Skip to main content
SpringerLink
Log in

Unusual Presentations of the Chiari I Malformation

  • Chapter
  • First Online:
The Chiari Malformations

Abstract

Chiari type I malformations [CM-I] most commonly present with exertional occipital headaches and neck pain. In infants, these headaches may simply present as irritability. Cerebellar tonsil herniation can also cause compression of the medulla and lower cranial nerves leading to neurologic symptoms as well. Syringomyelia, commonly associated with CM-I, also can result in neurologic sequelae. This spectrum of symptoms includes extremity weakness, sensory deficits, and abnormal reflexes. Lower cranial nerves can also be affected resulting in vocal cord paralysis, tongue weakness, aspiration, hoarseness, nystagmus, palatal weakness, and sleep apnea.

Although the vast majority of patients with CM-I present with the symptoms listed above, there have been reports in the literature of more unusual presentations. This chapter will review the less common and more unusual presentations of CM-I, including syncope, hearing loss, cranial nerve neuralgias, respiratory failure, endocrinologic dysfunction, hiccups, esotropia, peripheral nerve syndromes, and acute sensory or motor deficits.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 239.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Heuer GG, Gabel B, Lemberg PS, Sutton LN. Chiari I malformation presenting with hearing loss: surgical treatment and literature review. Childs Nerv Syst. 2008;24(9):1063–6.

    Article  PubMed  Google Scholar 

  2. Hendrix RA, Bacon CK, Sclafani AP. Chiari-I malformation associated with asymmetric sensorineural hearing loss. J Otolaryngol. 1992;21:102–7.

    PubMed  CAS  Google Scholar 

  3. Papanastassiou AM, Schwartz RB, Friedlander RM. Chiari I malformation as a cause of trigeminal neuralgia: case report. Neurosurgery. 2008;63(3):E614–5; discussion E615.

    Article  PubMed  Google Scholar 

  4. Yglesias A, Narbona J, Vanaclocha V, et al. Chiari type I malformation, glossopharyngeal neuralgia and central sleep apnoea in a child. Dev Med Child Neurol. 1996;38:1126–30.

    Article  PubMed  CAS  Google Scholar 

  5. Yarbrough CK, Powers AK, Park TS, Leonard JR, Limbrick DD, Smyth MD. Patients with Chiari malformation type I presenting with acute neurological deficits: case series. J Neurosurg Pediatr. 2011;7(3):244–7.

    Article  PubMed  Google Scholar 

  6. Selmi F, Davies KG, Weeks RD. Type I Chiari deformity presenting with profound sinus bradycardia: case report and literature review. Br J Neurosurg. 1995;9:543–5.

    Article  PubMed  CAS  Google Scholar 

  7. Hampton F, Williams B, Loizou LA. Syncope as a presenting feature of hindbrain herniation with syringomyelia. J Neurol Neurosurg Psychiatry. 1982;45:919–22.

    Article  PubMed  CAS  Google Scholar 

  8. Titlic M, Jukic I, Tonkic A, Buca A, Dolic K. Vertigo associated with Chiari I malformation and syringomyelia. Bratisl Lek Listy. 2008;109(4):168–70.

    PubMed  CAS  Google Scholar 

  9. Alvarez D, Requena I, Arias M, Valdés L, Pereiro I, De la Torre R. Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia. Eur Respir J. 1995;8(4):661–3.

    PubMed  CAS  Google Scholar 

  10. Bokinsky GE, Hudson LD, Weil JV. Impaired peripheral chemosensitivity and acute respiratory failure in Arnold-Chiari malformation and syringomyelia. N Engl J Med. 1973;288(18):947–8.

    Article  PubMed  CAS  Google Scholar 

  11. Martinot A, Hue V, Leclerc F, et al. Sudden death revealing Chiari type 1 malformation in two children. Intensive Care Med. 1993;19:73–4.

    Article  PubMed  CAS  Google Scholar 

  12. Rekate HL, Nadkarni TD, Teaford PA, et al. Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism. Neurosurgery. 1999;45:386–91.

    Article  PubMed  CAS  Google Scholar 

  13. Interlici S, Montanari E, Conrieri M, et al. A patient with early puberty associated with Chiari 1 malformation. J Pediatr Endocrinol Metab. 2000;13:849–51.

    Article  PubMed  Google Scholar 

  14. Gingold SI, Winfield JA. Oscillopsia and primary cerebellar ectopia: case report and review of the literature. Neurosurgery. 1991;29:932–6.

    Article  PubMed  CAS  Google Scholar 

  15. Hentschel SJ, Yen KG, Lang FF. Chiari I malformation and acute acquired comitant esotropia: case report and review of the literature. J Neurosurg. 2005;102(4 Suppl):407–12. Review.

    PubMed  Google Scholar 

  16. Weeks CL, Hamed LM. Treatment of acute comitant esotropia in Chiari I malformation. Ophthalmology. 1999;106(12):2368–71.

