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Published in: Current Pain and Headache Reports 6/2015

01-06-2015 | Imaging (L Mechtler, Section Editor)

Neuroimaging and the Clinical Manifestations of Chiari Malformation Type I (CMI)

Authors: Jennifer Williams McVige, Jody Leonardo

Published in: Current Pain and Headache Reports | Issue 6/2015

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Abstract

Chiari malformation type I (CMI) involves the caudal displacement of the cerebellar tonsils through the foramen magnum with resultant brainstem compression in some individuals. Due to pathophysiologic changes, secondary conditions may arise, such as syringohydromyelia (SH) and scoliosis. This disorder is unique, as the diagnosis is confirmed through radiologic findings. At times CMI is discovered incidentally on neuroimaging, but more frequently a patient will present with specific symptoms, the most common being a prototypic occipital headache. Although the true etiology of this complex condition remains speculative, the advent of neuroimaging has allowed for clarification of the enigmatic relationship between cerebrospinal fluid (CSF) dynamics, neuroanatomical compression, and clinical symptoms. Recent advancements in magnetic resonance imaging (MRI) such as diffusion tensor imaging (DTI) and CSF flow studies show promise in clarifying the underlying fluid dynamics in CMI patients and can aid in the prognosis and diagnosis of this complex disorder.
Literature
2.
go back to reference Meadows J, Kraut M, Guarnieri M, et al. Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92:920–6.PubMedCrossRef Meadows J, Kraut M, Guarnieri M, et al. Asymptomatic Chiari type I malformations identified on magnetic resonance imaging. J Neurosurg. 2000;92:920–6.PubMedCrossRef
3.
go back to reference Speer MC, Enterline DS, Mehltretter L, et al. Chiari I malformation with or without syringomyelia: prevelance and genetics. J Genet Couns. 2003;12:297–311.CrossRef Speer MC, Enterline DS, Mehltretter L, et al. Chiari I malformation with or without syringomyelia: prevelance and genetics. J Genet Couns. 2003;12:297–311.CrossRef
4.•
go back to reference McVige J, Leonardo J. Imaging of chiari type I malformation and syringohydromyelia. Neurol Clin. 2014;32:95–126. The most current overview of Chiari Malfomation and Neuroimaging. McVige J, Leonardo J. Imaging of chiari type I malformation and syringohydromyelia. Neurol Clin. 2014;32:95–126. The most current overview of Chiari Malfomation and Neuroimaging.
5.
go back to reference Alperin N, Sivarmakrishnan A, Lichtor T. Magnetic resonance imaging-based measurements of cerebrospinal fluid and blood flow as indicators of intracranial compliance in patients with Chiari malformation. J Neurosurg. 2005;103:46–52.PubMedCrossRef Alperin N, Sivarmakrishnan A, Lichtor T. Magnetic resonance imaging-based measurements of cerebrospinal fluid and blood flow as indicators of intracranial compliance in patients with Chiari malformation. J Neurosurg. 2005;103:46–52.PubMedCrossRef
6.
go back to reference Osborn, A. Diagnostic Radiology. Mosby, 1994 Osborn, A. Diagnostic Radiology. Mosby, 1994
7.
go back to reference Guinto G, Zamorano C, Dominguez F, et al. Chiari I malformation: part 1. Contemp Neurosurg. 2004;26:1–7. Guinto G, Zamorano C, Dominguez F, et al. Chiari I malformation: part 1. Contemp Neurosurg. 2004;26:1–7.
8.
go back to reference Barkovich AJ. Pediatric neuroimaging. 4th ed. Philadelphia: Lippincott; 2005. p. 932. Barkovich AJ. Pediatric neuroimaging. 4th ed. Philadelphia: Lippincott; 2005. p. 932.
9.•
go back to reference Massimi L, Novegno F, di Rocco C. Chiari type I malformation in children. Adv Tech Stand Neurosurg. 2011;37:143–211. Age related changes seen in Chiari Malformation. How the appearance may change over time. Massimi L, Novegno F, di Rocco C. Chiari type I malformation in children. Adv Tech Stand Neurosurg. 2011;37:143–211. Age related changes seen in Chiari Malformation. How the appearance may change over time.
10.
go back to reference Mikulis DJ, Diaz O, Egglin TK, et al. Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology. 1992;183:725–8.PubMedCrossRef Mikulis DJ, Diaz O, Egglin TK, et al. Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology. 1992;183:725–8.PubMedCrossRef
11.•
