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Headaches and Arnold-Chiari Syndrome: When to Suspect and How to Investigate

  • Uncommon Headache Syndromes (ME Bigal, Section editor)
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Abstract

Migraine and tension-type headache are common clinical problems, occurring even at a young age. When patients report headache as a symptom, it is necessary to exclude a secondary headache induced by an organic disease. Proper diagnosis and management of headache depends on a thorough history review and comprehensive clinical examination. A Chiari malformation is one organic cause that should not be overlooked. A thorough clinical screening is always recommended, including a complete neurological, mental status and physical examination. However, when the symptom pattern suggests a Chiari malformation, neuroimaging is warranted to identify correctly the pathologic condition and the most appropriate therapeutic approach. This paper reviews this condition, the signs and symptoms suggestive of its presence and how to arrive a the proper diagnosis.

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References

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

  1. Welch K, Shillito J, Strand E, Fischer EG, Winston KR. Chiari I malformations—an acquired disorder? J Neurosurg. 1981;55(4):604–9.

    Article  PubMed  CAS  Google Scholar 

  2. Atkinson JL, Weinshenker G, Miller GM, Piepgras DG, Mokri B. Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 1998;88(2):237–42.

    Article  PubMed  CAS  Google Scholar 

  3. Johnston I, Jacobson E, Besser M. The acquired Chiari malformation and syringomyelia following spinal CSF drainage: a study of incidence and management. Acta Neurochir (Wein). 1998;140(5):417–27.

    Article  CAS  Google Scholar 

  4. Morioka T, Shono T, Yoshida N, Hasuo K, Fukui M. acquired Chiari I malformation and syringomyelia associated with bilateral chronic subdural hematoma. Case report. J Nneurosurg. 1955;83(3):556–8.

    Google Scholar 

  5. •• De Souza MR, Zador Z, Frim D. Chiari malformation type 1: related conditions. Neurol Res. 2011;33(3):278–84. One of the most recent reviews concerning CMI and differential diagnosis.

    Google Scholar 

  6. •• Taylor F, Larkins M. Headache and Chiari I malformation: clinical presentation, diagnosis, and controversies in management. Curr Pain Headache Rep. 2002;6:331–7. Interesting paper concerning history, clinical aspect differential diagnosis and management of CMI.

    Article  PubMed  Google Scholar 

  7. Pascual J, Oterino A, Berciano J. Headache in type 1 Chiari malformation. Neurology. 1992;42(8):1519–21.

    Article  PubMed  CAS  Google Scholar 

  8. Pascual J, Iglesias F, Oterino A. Cough, exertional and sexual headaches: an analysiss of 72 benign and symptomatic cases. Neurology. 1996;46(6):1520–4.

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  10. Huang PP, Constantini S. Acquired Chiari I malformation. J Neurosurg. 1994;80:1099–102.

    Article  PubMed  CAS  Google Scholar 

  11. Haroun RI, Guarnieri M, Meadow JJ. Current opinions for the treatment of syringomyelia and Chiari malformations: survey of the pediatric section of the American Association of Neurological Surgeons. Pediatr Neurosurg. 2000;33(6):311–7.

    Article  PubMed  CAS  Google Scholar 

  12. Pearce JM. Hystorical note: Arnold Chiari or “Cruvilhier Cleland Chiari” malformation. J Neurol Neurosurg Psychiatry. 2000;68(1):13.

    Article  Google Scholar 

  13. Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorder, cranial neuralgias and facial pain. Cephalalgia. 1988;8 Suppl 7:1–96.

    Google Scholar 

  14. Elster AD, Chen MY. Chiari malformations: clinical and radiologic reappraisal. Radiology. 1992;183:347–53.

    PubMed  CAS  Google Scholar 

  15. Levy WJ, Mason L, Hahn JF. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery. 1983;12(4):377–90.

    Article  PubMed  CAS  Google Scholar 

  16. Aboulezz AO, Sartor K, Geyger CA, Gado MH. Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr. 1985;9(6):1033–6.

    Article  PubMed  CAS  Google Scholar 

  17. Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 1986;7(5):795–9.

    PubMed  CAS  Google Scholar 

  18. Meadows J, Kraut M, Guarnieri M. Asymptomatic Chiari type I malformation identified on magnetic resonance imaging. J Neurosurg. 2000;92(6):920–6.

    Article  PubMed  CAS  Google Scholar 

  19. Sansur CA, Heiss JD, DeVroom HL, et al. Pathophysiology of headache associated with cough in patients with chiari I malformation. J Neurosurg. 2008;98:453–8.

    Google Scholar 

  20. Kaplan Y, Oksuz E. Chronic migraine associated with the Chiari type I malformation. Clin Neurol Neurosurg. 2008;110:818–22.

    Article  PubMed  Google Scholar 

  21. Weinberg JS, Freed DL, Sadock J, et al. Headache and Chiari I malformation in the pediatric population. Pediatr Neurosurg. 1998;29:14–8.

    Article  PubMed  CAS  Google Scholar 

  22. Headache Classification Subcommittee. The international classification of headache disorders. Cephalalgia. 2004;24 Suppl 1:1–160.

    Google Scholar 

  23. Rothner D, Winner P. Headaches in children and adolescents. In: Silberstein SD, Lipton RB, Dalessio DJ (Eds) Wolff’s Headache and other head pain. 7th ed. New York: Oxford University Press; 2001. p. 539–61.

  24. Grazzi L, Usai S. Headache and Chiari malformation in young age: clinical aspects and differential diagnosis. Neurol Sci. 2011;32 Suppl 3:S299–301.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Neurological Institute C. Besta, Via Celoria 11, 20133, Milan, Italy

    Licia Grazzi

  2. Department of Psychology, University of Memphis, TN, 400 Innovation Drive, Memphis, TN, 38152, USA

    Frank Andrasik

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  1. Licia Grazzi
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  2. Frank Andrasik
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Correspondence to Licia Grazzi.

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Grazzi, L., Andrasik, F. Headaches and Arnold-Chiari Syndrome: When to Suspect and How to Investigate. Curr Pain Headache Rep 16, 350–353 (2012). https://doi.org/10.1007/s11916-012-0270-2

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