Fahr's Disease

Important
It is possible that the main title of the report Fahr's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

Cerebrovascular Ferrocalcinosis
Fahr Disease
Nonarteriosclerotic Cerebral Calcifications
SPD Calcinosis
Striopallidodentate Calcinosis
Idiopathic Basal Ganglia Calcification (IBGC)

Disorder Subdivisions

None

General Discussion

Fahr's Disease is a rare degenerative neurological disorder characterized by the presence of abnormal calcium deposits (calcifications) and associated cell loss in certain areas of the brain (e.g., basal ganglia). The condition is often referred to as idiopathic basal ganglia calcification or IBGC because there is no apparent explanation for such calcification in these brain regions (idiopathic). Associated symptoms include progressive deterioration of cognitive abilities (dementia) and loss of acquired motor skills. As the condition progresses, paralysis may develop that is associated with increased muscle stiffness (rigidity) and restricted movements (spastic paralysis). Additional abnormalities may include relatively slow, involuntary, continual writhing movements (athetosis) or chorea, a related condition characterized by irregular, rapid, jerky movements. In some affected individuals, there may also be gradual deterioration of the nerve fibers that transmit impulses from the retinas to the brain (optic atrophy), a condition associated with partial or near complete visual impairment.

According to reports in the medical literature, Fahr's Disease is often familial. Familial Fahr's Disease may be transmitted as an autosomal recessive trait or, in other affected families (kindreds), may have autosomal dominant inheritance. In other instances, the condition appears to occur randomly for unknown reasons (sporadically). Some experts suggest that the condition may sometimes result from an unidentified infection during pregnancy affecting the developing fetus (intrauterine infection).

Resources

Parkinson's Disease - Movement Disorders Group
Neurological Institute
Box 57
710 W. 168th Street
New York, NY 10032
Tel: (212)305-5779
Fax: (212)305-1304
Email: deleon@movdis.cis.columbia.edu

National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/

C-Mac Informational Services, Inc.
120 Clinton Lane
Cookeville, TN 38501-8946
Tel: (931)268-1201
Email: caregiver_cmi@hotmail.com
Internet: http://www.caregivernews.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
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.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 3/9/2000
Copyright 1989, 1996, 1999, 2000 National Organization for Rare Disorders, Inc.

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