go back to reference Chen K, Kuo M, Lee C, Tu Y. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report. Childs Nerv Syst. 2014. Discusses cerebellar tonsillar descent due to secondary causes. Chen K, Kuo M, Lee C, Tu Y. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report. Childs Nerv Syst. 2014. Discusses cerebellar tonsillar descent due to secondary causes.
12.
go back to reference Lewis DW, Ashwal S, Dahl G, Dorbad D, Hirtz D, Prenskyand A, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches, report of the quality standards subcommittee of the American Academy of Neurology and The Practice Committee of The Child Neurology Society. Neurology. 2002;59(4):490–8.PubMedCrossRef Lewis DW, Ashwal S, Dahl G, Dorbad D, Hirtz D, Prenskyand A, et al. Practice parameter: evaluation of children and adolescents with recurrent headaches, report of the quality standards subcommittee of the American Academy of Neurology and The Practice Committee of The Child Neurology Society. Neurology. 2002;59(4):490–8.PubMedCrossRef
13.
go back to reference Pascual J, Oterino A, Bercaino J. Headache in type I Chiari malformation. Neurology. 1992;42:1519–21.PubMedCrossRef Pascual J, Oterino A, Bercaino J. Headache in type I Chiari malformation. Neurology. 1992;42:1519–21.PubMedCrossRef
14.
go back to reference Silberstein SD, Lipton RB, Dodick DW. 2008. Wolff’s headache and other head pain. 85–760. New York: Oxford University Press. Silberstein SD, Lipton RB, Dodick DW. 2008. Wolff’s headache and other head pain. 85–760. New York: Oxford University Press.
15.
go back to reference Toldo I, Tangari M, Mardari R, Perissinotto E, Sartori S, Gatta M, et al. Children with Chiari I malformation. Headache: J Head Face Pain. 2014;54(5):899–908.CrossRef Toldo I, Tangari M, Mardari R, Perissinotto E, Sartori S, Gatta M, et al. Children with Chiari I malformation. Headache: J Head Face Pain. 2014;54(5):899–908.CrossRef
16.
go back to reference Wu YW, Chin CT, Chan KM, Barkovich, Ferriero DM. Pediatric Chiari I malformations - Do clinical and radiologic features correlate. Neurology. 1999;53(6). Wu YW, Chin CT, Chan KM, Barkovich, Ferriero DM. Pediatric Chiari I malformations - Do clinical and radiologic features correlate. Neurology. 1999;53(6).
17.
go back to reference Pujol J, Roig C, Capdevila A, Pou A, Mart-Vilalta JL, Kulisevsky J, et al. Motion of the cerebellar tonsils in Chiari type I malformation studied by cine phase-contrast MRI. Neurology. 1995;45(9):1746–53.PubMedCrossRef Pujol J, Roig C, Capdevila A, Pou A, Mart-Vilalta JL, Kulisevsky J, et al. Motion of the cerebellar tonsils in Chiari type I malformation studied by cine phase-contrast MRI. Neurology. 1995;45(9):1746–53.PubMedCrossRef
18.
go back to reference Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery. 1995;37:214–8.PubMedCrossRef Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery. 1995;37:214–8.PubMedCrossRef
20.
go back to reference Williams B. Cerebrospinal fluid pressure-gradients in spina bifida cystica, with special reference to the Arnold-Chiari malformation and aqueductal stenosis. Dev Med Child Neurol Suppl. 1975;35:138–50.PubMed Williams B. Cerebrospinal fluid pressure-gradients in spina bifida cystica, with special reference to the Arnold-Chiari malformation and aqueductal stenosis. Dev Med Child Neurol Suppl. 1975;35:138–50.PubMed
22.
go back to reference Aboulker J. Syringomyelia and intra-rachidian fluids. X. Rachidian fluid stasis. Neurochirurgie. 1979;25 Suppl 1:98–107.PubMed Aboulker J. Syringomyelia and intra-rachidian fluids. X. Rachidian fluid stasis. Neurochirurgie. 1979;25 Suppl 1:98–107.PubMed
23.
go back to reference Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg. 1999;91:553–62.PubMedCrossRef Heiss JD, Patronas N, DeVroom HL, et al. Elucidating the pathophysiology of syringomyelia. J Neurosurg. 1999;91:553–62.PubMedCrossRef
24.
go back to reference Bhadelia RA, Wopert SM. CSF flow dynamics in Chiari I malformation. AJNR. 2000;21:1564.PubMed Bhadelia RA, Wopert SM. CSF flow dynamics in Chiari I malformation. AJNR. 2000;21:1564.PubMed
25.
go back to reference Sivaramakrishnan A, Alperin N, Surapaneni S, et al. Evaluating the effect of decompression surgery on cerebrospinal fluid flow and intracranial compliance in patients with Chiari malformation with magnetic resonance imaging flow studies. Neurosurgery. 2004;55:1344–51.PubMedCrossRef Sivaramakrishnan A, Alperin N, Surapaneni S, et al. Evaluating the effect of decompression surgery on cerebrospinal fluid flow and intracranial compliance in patients with Chiari malformation with magnetic resonance imaging flow studies. Neurosurgery. 2004;55:1344–51.PubMedCrossRef
