Essential tremor: Clinical features and diagnosis
- Daniel Tarsy, MD
Daniel Tarsy, MD
- Professor of Neurology
- Harvard Medical School
- Ludy Shih, MD
Ludy Shih, MD
- Assistant Professor of Neurology
- Harvard Medical School
Tremor is defined as a rhythmic and oscillatory movement of a body part with a relatively constant frequency and variable amplitude. It is caused by alternating contractions of antagonistic muscles. Tremor is the most common of all movement disorders, and essential tremor (ET) is the most common neurologic cause of postural or action tremor.
This topic will cover the classification, clinical features, diagnostic evaluation, and treatment of ET. The treatment and prognosis of ET are discussed separately. (See "Essential tremor: Treatment and prognosis" and "Surgical treatment of essential tremor".)
Other types of tremor are reviewed elsewhere. (See "Overview of tremor".)
CLASSIFICATION AND TERMINOLOGY
ET is a type of postural and action tremor; these are elicited during examination under two circumstances: with the arms suspended against gravity in a fixed posture; and during the course of goal-directed activity. (See "Overview of tremor", section on 'Postural and action tremors'.)
ET is referred to as familial tremor when there is a family history (see 'Genetics' below). The term "benign essential tremor" was used in the past to distinguish ET from Parkinson disease. However, the use of "benign" as a modifier for ET is best omitted, since the tremor can be severe and disabling. (See "Essential tremor: Treatment and prognosis", section on 'Prognosis'.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Louis ED. Environmental epidemiology of essential tremor. Neuroepidemiology 2008; 31:139.
- Louis ED. 'Essential tremor' or 'the essential tremors': is this one disease or a family of diseases? Neuroepidemiology 2014; 42:81.
- Hopfner F, Haubenberger D, Galpern WR, et al. Knowledge gaps and research recommendations for essential tremor. Parkinsonism Relat Disord 2016; 33:27.
- Clark LN, Louis ED. Challenges in essential tremor genetics. Rev Neurol (Paris) 2015; 171:466.
- Louis ED, Dogu O. Does age of onset in essential tremor have a bimodal distribution? Data from a tertiary referral setting and a population-based study. Neuroepidemiology 2007; 29:208.
- Gulcher JR, Jónsson P, Kong A, et al. Mapping of a familial essential tremor gene, FET1, to chromosome 3q13. Nat Genet 1997; 17:84.
- Higgins JJ, Pho LT, Nee LE. A gene (ETM) for essential tremor maps to chromosome 2p22-p25. Mov Disord 1997; 12:859.
- Shatunov A, Sambuughin N, Jankovic J, et al. Genomewide scans in North American families reveal genetic linkage of essential tremor to a region on chromosome 6p23. Brain 2006; 129:2318.
- Kovach MJ, Ruiz J, Kimonis K, et al. Genetic heterogeneity in autosomal dominant essential tremor. Genet Med 2001; 3:197.
- Bain PG, Findley LJ, Thompson PD, et al. A study of hereditary essential tremor. Brain 1994; 117 ( Pt 4):805.
- Jasinska-Myga B, Wider C. Genetics of essential tremor. Parkinsonism Relat Disord 2012; 18 Suppl 1:S138.
- Merner ND, Girard SL, Catoire H, et al. Exome sequencing identifies FUS mutations as a cause of essential tremor. Am J Hum Genet 2012; 91:313.
- Higgins JJ, Loveless JM, Jankovic J, Patel PI. Evidence that a gene for essential tremor maps to chromosome 2p in four families. Mov Disord 1998; 13:972.
- Louis ED, Ford B, Frucht S, et al. Risk of tremor and impairment from tremor in relatives of patients with essential tremor: a community-based family study. Ann Neurol 2001; 49:761.
- Tanner CM, Goldman SM, Lyons KE, et al. Essential tremor in twins: an assessment of genetic vs environmental determinants of etiology. Neurology 2001; 57:1389.
- Stefansson H, Steinberg S, Petursson H, et al. Variant in the sequence of the LINGO1 gene confers risk of essential tremor. Nat Genet 2009; 41:277.
- Thier S, Lorenz D, Nothnagel M, et al. LINGO1 polymorphisms are associated with essential tremor in Europeans. Mov Disord 2010; 25:717.
- Jiménez-Jiménez FJ, García-Martín E, Lorenzo-Betancor O, et al. LINGO1 and risk for essential tremor: results of a meta-analysis of rs9652490 and rs11856808. J Neurol Sci 2012; 317:52.
