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Clinical Presentation of the Adult Chiari I Malformation

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The Chiari Malformations

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 which can be attributed to interference with normal 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 brain stem 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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Authors and Affiliations

  1. Department of Neurosurgery, Ronald Reagan UCLA Medical Center, 757 Westwood Plaza, Los Angeles, CA, 90095, USA

    Ulrich Batzdorf MD

  2. Department of Neurosurgery, Santa Monica – UCLA Medical Center and Orthopaedic Hospital, 1250 Sixteenth Street, Santa Monica, CA, 90404, USA

    Ulrich Batzdorf MD

  3. Department of Neurosurgery, David Geffen School of Medicine, University of California, 10833 LeConte Avenue, 6901, Los Angeles, CA, 90095-6901, USA

    Ulrich Batzdorf MD

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  1. Ulrich Batzdorf MD
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Correspondence to Ulrich Batzdorf MD .

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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

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Batzdorf, U. (2013). Clinical Presentation of the Adult 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_21

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  • DOI: https://doi.org/10.1007/978-1-4614-6369-6_21

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