National Organization for Rare Disorders, Inc.
Important
It is possible that the main title of the report Benign Essential Tremor
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
- Presenile Tremor Syndrome
- Tremor, Familial Essential
- Tremor, Hereditary Benign
Disorder Subdivisions
- None
General Discussion
Benign Essential Tremor is a neurologic movement disorder characterized by involuntary fine rhythmic tremor of a body part or parts, primarily the hands and arms (upper limbs). In many affected individuals, upper limb tremor may occur as an isolated finding. However, in others, tremor may gradually involve other anatomic regions, such as the head, voice, tongue, or roof of the mouth (palate), leading to difficulties articulating speech (dysarthria). Less commonly, tremor may affect muscles of the trunk or legs.
In individuals with the condition, tremor tends to occur while voluntarily maintaining a fixed posture against gravity ("postural tremor") or while performing certain goal-directed movements ("kinetic intention tremor"). Although tremor is typically absent with rest--i.e., when the affected muscle is not voluntary activated--some individuals with advanced disease may develop resting tremors.
Although symptom onset may occur during childhood or adolescence, the condition most commonly becomes apparent during adulthood, at an average age of 45 years. Benign Essential Tremor is generally considered a slowly progressive disorder. Disease progression is characterized by an increase in tremor amplitude, causing difficulties in performing fine motor skills and varying degrees of functional disability. For example, hand tremor may gradually cause difficulties with manipulating small objects, drinking fluids from a glass, eating, writing, or dressing. (As mentioned above, in some affected individuals, disease progression may also include extension of tremor to other muscle groups.)
Benign Essential Tremor may appear to occur randomly for unknown reasons (sporadically) or be transmitted as an autosomal dominant trait. Researchers suggest that changes (mutations) of different genes may be responsible for the disorder (genetic heterogeneity). For example, during genetic analysis of several affected families (kindreds), investigators located a gene for the disorder, known as "FET1," on the long arm (q) of chromosome 3 (3q13). In another kindred, the disorder was determined to result from mutations of a gene, designated "ETM2," on the short arm (p) of chromosome 2 (2p22-p25).
Resources
WE MOVE (Worldwide Education and Awareness for Movement Disorders)
204 West 84th Street
New York, NY 10024
USA
Tel: 2128758312
Fax: 2128758389
Email: wemove@wemove.org
Internet: http://www.wemove.org
International Essential Tremor Foundation
11111 West 95th Street
Suite 260
Overland Park, KS 66214-1824
USA
Tel: 9133413880
Fax: 9133411296
Tel: 8883873667
Email: Staff@essentialtremor.org
Internet: http://www.essentialtremor.org
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)428-7100
Fax: (914)997-4763
Tel: (888)663-4637
Email: Askus@marchofdimes.com
Internet: http://www.marchofdimes.com
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/
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: 3/27/2008
Copyright 1986, 1990, 1994, 1998, 2000, 2002 National Organization for Rare Disorders, Inc.
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