- Thier S, Lorenz D, Nothnagel M, et al. Polymorphisms in the glial glutamate transporter SLC1A2 are associated with essential tremor. Neurology 2012; 79:243.
- Müller SH, Girard SL, Hopfner F, et al. Genome-wide association study in essential tremor identifies three new loci. Brain 2016; 139:3163.
- Louis ED. Essential tremor: evolving clinicopathological concepts in an era of intensive post-mortem enquiry. Lancet Neurol 2010; 9:613.
- Louis ED. Understanding essential tremor: progress on the biological front. Curr Neurol Neurosci Rep 2014; 14:450.
- Jellinger KA. Is there cerebellar pathology in essential tremor? Mov Disord 2014; 29:435.
- Louis ED, Faust PL, Vonsattel JP, et al. Neuropathological changes in essential tremor: 33 cases compared with 21 controls. Brain 2007; 130:3297.
- Rajput AH, Robinson CA, Rajput ML, Rajput A. Cerebellar Purkinje cell loss is not pathognomonic of essential tremor. Parkinsonism Relat Disord 2011; 17:16.
- Symanski C, Shill HA, Dugger B, et al. Essential tremor is not associated with cerebellar Purkinje cell loss. Mov Disord 2014; 29:496.
- Rajput AH, Adler CH, Shill HA, Rajput A. Essential tremor is not a neurodegenerative disease. Neurodegener Dis Manag 2012; 2:259.
- Shill HA, Adler CH, Sabbagh MN, et al. Pathologic findings in prospectively ascertained essential tremor subjects. Neurology 2008; 70:1452.
- Cerasa A, Quattrone A. Linking Essential Tremor to the Cerebellum-Neuroimaging Evidence. Cerebellum 2016; 15:263.
- Louis ED, Ottman R, Hauser WA. How common is the most common adult movement disorder? estimates of the prevalence of essential tremor throughout the world. Mov Disord 1998; 13:5.
- Dogu O, Sevim S, Camdeviren H, et al. Prevalence of essential tremor: door-to-door neurologic exams in Mersin Province, Turkey. Neurology 2003; 61:1804.
- Benito-León J, Bermejo-Pareja F, Morales JM, et al. Prevalence of essential tremor in three elderly populations of central Spain. Mov Disord 2003; 18:389.
- Jankovic J. Essential tremor: a heterogenous disorder. Mov Disord 2002; 17:638.
- Louis ED. Essential tremors: a family of neurodegenerative disorders? Arch Neurol 2009; 66:1202.
- Louis ED, Dogu O. Isolated head tremor: part of the clinical spectrum of essential tremor? Data from population-based and clinic-based case samples. Mov Disord 2009; 24:2281.
- Lombardi WJ, Woolston DJ, Roberts JW, Gross RE. Cognitive deficits in patients with essential tremor. Neurology 2001; 57:785.
- Gasparini M, Bonifati V, Fabrizio E, et al. Frontal lobe dysfunction in essential tremor: a preliminary study. J Neurol 2001; 248:399.
- Benito-León J, Louis ED, Bermejo-Pareja F, Neurological Disorders in Central Spain (NEDICES) Study Group. Population-based case-control study of cognitive function in essential tremor. Neurology 2006; 66:69.
- Benito-León J, Louis ED, Sánchez-Ferro Á, Bermejo-Pareja F. Rate of cognitive decline during the premotor phase of essential tremor: a prospective study. Neurology 2013; 81:60.
- Benito-León J, Louis ED, Bermejo-Pareja F, Neurological Disorders in Central Spain Study Group. Elderly-onset essential tremor is associated with dementia. Neurology 2006; 66:1500.
- Thawani SP, Schupf N, Louis ED. Essential tremor is associated with dementia: prospective population-based study in New York. Neurology 2009; 73:621.
- Findley LJ, Koller WC. Essential tremor: a review. Neurology 1987; 37:1194.
- Thenganatt MA, Louis ED. Distinguishing essential tremor from Parkinson's disease: bedside tests and laboratory evaluations. Expert Rev Neurother 2012; 12:687.
- Pahwa R, Koller WC. Is there a relationship between Parkinson's disease and essential tremor? Clin Neuropharmacol 1993; 16:30.
- Stolze H, Petersen G, Raethjen J, et al. The gait disorder of advanced essential tremor. Brain 2001; 124:2278.