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Ashizawa, Tetsuo; Xia, Guangbin

doi: 10.1212/CON.0000000000000362
Review Articles

Purpose of Review: This article introduces the background and common etiologies of ataxia and provides a general approach to assessing and managing the patient with ataxia.

Recent Findings: Ataxia is a manifestation of a variety of disease processes, and an underlying etiology needs to be investigated. Pure ataxia is rare in acquired ataxia disorders, and associated symptoms and signs almost always exist to suggest an underlying cause. While the spectrum of hereditary degenerative ataxias is expanding, special attention should be addressed to those treatable and reversible etiologies, especially potentially life-threatening causes. This article summarizes the diseases that can present with ataxia, with special attention given to diagnostically useful features. While emerging genetic tests are becoming increasingly available for hereditary ataxia, they cannot replace conventional diagnostic procedures in most patients with ataxia. Special consideration should be focused on clinical features when selecting a cost-effective diagnostic test.

Summary: Clinicians who evaluate patients with ataxia should be familiar with the disease spectrum that can present with ataxia. Following a detailed history and neurologic examination, proper diagnostic tests can be designed to confirm the clinical working diagnosis.

Address correspondence to Dr Tetsuo Ashizawa, 6670 Bertner Ave, Houston Methodist Research Institute, R11-117, Houston, TX 77030,

Relationship Disclosure: Dr Ashizawa receives research/grant support as principal investigator of studies for Ionis Pharmaceuticals, Inc (1515598769-CS2); the Myotonic Dystrophy Foundation; and the National Institutes of Health (NS083564). Dr Xia receives research/grant support as principal investigator of studies for Acorda Therapeutics, Grand Aerie Fraternal Order of Eagles, the National Institutes of Health (AR065836-01), ReproCell Inc, and ThermoFisher Scientific.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Ashizawa and Xia report no disclosures.

Supplemental digital content: Videos accompanying this article are cited in the text as Supplemental Digital Content. Videos may be accessed by clicking on links provided in the HTML, PDF, and app versions of this article; the URLs are provided in the print version. Video legends begin on page 1225.

© 2016 American Academy of Neurology
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