    Article  PubMed  CAS  Google Scholar 

  17. Bixenman WW, Laguna JF. Acquired esotropia as initial manifestation of Arnold–Chiari malformation. J Pediatr Ophthalmol Strabismus. 1987;24:83–6.

    PubMed  CAS  Google Scholar 

  18. Lewis AR, Kline LB, Sharpe JA. Acquired esotropia due to Arnold–Chiari I malformation. J Neuroophthalmol. 1996;16:49–54.

    Article  PubMed  CAS  Google Scholar 

  19. Passo M, Shults WT, Talbot T, Palmer EA. Acquired esotropia. A manifestation of Chiari I malformation. J Clin Neuroophthalmol. 1984;4:151–4.

    Article  PubMed  CAS  Google Scholar 

  20. Loft LM, Ward RF. Hiccups. A case presentation and etiologic review. Arch Otolaryngol Head Neck Surg. 1992;118:1115–9.

    Article  PubMed  CAS  Google Scholar 

  21. Brill CB, Gutierrez J, Mishkin MM. Chiari I malformation: association with seizures and developmental disabilities. J Child Neurol. 1997;12:101–6.

    Article  PubMed  CAS  Google Scholar 

  22. Hudgins RJ. Paroxysmal rage as a presenting symptom of the Chiari I malformation. Report of two cases. J Neurosurg. 1999;91:328–9.

    Article  PubMed  CAS  Google Scholar 

  23. Callaway GH, O’Brien SJ, Tehrany AM. Chiari I malformation and spinal cord injury: cause for concern in contact athletes? Med Sci Sports Exerc. 1996;28:1218–20.

    Article  PubMed  CAS  Google Scholar 

  24. Scelsa SN. Syringomyelia presenting as ulnar neuropathy at the elbow. Clin Neurophysiol. 2000;111(9):1527–30.

    Article  PubMed  CAS  Google Scholar 

  25. Ziadeh MJ, Richardson JK. Arnold-Chiari malformation with syrinx presenting as carpal tunnel syndrome: a case report. Arch Phys Med Rehabil. 2004;85(1):158–61.

    Article  PubMed  Google Scholar 

  26. Laufer I, Engel M, Feldstein N, Souweidane MM. Chiari malformation presenting as a focal motor deficit. Report of two cases. J Neurosurg Pediatr. 2008;1(5):392–5.

    Article  PubMed  Google Scholar 

  27. Tubbs RS, Wellons JC, Blount JP, Oakes WJ, Grabb PA. Cessation of chronic hypertension after posterior fossa decompression in a child with Chiari I malformation. Case report. J Neurosurg. 2004;100:194–6.

    PubMed  Google Scholar 

  28. Stovner LJ, Sjaastad O. Segmental hyperhidrosis in two siblings with Chiari type I malformation. Eur Neurol. 1995;35:149–55.

    Article  PubMed  CAS  Google Scholar 

  29. Mukerji N, Newman P, Nath FP. Hemifacial spasm as a feature of Chiari malformation: case report. Neurosurgery. 2010;67:E1826–30.

    Article  PubMed  Google Scholar 

  30. Mortazavi MM, Tubbs RS, Harmon D, Oakes WJ. Chronic emesis due to compression of the area postrema by the posterior inferior cerebellar artery: resolution following microvascular decompression. J Neurosurg Pediatr. 2010;6:583–5.

    Article  PubMed  Google Scholar 

  31. Zeegers M, Van Der Grond J, Durston S, Nievelstein RJ, Witkamp T, Van Daalen E, et al. Radiological findings in autistic and developmentally delayed children. Brain Dev. 2006;28:495–9.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurological Surgery, University of Pittsburgh, UPMC Presbyterian, Suite B400, 200 Lothrop Street, Pittsburgh, PA, 15213, USA

    Christopher M. Bonfield MD

  2. Department of Neurological Surgery, Childrens Hospital of Pittsburgh of UPMC, 4401 Penn Avenue, Faculty Pavilion, 4th Floor, Pittsburgh, PA, 15224, USA

    Elizabeth Tyler-Kabara MD

Authors
  1. Christopher M. Bonfield MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Elizabeth Tyler-Kabara MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Elizabeth Tyler-Kabara MD .

Editor information

Editors and Affiliations

  1. Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    R. Shane Tubbs

  2. The Children's Hospital of Alabama, 7th Ave S 1600, Birmingham, 35233-1711, Alabama, USA

    W. Jerry Oakes

Rights and permissions

Reprints and permissions

Copyright information

© 2013 Springer Science+Business Media New York

About this chapter

Cite this chapter

Bonfield, C.M., Tyler-Kabara, E. (2013). Unusual Presentations of the Chiari I Malformation. In: Tubbs, R., Oakes, W. (eds) The Chiari Malformations. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6369-6_22

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_22

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-6368-9

  • Online ISBN: 978-1-4614-6369-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation