Benign Essential Tremor

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: (212)875-8312
Fax: (212)875-8389
Email: wemove@wemove.org
Internet: http://www.wemove.org

International Essential Tremor Foundation
P.O. Box 14005
Lenexa, KS 66285-4005
USA
Tel: (913)341-3880
Fax: (913)341-1296
Tel: (888)387-3667
Email: info@essentialtremor.org
Internet: http://www.essentialtremor.org

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)997-4488
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)
P.O. Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
TDD: (301)468-5981
Email: me20t@nih.gov
Internet: http://www.ninds.nih.gov/

MUMS National Parent-to-Parent Network
150 Custer Court
Green Bay, WI 54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
Tel: (877)336-5333
Email: mums@netnet.net
Internet: http://www.netnet.net/mums/

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
TDD: (888)205-3223
Email: ordr@od.nih.gov
Internet: http://rarediseases.info.nih.gov/Default.aspx

For a Complete Report

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

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