Skip to main content
SpringerLink
Log in

Chiari Headache

  • Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Chiari malformations (CM) are a group of neuroanatomical pathologies resulting from overcrowding of the hindbrain. The purpose of this review is to characterize Chiari headache (CH) and describe diagnosis and treatment of the condition.

Recent Findings

Recent research has helped solidify the criteria for diagnosis of CH. Imaging studies have expanded our understanding of the morphological features producing them and helped to better characterize the pathophysiology. Additionally, identifying this unusual headache disorder accurately has helped with specific treatment options.

Summary

CH is a disabling condition which can effect multiple domains of a patient’s life. The diagnostic criteria has improved, and we now have a better understanding of the pathophysiology and imaging findings associated with CH. Future research is warranted to find new treatment options for individuals suffering from this condition.

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. Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA. The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat. 2014;28(2):184–94. https://doi.org/10.1002/ca.22442.

    Article  PubMed  Google Scholar 

  2. •• Garzon-Muvdi T, Jallo G, Poretti A, Ashmawy R, Huisman T, Raybaud C. Chiari type 1 deformity in children: pathogenetic, clinical, neuroimaging, and management aspects. Neuropediatrics. 2016;47(05):293–307. https://doi.org/10.1055/s-0036-1584563. An excellent review on the pathogenesis of Chiari type I malformations.

    Article  PubMed  Google Scholar 

  3. Taylor FR, Larkins MV. Headache and chiari i malformation: clinical presentation, diagnosis, and controversies in management. Curr Pain Headache Rep. 2002;6(4):331–7. https://doi.org/10.1007/s11916-002-0056-z.

    Article  PubMed  Google Scholar 

  4. Doberstein CA, Torabi R, Klinge PM. Current concepts in the pathogenesis, diagnosis, and management of type I Chiari malformations. Rhode Island Med J. 2017;100(6):47–9.

    Google Scholar 

  5. Grangeon L, et al. Predictive factors of headache resolution after Chiari type 1 malformation surgery. World Neurosurg. 2017;1878–8750(17):31797–7.

  6. Aitken LA, Lindan CE, Sidney S, Gupta N, Barkovich AJ, Sorel M, et al. Chiari type I malformation in a pediatric population. Pediatr Neurol. 2009;40(6):449–54. https://doi.org/10.1016/j.pediatrneurol.2009.01.003.

  7. Brickell KL. Ethnic differences in syringomyelia in New Zealand. J Neurol Neurosurg Psychiatry. 2006;77(8):989–91. https://doi.org/10.1136/jnnp.2005.081240.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  8. Noudel R, Jovenin N, Eap C, Scherpereel B, Pierot L, Rousseaux P. Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. J Neurosurg. 2009;111(5):1046–52. https://doi.org/10.3171/2009.2.jns08284.

    Article  PubMed  Google Scholar 

  9. IHS. The International Classification of Headache Disorders: 3rd edition (beta).

  10. IHS. The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;24(Suppl 1):9–160.

    Google Scholar 

  11. Toldo, I., Tangari, M., Mardari, R., Perissinotto, E., Sartori, S., Gatta, M., . . . Battistella, P. A. (2014). Headache in children with Chiari I malformation. Headache: J Head Face Pain, 54(5), 899–908. https://doi.org/10.1111/head.12341.

    Article  Google Scholar 

  12. Milhorat TH, Chou MW, Trinidad EM, Kotzen RM, Speer MC. Chiaril malformation: description of syndrome, clinical manifestation, and inheritance patterns in 364 symptomatic patients. Neurosurgery. 1998;43(3):674–4. https://doi.org/10.1097/00006123-199809000-00191.

  13. Sansur CA, Heiss JD, Devroom HL, Eskioglu E, Ennis R, Oldfield EH. Pathophysiology of headache associated with cough in patients with Chiari I malformation. J Neurosurg. 2003;98(3):453–8. https://doi.org/10.3171/jns.2003.98.3.0453.

    Article  PubMed  Google Scholar 

  14. • Alperin, N., Loftus, J. R., Oliu, C. J., Bagci, A. M., Lee, S. H., Ertl-Wagner, B., . . . Green, B. A. (2015). Imaging-based features of headaches in Chiari malformation type I. Neurosurgery, 77(1), 96–103. https://doi.org/10.1227/neu.0000000000000740. An important study investigating specific neuroradiological features characteristic of Chiari headaches.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Bapuraj, J. R., Londy, F. J., Delavari, N., Maher, C. O., Garton, H. J., Martin, B. A., . . . Quint, D. J. (2016). Cerebrospinal fluid velocity amplitudes within the cerebral aqueduct in healthy children and patients with Chiari I malformation. J Magn Reson Imaging, 44(2), 463–470. https://doi.org/10.1002/jmri.25160.

    Article  PubMed  Google Scholar 

  16. Bhadelia RA, Patz S, Heilman C, Khatami D, Kasper E, Zhao Y, et al. Cough-associated changes in CSF flow in Chiari I malformation evaluated by real-time MRI. Am J Neuroradiol. 2015;37(5):825–30. https://doi.org/10.3174/ajnr.a4629.

  17. Abu-Arafeh I, Campbell E. Headache, Chiari malformation type 1 and treatment options. Arch Dis Child. 2017;102(3):210–1. https://doi.org/10.1136/archdischild-2016-312137.

    Article  PubMed  Google Scholar 

  18. Beretta E, Vetrano IG, Curone M, Chiapparini L, Furlanetto M, Bussone G, et al. Chiari malformation-related headache: outcome after surgical treatment. Neurol Sci. 2017;38(S1):95–8. https://doi.org/10.1007/s10072-017-2950-5.

  19. •• Raza-Knight S, Mankad K, Prabhakar P, Thompson D. Headache outcomes in children undergoing foramen magnum decompression for Chiari I malformation. Arch Dis Childhood. 2017;102(3):238–43. https://doi.org/10.1136/archdischild-2016-310662. A very good study comparing the two surgical treatment options and outcomes for patients with Chiari headaches.

    Article  Google Scholar 

  20. Hong J, Roberts D. The surgical treatment of headache. Headache. 2014;54:409–29.

    Article  PubMed  Google Scholar 

  21. Mcgirt MJ, et al. Correlation of cerebrospinal fluid flow dynamics and headache in Chiari I malformation. Neurosurgery. 2005;56:716–21.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Yale School of Medicine, New Haven, USA

    Amit Mehta, Priyanka Chilakamarri, Adeel Zubair & Deena Kuruvilla

Authors
  1. Amit Mehta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Priyanka Chilakamarri
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Adeel Zubair
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Deena Kuruvilla
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Deena Kuruvilla.

Ethics declarations

Conflict of Interest

Amit Mehta, Priyanka Chilakamarri, and Adeel Zubair declare no conflict of interest. Deena Kuruvilla has been a speaker for Amgen Migraine program and a Neurology consultant for GLG consulting group, both outside of this work.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Uncommon and/or Unusual Headaches and Syndromes

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mehta, A., Chilakamarri, P., Zubair, A. et al. Chiari Headache. Curr Pain Headache Rep 22, 49 (2018). https://doi.org/10.1007/s11916-018-0702-8

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11916-018-0702-8

Keywords

Search

Navigation