyses. Anesthesiology. 1994;81:1376–83.

    Article  PubMed  CAS  Google Scholar 

  51. Strupp M, Schueler O, Straube A, Von Stuckrad-Barre S, Brandt T. “Atraumatic” Sprotte needle reduces the incidence of post-lumbar puncture headaches. Neurology. 2001;57:2310–2.

    PubMed  CAS  Google Scholar 

  52. Strupp M, Brandt T, Muller A. Incidence of post-lumbar puncture syndrome reduced by reinserting the stylet: a randomized prospective study of 600 patients. J Neurol. 1998;245:589–92.

    Article  PubMed  CAS  Google Scholar 

  53. Wu XM, Todo H, Sugibayashi K. Effects of pretreatment of needle puncture and sandpaper abrasion on the in vitro skin permeation of fluorescein isothiocyanate (FITC)-dextran. Int J Pharm. 2006;316:102–8.

    Article  PubMed  CAS  Google Scholar 

  54. Nettis E, Calogiuri G, Colanardi MC, Ferrannini A, Tursi A. Drug-induced aseptic meningitis. Curr Drug Targets Immune Endocr Metabol Disord. 2003;3:143–9.

    Article  PubMed  CAS  Google Scholar 

  55. Jolles S, Sewell WA, Leighton C. Drug-induced aseptic meningitis: diagnosis and management. Drug Saf. 2000;22:215–26.

    Article  PubMed  CAS  Google Scholar 

  56. Tyler KL, Adler D. Twenty-eight years of benign recurring Mollaret meningitis. Arch Neurol. 1983;40:42–3.

    PubMed  CAS  Google Scholar 

  57. Bender A, Elstner M, Paul R, Straube A. Severe symptomatic aseptic chemical meningitis following myelography: the role of procalcitonin. Neurology. 2004;63:1311–3.

    PubMed  Google Scholar 

  58. Eustace S, Buff B. Magnetic resonance imaging in drug-induced meningitis. Can Assoc Radiol J. 1994;45:463–5.

    PubMed  CAS  Google Scholar 

  59. Coleman WS, Lischner HW, Grover WD. Recurrent aseptic meningitis without sequelae. J Pediatr. 1975;87:89–91.

    Article  PubMed  CAS  Google Scholar 

  60. Kinnman J, Kam-Hansen S, Link H, Norrby E. Studies on the humoral and cell-mediated immune response in a patient with Mollaret’s meningitis. J Neurol Sci. 1979;43:265–75.

    Article  PubMed  CAS  Google Scholar 

  61. Graman PS. Mollaret’s meningitis associated with acute Epstein-Barr virus mononucleosis. Arch Neurol. 1987;44:1204–5.

    PubMed  CAS  Google Scholar 

  62. Mora JS, Gimeno A. Mollaret meningitis: report of a case with recovery after colchicine. Ann Neurol. 1980;8:631–3.

    Article  PubMed  CAS  Google Scholar 

  63. Stamm AM, Livingston WK, Cobbs CG, Dismukes WE. Failure of colchicine in the treatment of Mollaret’s meningitis. Arch Intern Med. 1984;144:2265–6.

    Article  PubMed  CAS  Google Scholar 

  64. Schwartz JF, Balentine JD. Recurrent meningitis due to an intracranial epidermoid. Neurology. 1978;28:124–9.

    PubMed  CAS  Google Scholar 

  65. Crossley GH, Dismukes WE. Central nervous system epidermoid cyst: a probable etiology of Mollaret’s meningitis. Am J Med. 1990;89:805–6.

    Article  PubMed  CAS  Google Scholar 

  66. Tanaka S, Tatsumi KI, Kimura M, Takano T, Murakami Y, Takao T, et al. Detection of autoantibodies against the pituitary-specific proteins in patients with lymphocytic hypophysitis. Eur J Endocrinol. 2002;147:767–75.

    Article  PubMed  CAS  Google Scholar 

  67. Bellastella A, Bizzarro A, Coronella C, Bellastella G, Sinisi AA, De Bellis A. Lymphocytic hypophysitis: a rare or underestimated disease? Eur J Endocrinol. 2003;149:363–76.

    Article  PubMed  CAS  Google Scholar 

  68. O’Dwyer DT, Smith AI, Matthew ML, Andronicos NM, Ranson M, Robinson PJ, et al. Identification of the 49-kDa autoantigen associated with lymphocytic hypophysitis as alpha-enolase. J Clin Endocrinol Metab. 2002;87:752–7.

    Article  PubMed  Google Scholar 

  69. Takahashi N, Kakinuma H, Hamada K, Shimazaki K, Takahashi K, Niihata S, et al. Efficient screening for catalytic antibodies using a short transition-state analog and detailed characterization of selected antibodies. Eur J Biochem. 1999;261:108–14.

    Article  PubMed  CAS  Google Scholar 

  70. Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S. Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings. J Clin Endocrinol Metab. 1995;80:2302–11.

