Classic essential tremor is a clinical syndrome of action tremor in the upper limbs (at least 95 % of patients) and less commonly the head, face/jaw, voice, tongue, trunk, and lower limbs, in the absence of other neurologic signs. However, the longstanding notion that essential tremor is a monosymptomatic tremor disorder is being challenged by a growing literature describing associated disturbances of tandem walking, personality, mood, hearing, and cognition. There is also epidemiologic, pathologic, and genetic evidence that essential tremor is pathophysiologically heterogeneous. Misdiagnosis of essential tremor is common because clinicians frequently overlook other neurologic signs and because action tremor in the hands is caused by many conditions, including dystonia, Parkinson disease, and drug-induced tremor. Thus, essential tremor is nothing more than a syndrome of idiopathic tremulousness, and the challenge for researchers and clinicians is to find specific etiologies of this syndrome.
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Louis ED, Broussolle E, Goetz CG, Krack P, Kaufmann P, Mazzoni P. Historical underpinnings of the term essential tremor in the late 19th century. Neurology. 2008;71:856–9.
• Critchley M. Observations on essential (heredofamial) tremor. Brain. 1949;72:113–39. This is a classic historical review of essential tremor.
Benito-León J, Louis ED, Bermejo-Pareja F. Elderly-onset essential tremor is associated with dementia. Neurology. 2006;66:1500–5.
Benito-León J, Louis ED, Bermejo-Pareja F. Risk of incident Parkinson's disease and parkinsonism in essential tremor: a population based study. J Neurol Neurosurg Psychiatry. 2009;80:423–5.
Louis ED, Benito-Leon J, Ottman R, Bermejo-Pareja F. A population-based study of mortality in essential tremor. Neurology. 2007;69:1982–9.
Louis ED, Faust PL, Vonsattel JP, et al. Older onset essential tremor: more rapid progression and more degenerative pathology. Mov Disord. 2009;24:1606–12.
• Deuschl G, Elble R. Essential tremor - neurodegenerative or nondegenerative disease towards a working definition of ET. Mov Disord. 2009;24:2033–41. This is a recent review of the pathophysiogy of essential tremor.
Marsden CD, Obeso JA, Rothwell JC. Benign essential tremor is not a single entity. In: Yahr MD, editor. Current concepts of parkinson's disease and related disorders. Amsterdam: Excerpta Medica; 1983. p. 31–46.
• Elble RJ, Deuschl G. Tremor. In: Brown WF, Bolton CF, Aminoff M, editors. Neuromuscular function and disease: basic, clinical, and electrodiagnostic aspects. Philadelphia: W. B. Saunders Co; 2002. p. 1759–79. This is a review of the clinical neurophysiology of tremor.
Elble RJ. Physiologic and essential tremor. Neurology. 1986;36:225–31.
Benito-León J, Bermejo-Pareja F, Louis ED. Incidence of essential tremor in 3 elderly populations of central Spain. Neurology. 2005;64:1721–5.
Dogu O, Louis ED, Sevim S, Kaleagasi H, Aral M. Clinical characteristics of essential tremor in Mersin, Turkey–a population-based door-to-door study. J Neurol. 2005;252:570–4.
Lorenz D, Poremba C, Papengut F, Schreiber S, Deuschl G. The psychosocial burden of essential tremor in an outpatient- and a community-based cohort. Eur J Neurol. 2011;18:972–9.
•• Quinn NP, Schneider SA, Schwingenschuh P, Bhatia KP. Tremor-some controversial aspects. Mov Disord. 2011;26:18–23. The authors summarize many of the uncertainties and controversies regarding the etiology and pathophysiology of essential tremor.
Lee MS, Kim YD, Im JH, Kim HJ, Rinne JO, Bhatia KP. 123I-IPT brain SPECT study in essential tremor and Parkinson's disease. Neurology. 1999;52:1422–6.
Pellecchia MT, Varrone A, Annesi G, et al. Parkinsonism and essential tremor in a family with pseudo-dominant inheritance of PARK2. An FP-CIT SPECT study. Mov Disord. 2007;22:559–63.
Rivest J, Marsden CD. Trunk and head tremor as isolated manifestations of dystonia. Mov Disord. 1990;5:60–5.
