CLINICAL AND RADIOLOGICAL FINDINGS IN ARNOLD CHIARI MALFORMATION
Abstract
Background: The Chiari Malformation I (CMI) is a disorder of uncertain origin that has beentraditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum.The anomaly is a leading cause of syringomyelia and occurs inassociation with osseus abnormalities atthe craniovertebral junction. In contrast to other Chiari malformations, CMI tends to present in thesecond or third decade of life and is sometimes referred to as the ‘adult-type’ Chiari malformation. Theobjective was to document clinical and radiological findings in Arnold Chiari Malformation-I.Method: This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad atNeurosurgery Department during July 2008–July 2010. We examined a prospective cohort of 60symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine.Results: There were 40 female and 20 male patients. The age of onset was 24.9±15.8 years. Commonassociated radiological problems included syringomyelia (60%), scoliosis (25%), andbasilarinvagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%).The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellarcerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), andvarying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-likeepisodes, a Meniere’s disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances inthe absence of syringomyelia. Conclusion: These data support accumulating evidence that CMI is adisorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranialfossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mmdoes not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluiddisturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops,syringomyelia, and hydrocephalus) and direct compression of nervous tissue.Keywords: Arnold Chiari Malformation, Posterior cranial fossa, Cerebrospinal fluidReferences
Banerji NK, Millar JHD. Chiari malformation presenting in adult
life. Brain 1974;97:157–68.
Dyste GN, Menezes AH, VanGilder JC. Symptomatic Chiari
malformations: An analysis of presentation, management, and
long-term outcome. J Neurosurg 1989;71:159–168.
Milhorat TH: Cerebrospinal fluid physiology. In: Milhorat TH:
Hydrocephalus and the Cerebrospinal Fluid. Baltimore: Williams
& Wilkins; 1972. p.1–41.
Chiari H. Ueber Veranderungen des Kleinhirns in folge von
Hydrocephalie des Grosshirns. Dtsch Med Wochenschr
;17:1172–5.
Badie B, Mendoza D, Batzdorf U. Posterior fossa volume and
response to suboccipital decompression with Chiari I
malformation. Neurosurgery 1995;37:214–8.
Atkinson JLD, Kokmen E, Miller GM. Evidence of posterior
fossa hypoplasia in the familial variant of adult Chiari I
malformation: Case report. Neurosurgery 1998;42:401–4.
Pillay PK, Awad IA, Little JR, Hahn JF. Symptomatic Chiari
malformation in adults: A new classification based on magnetic
resonance imaging with clinical and prognostic significance.
Neurosurgery 1991;28:639–45.
Barry A, Patten BM, Stewart BH. Possible factors in the
development of the Arnold-Chiari malformation. J Neurosurg
;14:285–301.
Friede RL, Roessmann U: Chronic tonsillar herniation: An
attempt at classifying chronic herniations at the foramen
magnum. Acta Neuropathol (Berl) 1976;34:219–35.
Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y.
Pathogenesis of Chiari malformation: A morphometric study of
the posterior cranial fossa. J Neurosurg 1997;86:40–7.
National Organization of Rare Disorder: Directory. New
Fairfield, CT, National Organization of Rare Disorder, 1997.
Busis NA, Hochberg FH. Familial syringomyelia. J Neurol
Neurosurg Psychiatry 1985;48:936–8.
Bindal AK, Dunsker SB, Tew JM Jr. Chiari I malformation:
Classification and management. Neurosurgery 1995; 37:1069–
Pascual J, Oterino A, Berciano J. Headache in type I Chiari
malformation. Neurology 1992;42:1519–21.
Maroun FB, Jacob JC, Mangan M. The Chiari malformation in
adults. Can J Neurol Sci 1975;2:115–20.
Da Silva JA, Brito JC, Da Nobrega PV. Autonomic nervous
system disorders in 230 cases of basilar impression and ArnoldChiari deformity. Neurochirurgia 1992;35:183–8.
Williams B. On the pathogenesis of syringomyelia: A review. J R
Soc Med 1980;73:798–806.
Gripp KW, Scott CI Jr, Nicholson L, Magram G, Grissom LE.
Chiari malformation and tonsillar ectopia in twin brothers and
father with autosomal dominant spondylo-epiphyseal dysplasia
tarda. Skeletal Radiol 1997;26:131–3.
Pujol J, Roig C, Capdevilla A, Pou A, Marti-Vilalta JL,
Kulisevsky J, et al. Motion of the cerebellar tonsils in Chiari type
I malformation studied by CINE phase-contrast MRI. Neurology
;45:1746–53.
Published
Issue
Section
License
Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.
USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.
AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.
