Corticobasal Degeneration

National Organization for Rare Disorders, Inc.

Skip to the navigation

Important
It is possible that the main title of the report Corticobasal Degeneration is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Summary

Corticobasal degeneration (CBD) is a rare progressive neurological disorder characterized by cell loss and deterioration of specific areas of the brain. Affected individuals often initially experience motor abnormalities in one limb that eventually spreads to affect all the arms and legs. Such motor abnormalities include muscle rigidity and the inability to perform purposeful or voluntary movements (apraxia). Affected individuals may have sufficient muscle power for manual tasks, but often have difficulty directing their movements appropriately. Although CBD was historically described as a motor disease, it is now recognized that cognitive and behavioral symptoms also herald CBD and not uncommonly predate motor symptoms. Initial symptoms typically appear in people during the sixth decade, and may include poor coordination or difficulty accomplishing goal-directed tasks (e.g., buttoning a shirt). The exact cause of corticobasal degeneration is unknown.

Introduction

Because signs and symptoms associated with corticobasal degeneration are frequently caused by other neurodegenerative disorders, researchers use the term "corticobasal syndrome" to indicate the clinical diagnosis based on signs and symptoms. The term "corticobasal degeneration" refers to those meeting the neuropathological criteria for the disorder at autopsy. This is an important distinction because clinicopathological series indicate that about less than half of patients diagnosed with corticobasal syndrome during life actually has corticobasal degeneration at autopsy.

Supporting Organizations

Association for Frontotemporal Degeneration (AFTD)

Radnor Station Building #2, Suite 320
290 King of Prussia Road
Radnor, PA 19087
Tel: (267)514-7221
Tel: (866)507-7222
Email: info@theaftd.org
Website: http://www.theaftd.org

CurePSP: Foundation for PSP l CBD & Related Brain Diseases

30 E. Padonia Road, Suite 201
Timonium, MD 21093
Tel: (410)785-7004
Fax: (410)785-7009
Tel: (800)457-4777
Email: info@curepsp.org
Website: http://www.curepsp.org

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
Website: http://rarediseases.info.nih.gov/GARD/

NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Website: http://www.ninds.nih.gov/

Parkinson's Disease Foundation, Inc.

1359 Broadway
Suite 1509
New York, NY 10018
Tel: (212)923-4700
Fax: (212)923-4778
Tel: (800)457-6676
Email: info@pdf.org
Website: http://www.pdf.org

UCSF Memory and Aging Center

350 Parnassus Avenue
Suite 905
San Francisco, CA 94117
Tel: (415) 353-2057
Fax: (415)476-4800
Email: mgeschwind@memory.ucsf.edu
Website: http://www.memory.ucsf.edu

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  11/26/2012
Copyright  2012 National Organization for Rare Disorders, Inc.