Schiebler S, Schmidt A, Zittel S, et al. Arm tremor in cervical dystonia-is it a manifestation of dystonia or essential tremor? Mov Disord. 2011;26:1789–92.
Yanagisawa N, Goto A, Narabayashi H. Familial dystonia musculorum deformans and tremor. J Neurol Sci. 1972;16:125–36.
Elble RJ. Defining dystonic tremor. Curr Neuropharmacol. 2013;11:in press.
• Bain PG, Findley LJ, Thompson PD, et al. A study of hereditary essential tremor. Brain. 1994;117:805–24. This paper provides a thorough description of essential tremor in 21 families.
Louis ED, Hernandez N, Alcalay RN, Tirri DJ, Ottman R, Clark LN. Prevalence and features of unreported dystonia in a family study of "pure" essential tremor. Parkinsonism Relat Disord. 2012;[Epub ahead of print].
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;129:2318–31.
Chouinard S, Louis ED, Fahn S. Agreement among movement disorder specialists on the clinical diagnosis of essential tremor. Mov Disord. 1997;12:973–6.
Poston KL, Rios E, Louis ED. Action tremor of the legs in essential tremor: prevalence, clinical correlates, and comparison with age-matched controls. Parkinsonism Relat Disord. 2009;15:602–5.
Hsu YD, Chang MK, Sung SC, Hsein HH, Deng JC. Essential tremor: clinical, electromyographical, and pharmacological studies in 146 Chinese patients. Chung Hua I Hsueh Tsa Chih. 1990;45:93–9.
Whaley NR, Putzke JD, Baba Y, Wszolek ZK, Uitti RJ. Essential tremor: phenotypic expression in a clinical cohort. Parkinsonism Relat Disord. 2007;13:333–9.
Louis ED, Ford B, Wendt KJ, Cameron G. Clinical characteristics of essential tremor: data from a community- based study. Mov Disord. 1998;13:803–8.
Elble RJ. Characteristics of physiologic tremor in young and elderly adults. Clin Neurophysiol. 2003;114:624–35.
Elble RJ, Higgins C, Elble S. Electrophysiologic transition from physiologic tremor to essential tremor. Mov Disord. 2005;20:1038–42.
Louis ED, Ford B, Bismuth B. Reliability between two observers using a protocol for diagnosing essential tremor. Mov Disord. 1998;13:287–93.
Lorenz D, Papengut F, Frederiksen H, et al. Evaluation of a screening instrument for essential tremor. Mov Disord. 2008;23:1006–12.
Louis ED, Pullman SL. Comparison of clinical vs electrophysiological methods of diagnosing of essential tremor. Mov Disord. 2001;16:668–73.
•• Deuschl G, Bain P, Brin M. Consensus statement of the Movement Disorder Society on Tremor. Ad Hoc Scientific Committee. Mov Disord. 1998;13:2–23. This paper summarizes the TRIG and MDS criteria for essential tremor.
Gerasimou G, Costa DC, Papanastasiou E, et al. SPECT study with I-123-Ioflupane (DaTSCAN) in patients with essential tremor. Is there any correlation with Parkinson's disease? Ann Nucl Med. 2012;26:337–44.
Schwingenschuh P, Ruge D, Edwards MJ, et al. Distinguishing SWEDDs patients with asymmetric resting tremor from Parkinson's disease: a clinical and electrophysiological study. Mov Disord. 2010;25:560–9.
Kagi G, Bhatia KP, Tolosa E. The role of DAT-SPECT in movement disorders. J Neurol Neurosurg Psychiatry. 2010;81:5–12.
Jain S, Lo SE, Louis ED. Common misdiagnosis of a common neurological disorder: how are we misdiagnosing essential tremor? Arch Neurol. 2006;63:1100–4.
Schrag A, Münchau A, Bhatia KP, Quinn NP, Marsden CD. Essential tremor: an overdiagnosed condition? J Neurol. 2000;247:955–9.
Mostile G, Jankovic J. Alcohol in essential tremor and other movement disorders. Mov Disord. 2010;25:2274–84.
Rajput AH, Jamieson H, Hirsh S, Quraishi A. Relative efficacy of alcohol and propranolol in action tremor. Can J Neurol Sci. 1975;2:31–5.
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;24:2281–5.
