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Clinical Presentation of Adult Chiari I

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The Chiari Malformations

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Abstract

Adult patients with Chiari malformation, sometimes referred to as hindbrain descent, most frequently present with symptoms in their 20s and 30s. Symptoms include those that can be attributed to interference with normal cerebrospinal fluid (CSF) flow equalization. Exertionally related headaches, characteristic for Chiari malformation, are due to this mechanism and are also the most common of all Chiari-related symptoms. Symptoms may also be related to brainstem compression and compression of cerebellar connections, reflected in balance problems, visual problems, and autonomic symptoms. Symptoms related to traction on lower cranial nerves may include swallowing and voice problems and may contribute to sleep apnea. Chiari malformation and related symptoms may present as a manifestation of other diseases, including hypophosphatemic rickets, Paget’s disease, Crouzon’s disease, and achondroplasia. Secondary Chiari malformation may be seen with pseudotumor cerebri (idiopathic intracranial hypertension) and occult cerebrospinal fluid leaks.

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References

  1. Gardner WJ, Abdullah AF, McCormack LJ. The varying expressions of embryonal atresia of the fourth ventricle in adults. J Neurosurg. 1957;14:591–605.

    Article  CAS  PubMed  Google Scholar 

  2. Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg. 1998;80:212–6.

    Article  Google Scholar 

  3. Tubbs SR, Iskandar BJ, Bartolucci AA, Oakes WJ. A critical analysis of the Chiari 1.5 malformation. J Neurosurg Ped. 2004;101:179–83.

    Article  Google Scholar 

  4. Brockmeyer DL. The complex Chiari: issues and management strategies. Neurol Sci. 2011;32(Suppl 3): S347.

    Google Scholar 

  5. Meadows J, Kraut M, Guarnieri M, Haroun RI, Carson BC. Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92:920–6.

    Article  CAS  PubMed  Google Scholar 

  6. Gagnadoux F, Meslier N, Svab I, Menei P, Racineux JL. Sleep-disordered breathing in patients with Chiari malformation: improvement after surgery. Neurology. 2006;66:136–8.

    Article  CAS  PubMed  Google Scholar 

  7. Nogués M, Gené R, Benarroch E, Leiguarda R, Calderón C, Encabo H. Respiratory disturbances during sleep in syringomyelia and syringobulbia. Neurology. 1999;52:1777–83.

    Article  PubMed  Google Scholar 

  8. Paul KS, Lye RH, Strang F, Dutton J. Arnold-Chiari malformation. J Neurosurg. 1983;58:183–7.

    Article  CAS  PubMed  Google Scholar 

  9. Saez RJ, Onofrio BM, Yanagihara T. Experience with Arnold-Chiari malformation, 1960 to 1970. J Neurosurg. 1976;45:416–22.

    Article  CAS  PubMed  Google Scholar 

  10. Ellenbogen RG, Armonda RA, Shaw DWW, Winn HR. Toward a rational treatment of Chiari I malformation and syringomyelia. Neurosurg Focus. 2000;8(3):Article 6, 1–10.

    Google Scholar 

  11. Mampalam TJ, Andrews BT, Gelb D, Ferriero D, Pitts LH. Presentation of Type I Chiari malformation after head trauma. Neurosurgery. 1988;23(6):760–2.

    Article  CAS  PubMed  Google Scholar 

  12. 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.

    CAS  PubMed  Google Scholar 

  13. Dyste GN, Menezes AH, VanGilder JC. Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg. 1989;71:159–68.

    Article  CAS  PubMed  Google Scholar 

  14. Bell HS. Paralysis of both arms from injury of the upper portion of the pyramidal decussation: “cruciate paralysis”. J Neurosurg. 1970;33:376–80.

    Article  CAS  PubMed  Google Scholar 

  15. Bejjani GK, Cockerham KP. Adult Chiari Malformation. Contemp Neurosurg. 2001;23(26):1–7.

    Article  Google Scholar 

  16. Dobkin BH. The adult Chiari malformation. Bull Los Angel Neurol Soc. 1977;42(1):23–7.

    CAS  Google Scholar 

  17. Kumar A, Patni AH, Charbel F. The Chiari malformation and the Neurotologist. Otol Neurotol. 2002;23:727–35.

    Article  PubMed  Google Scholar 

  18. Menezes AH, Smoker WRK, Dyste GN. Syringomyelia, Chiari malformations, and hydromyelia. Chapter 46. In: Youmans JR, editor. Neurological surgery. 3rd ed. Philadelphia: WB Saunders Co.; 1990. p. 1421–59.

    Google Scholar 

  19. Foster JB, Hudgson P. The clinical features of communicating Syringomyelia. In: Barnett HJM, Foster JB, Hudgson P, editors. Syringomyelia. London: WB Saunders Co; 1973. p. 16–29.

    Google Scholar 

  20. Mohr PD, Strang FA, Sambrook MA, Boddie HG. The clinical and surgical features in 40 patients with primary cerebellar ectopia (adult Chiari malformation). Quart J Med (New Series). 1977;46:85–96.

    CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  22. Weig SG, Buckthal PE, Choi SK, Zellem RT. Recurrent syncope as the presenting symptom of Arnold-Chiari malformation. Neurology. 1991;41:1673–4.

    Article  CAS  PubMed  Google Scholar 

  23. Goncalves da Silva JA, de Farias Britto JC, Virgolino da Nóbrega P. Neurovegetative Störungen bei 230 Fällen basilärer Impression und Arnold-Chiarischer Miβbildung. Neurochirurgia. 1992;35:183–8.

