Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-cd9895bd7-dk4vv Total loading time: 0 Render date: 2024-12-27T22:36:50.386Z Has data issue: false hasContentIssue false

Chapter 16 - Episodic ataxias

Published online by Cambridge University Press:  06 December 2010

By
Monika Jeub ,
Thomas Klockgether  and
Michael Strupp
Edited by
Bettina Schmitz ,
Barbara Tettenborn  and
Donald L. Schomer
Bettina Schmitz
Affiliation:
Vivantes Humboldt-Klinikum, Berlin, Germany
Barbara Tettenborn
Affiliation:
Johannes Gutenberg Universität Mainz, Germany
Donald L. Schomer
Affiliation:
Harvard University, Massachusetts
Get access

Summary

Episodic ataxias are a clinically and genetically heterogeneous group of neurological disorders that are characterized by attacks of incoordination and imbalance. The prevalence of epilepsy in episodic ataxias type 1 (EA1) patients is approximately ten times that in the general population. To support a suspected diagnosis, the patient should undergo electromyography for myokymia. It should be explained that EA1 is in principle a benign condition. Episodic ataxias type 2 (EA2) is by far the most common episodic ataxia but precise data about the prevalence are missing. Based on the pathophysiological understanding of EA2, two pharmacologic approaches were established. They focus on modulation of the pH level and membrane ion conductance. Today, so far acetazolamide is the drug of choice for the preventive treatment of EA2. Aminopyridines may prevent attacks in EA2 (and improve downbeat nystagmus) by increasing the release of GABA in the Purkinje cells.

Keywords

anticonvulsant drugsaura symptomsdystonic movementssandifer syndromeparoxysmal dyskinesiasmovement disorderneurologic examination
Type
Chapter
Information
The Paroxysmal Disorders , pp. 196 - 208
Publisher: Cambridge University Press
Print publication year: 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Save book to Kindle

To save this book to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×