26.•
go back to reference Godzik J, Kelly MP, Radmanesh A, Kim D, Holekamp TF, Smyth MD, et al. Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr. 2014;13(4):368–74. Discusses the size of a syrinx as it relates to clinical findings, such as scoliosis. Godzik J, Kelly MP, Radmanesh A, Kim D, Holekamp TF, Smyth MD, et al. Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia. J Neurosurg Pediatr. 2014;13(4):368–74. Discusses the size of a syrinx as it relates to clinical findings, such as scoliosis.
27.
go back to reference Baker HL. Myelographic examination of the posterior fossa with positive contrast medium. Radiology. 1963;81:791–801.PubMedCrossRef Baker HL. Myelographic examination of the posterior fossa with positive contrast medium. Radiology. 1963;81:791–801.PubMedCrossRef
28.
go back to reference Tominaga T, Koshu K, Ogawa A, et al. Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation. Neurosurgery. 1991;28:883–5.PubMedCrossRef Tominaga T, Koshu K, Ogawa A, et al. Transoral decompression evaluated by cine-mode magnetic resonance imaging: a case of basilar impression accompanied by Chiari malformation. Neurosurgery. 1991;28:883–5.PubMedCrossRef
29.
go back to reference Ventureyra EC, Aziz HA, Vassilyadi M. The role of cine flow MRI in children with Chiari I malformation. Childs Nerv Syst. 2003;19:109–13.PubMed Ventureyra EC, Aziz HA, Vassilyadi M. The role of cine flow MRI in children with Chiari I malformation. Childs Nerv Syst. 2003;19:109–13.PubMed
30.•
go back to reference Vurdem UE, Acer N, Ertekin T. Analysis of the volumes of the posterior cranial fossa, cerebellum, and herniated tonsils using the stereological methods in patients with Chiari type I malformation. Sci World J. 2012;2012:616934. Assessing volumetric measure of the posterior fossa as a possible tool to predict prognosis in Chiari Type I Malformation. Vurdem UE, Acer N, Ertekin T. Analysis of the volumes of the posterior cranial fossa, cerebellum, and herniated tonsils using the stereological methods in patients with Chiari type I malformation. Sci World J. 2012;2012:616934. Assessing volumetric measure of the posterior fossa as a possible tool to predict prognosis in Chiari Type I Malformation.
31.•
go back to reference Eshetu T, Meoded A, Jallo GI, Carson BS, Huisman TA, Poretti A. Diffusion tensor imaging in pediatric Chiari type I malformation. Dev Med Child Neurol. 2014;56(8):742–8. Assessing the use of DTI as a predictor for level of severity in Chiari Malformation. Eshetu T, Meoded A, Jallo GI, Carson BS, Huisman TA, Poretti A. Diffusion tensor imaging in pediatric Chiari type I malformation. Dev Med Child Neurol. 2014;56(8):742–8. Assessing the use of DTI as a predictor for level of severity in Chiari Malformation.
32.•
go back to reference Losurdo A. The role of diffusion tensor imaging in paediatricChiari I malformation. Dev Med Child Neurol. 2014;56(8):706. Assessing the use of DTI as a predictor for level of severity in Chiari Malformation. Losurdo A. The role of diffusion tensor imaging in paediatricChiari I malformation. Dev Med Child Neurol. 2014;56(8):706. Assessing the use of DTI as a predictor for level of severity in Chiari Malformation.
33.
go back to reference Kizu O, Yamada K, Nishimura T. Proton chemical shift imaging in normal pressure hydrocephalus. Kizu O, Yamada K, Nishimura T. Proton chemical shift imaging in normal pressure hydrocephalus.
34.
go back to reference El-Ghandour NM. Long-term outcome of surgical management of adult Chiari I malformation. Childs Nerv Syst. 2008;24(11):1333–9.CrossRef El-Ghandour NM. Long-term outcome of surgical management of adult Chiari I malformation. Childs Nerv Syst. 2008;24(11):1333–9.CrossRef
35.
go back to reference McGirt MJ, Attenello FJ, Atiba A, et al. Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Headache: J Head Face Pain. 2014;54(5):899–908.CrossRef McGirt MJ, Attenello FJ, Atiba A, et al. Symptom recurrence after suboccipital decompression for pediatric Chiari I malformation: analysis of 256 consecutive cases. Headache: J Head Face Pain. 2014;54(5):899–908.CrossRef
Metadata
Title
Neuroimaging and the Clinical Manifestations of Chiari Malformation Type I (CMI)
Authors
Jennifer Williams McVige
Jody Leonardo
Publication date
01-06-2015
Publisher
Springer US
Published in
Current Pain and Headache Reports / Issue 6/2015
Print ISSN: 1531-3433
Electronic ISSN: 1534-3081
DOI
https://doi.org/10.1007/s11916-015-0491-2

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