    Google Scholar 

  24. Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ. A critical analysis of the Chiari 1.5 malformation. J Neurosurg (Ped 2). 2004;101:179–83.

    Google Scholar 

  25. Levitt P, Cohn MA. Sleep apnea and the Chiari I malformation: case report. Neurosurgery. 1988;23:508–10.

    Article  CAS  PubMed  Google Scholar 

  26. Friede RL, Roessmann U. Chronic tonsillar herniation. Acta Neuropathol. 1976;34:219–35.

    Article  CAS  PubMed  Google Scholar 

  27. Williams B. Chronic herniation of the hindbrain. Ann R Coll Surg Engl. 1981;63(1):9–17.

    CAS  PubMed  PubMed Central  Google Scholar 

  28. Penfield W, Coburn DF. Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatr. 1938;40:328–36.

    Article  Google Scholar 

  29. Kerr FWL. A mechanism to account for frontal headache in cases of posterior-fossa tumors. J Neurosurg. 1961;18:605–9.

    Article  CAS  PubMed  Google Scholar 

  30. Caldemeyer KS, Boaz JC, Wappner RS, Moran CC, Smith RR, Quets JP. Chiari I malformation: association with hypophosphatemic rickets and MR imaging appearance. Radiology. 1995;195:733–8.

    Article  CAS  PubMed  Google Scholar 

  31. Tubbs RS, Webb D, Abdullatif H, Conklin M, Doyle S, Oakes WJ. Posterior cranial fossa volume in patients with rickets: insights into the increased occurrence of Chiari I malformation in metabolic bone disease. Neurosurgery. 2004;55:380–4.

    Article  PubMed  Google Scholar 

  32. Schmidek HH. Neurologic and neurosurgical sequelae of Paget’s disease of bone. Clin Orthop. 1977;127:70–7.

    Google Scholar 

  33. Elisevich K, Fontaine S, Bertrand G. Syringomyelia as a complication of Paget’s disease. J Neurosurg. 1987;66:611–3.

    Article  CAS  PubMed  Google Scholar 

  34. Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E, Pierre-Kahn A. Chronic tonsillar herniation in Crouzon’s and Apert’s syndromes: the role of premature synostosis of the lambdoid suture. J Neurosurg. 1995;83:575–82.

    Article  CAS  PubMed  Google Scholar 

  35. Nakai T, Asato R, Miki Y, Tanaka F, Matsumoto S, Konishi J. A case of achondroplasia with downward displacement of the brain stem. Neuroradiology. 1995;37:293–4.

    Article  CAS  PubMed  Google Scholar 

  36. Ryken TC, Menezes AH. Cervicomedullary compression in achondroplasia. J Neurosurg. 1994;81:43–8.

    Article  CAS  PubMed  Google Scholar 

  37. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA. Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue. J Neurosurg Spine. 2007;7:601–9.

    Article  PubMed  Google Scholar 

  38. Royo-Salvador MB, Solé-Lienas J, Doménech JM, González-Adrio R. Results of section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation. Acta Neurochir. 2005;147(5):513–23.

    Article  Google Scholar 

  39. Milhorat TH, Bolognese PA, Nishikawa M, Francomano CA, McDonnell NB, Roonprapunt C, et al. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009;72:20–35.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Hoffman HJ, Tucker WS. Cephalocranial disproportion. A complication of the treatment of hydrocephalus in children. Childs Brain. 1976;2:167–76.

    CAS  PubMed  Google Scholar 

  41. Welch K, Shillito J, Strand R, Fischer EG, Winston KR. Chiari I “malformations” – an acquired disorder? J Neurosurg. 1981;55:604–9.

    Article  CAS  PubMed  Google Scholar 

  42. Samii C, Möbius E, Weber W, Heienbrok HW, Berlit P. Pseudo Chiari type I malformation secondary to cerebrospinal fluid leakage. J Neurol. 1999;246:162–4.

    Article  CAS  PubMed  Google Scholar 

  43. Bergsneider M, Yang I, Hu X, McArthur DL, Cook SW, Boscardin WJ. Relationship between valve opening pressure, body position and intracranial pressure in normal pressure hydrocephalus: paradigm for selection of programmable valve pressure setting. Neurosurgery. 2004;55:851–3.

    Article  PubMed  Google Scholar 

  44. Jonesco-Sisesti N. Syringobulbia. A contribution to the pathophysiology of the brainstem. Translated by Ross RT (ed). New York: Praeger Publishers; 1986.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

    Ulrich Batzdorf

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  1. Ulrich Batzdorf
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Correspondence to Ulrich Batzdorf .

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Editors and Affiliations

  1. Department of Neurosurgery, Tulane University School of Medicine, New Orleans, LA, USA

    R. Shane Tubbs

  2. Department of Neurosurgery, Aydın Adnan Menderes University School of Medicine, Efeler, Aydın, Turkey

    Mehmet Turgut

  3. Departments of Neurosurgery and Pediatrics, University of Alabama, Birmingham, AL, USA

    W. Jerry Oakes

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Batzdorf, U. (2020). Clinical Presentation of Adult Chiari I. In: Tubbs, R., Turgut, M., Oakes, W. (eds) The Chiari Malformations. Springer, Cham. https://doi.org/10.1007/978-3-030-44862-2_32

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  • DOI: https://doi.org/10.1007/978-3-030-44862-2_32

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  • Publisher Name: Springer, Cham

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  • Online ISBN: 978-3-030-44862-2

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