    Article  PubMed  CAS  Google Scholar 

  71. •• Levy MJ, Matharu MS, Meeran K, Powell M, Goadsby PJ. The clinical characteristics of headache in patients with pituitary tumours. Brain. 2005;128:1921–30. This is a great clinical description of headache associated with pituitary tumor, analyzing 84 patients.

    Article  PubMed  CAS  Google Scholar 

  72. Hashimoto K, Takao T, Makino S. Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J. 1997;44:1–10.

    Article  PubMed  CAS  Google Scholar 

  73. Kristof RA, Van Roost D, Klingmuller D, Springer W, Schramm J. Lymphocytic hypophysitis: non-invasive diagnosis and treatment by high dose methylprednisolone pulse therapy? J Neurol Neurosurg Psychiatry. 1999;67:398–402.

    Article  PubMed  CAS  Google Scholar 

  74. Cemeroglu AP, Blaivas M, Muraszko KM, Robertson PL, Vazquez DM. Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old girl: case report and review of the literature. Eur J Pediatr. 1997;156:684–8.

    Article  PubMed  CAS  Google Scholar 

  75. Pfund Z, Szapary L, Jaszberenyi O, Nagy F, Czopf J. Headache in intracranial tumors. Cephalalgia. 1999;19:787–90. discussion 765.

    Article  PubMed  CAS  Google Scholar 

  76. Forsyth PA, Posner JB. Headaches in patients with brain tumors: a study of 111 patients. Neurology. 1993;43:1678–83.

    PubMed  CAS  Google Scholar 

  77. Borgelt B, Gelber R, Kramer S, Brady LW, Chang CH, Davis LW, et al. The palliation of brain metastases: final results of the first two studies by the Radiation Therapy Oncology Group. Int J Radiat Oncol Biol Phys. 1980;6:1–9.

    PubMed  CAS  Google Scholar 

  78. Osoba D. Effects of temozolomide in malignant brain tumours. Lancet. 2000;356:342.

    Article  PubMed  CAS  Google Scholar 

  79. Andermann F. Migraine-epilepsy relationships. Epilepsy Res. 1987;1:213–26.

    Article  PubMed  CAS  Google Scholar 

  80. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44:1005–17.

    Article  PubMed  CAS  Google Scholar 

  81. Lowry JK, Snyder JJ, Lowry PW. Brain tumors in the elderly: recent trends in a Minnesota cohort study. Arch Neurol. 1998;55:922–8.

    Article  PubMed  CAS  Google Scholar 

  82. Pascual J, Oterino A, Berciano J. Headache in type I Chiari malformation. Neurology. 1992;42:1519–21.

    PubMed  CAS  Google Scholar 

  83. Stovner LJ. Headache associated with the Chiari type I malformation. Headache. 1993;33:175–81.

    Article  PubMed  CAS  Google Scholar 

  84. Alzate JC, Kothbauer KF, Jallo GI, Epstein FJ. Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus. 2001;11:E3.

    Article  PubMed  CAS  Google Scholar 

  85. McGirt MJ, Nimjee SM, Fuchs HE, George TM. Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformations. Neurosurgery. 2006;59:140–6. discussion 140–146.

    Article  PubMed  Google Scholar 

  86. Gomez-Aranda F, Canadillas F, Marti-Masso JF, Diez-Tejedor E, Serrano PJ, Leira R, et al. Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. A report of 50 cases. Brain. 1997;120(Pt 7):1105–13.

    Article  PubMed  Google Scholar 

  87. Pascual J, Valle N. Pseudomigraine with lymphocytic pleocytosis. Curr Pain Headache Rep. 2003;7:224–8.

    Article  PubMed  Google Scholar 

  88. Castels-van Daele M, Standaert L, Boel M, Smeets E, Colaert J, Desmyter J. Basilar migraine and viral meningitis. Lancet. 1981;1:1366.

    Article  PubMed  CAS  Google Scholar 

  89. Berg MJ, Williams LS. The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology. 1995;45:1648–54.

    PubMed  CAS  Google Scholar 

  90. Morrison DG, Phuah HK, Reddy AT, Dure LS, Kline LB. Ophthalmologic involvement in the syndrome of headache, neurologic deficits, and cerebrospinal fluid lymphocytosis. Ophthalmology. 2003;110:115–8.

    Article  PubMed  Google Scholar 

  91. International Headache Society. IHS Classification ICHD-II. Available at http://www.ihs-classification.org. Accessed March 2011.

Download references

Disclosures

No potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Department of Neurology, University of Duisburg-Essen, Hufelandstrasse 55, 45122, Essen, Germany

    Mark Obermann, Dagny Holle, Steffen Naegel & Hans-Christoph Diener

Authors
  1. Mark Obermann
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dagny Holle
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Steffen Naegel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hans-Christoph Diener
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mark Obermann.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Obermann, M., Holle, D., Naegel, S. et al. Headache Attributable to Nonvascular Intracranial Disorders. Curr Pain Headache Rep 15, 314–323 (2011). https://doi.org/10.1007/s11916-011-0193-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11916-011-0193-3

Keywords

Search

Navigation