Louis ED, Ferreira JJ. How common is the most common adult movement disorder? update on the worldwide prevalence of essential tremor. Mov Disord. 2010;25:534–41.
Wolraich D, Vasile Marchis-Crisan C, Redding N, Khella SL, Mirza N. Laryngeal tremor: co-occurrence with other movement disorders. ORL J Otorhinolaryngol Relat Spec. 2010;72:291–4.
Silverdale MA, Schneider SA, Bhatia KP, Lang AE. The spectrum of orolingual tremor—a proposed classification system. Mov Disord. 2008;23:159–67.
Bain P, Brin M, Deuschl G, et al. Criteria for the diagnosis of essential tremor. Neurology. 2000;54:S7.
Louis ED. Essential tremor as a neuropsychiatric disorder. J Neurol Sci. 2010;289:144–8.
Fasano A, Herzog J, Raethjen J, et al. Gait ataxia in essential tremor is differentially modulated by thalamic stimulation. Brain. 2010;133:3635–48.
Fasano A, Herzog J, Raethjen J, et al. Lower limb joints kinematics in essential tremor and the effect of thalamic stimulation. Gait Posture. 2012;36:187–93.
Pifl C, Kish SJ, Hornykiewicz O. Thalamic noradrenaline in Parkinson's disease: deficits suggest role in motor and non-motor symptoms. Mov Disord. 2012;27:1618–24.
• Raethjen J, Deuschl G. The oscillating central network of essential tremor. Clin Neurophysiol. 2012;123:61–4. This is a recent study of the so-called tremor network.
Schnitzler A, Munks C, Butz M, Timmermann L, Gross J. Synchronized brain network associated with essential tremor as revealed by magnetoencephalography. Mov Disord. 2009;24:1629–35.
Dupuis MJ, Evrard FL, Jacquerye PG, Picard GR, Lermen OG. Disappearance of essential tremor after stroke. Mov Disord. 2010;25:2884–7.
Hellwig B, Häussler S, Schelter B, et al. Tremor-correlated cortical activity in essential tremor. Lancet. 2001;357:519–23.
Hua SE, Lenz FA. Posture-related oscillations in human cerebellar thalamus in essential tremor are enabled by voluntary motor circuits. J Neurophysiol. 2005;93:117–27.
Boecker H, Brooks DJ. Functional imaging of tremor. Mov Disord. 1998;13:64–72.
Timmermann L, Gross J, Dirks M, Volkmann J, Freund HJ, Schnitzler A. The cerebral oscillatory network of parkinsonian resting tremor. Brain. 2003;126:199–212.
Sudmeyer M, Pollok B, Hefter H, et al. Synchronized brain network underlying postural tremor in Wilson's disease. Mov Disord. 2006;21:1935–40.
Timmermann L, Gross J, Kircheis G, Haussinger D, Schnitzler A. Cortical origin of mini-asterixis in hepatic encephalopathy. Neurology. 2002;58:295–8.
Pollok B, Gross J, Dirks M, Timmermann L, Schnitzler A. The cerebral oscillatory network of voluntary tremor. J Physiol. 2004;554:871–8.
Timmermann L, Gross J, Butz M, Kircheis G, Haussinger D, Schnitzler A. Pathological oscillatory coupling within the human motor system in different tremor syndromes as revealed by magnetoencephalography. Neurol Clin Neurophysiol. 2004;2004:26.
Lorenz D, Frederiksen H, Moises H, Kopper F, Deuschl G, Christensen K. High concordance for essential tremor in monozygotic twins of old age. Neurology. 2004;62:208–11.
Gulcher JR, Jonsson P, Kong A, et al. Mapping of a familial essential tremor gene, FET1, to chromosome 3q13. Nat Genet. 1997;17:84–7.
Higgins JJ, Pho LT, Nee LE. A gene (ETM) for essential tremor maps to chromosome 2p22-p25. Mov Disord. 1997;12:859–64.
Deng H, Le W, Jankovic J. Genetics of essential tremor. Brain. 2007;130:1456–64.
Kovach MJ, Ruiz J, Kimonis K, et al. Genetic heterogeneity in autosomal dominant essential tremor. Genet Med. 2001;3:197–9.
Ma S, Davis TL, Blair MA, et al. Familial essential tremor with apparent autosomal dominant inheritance: should we also consider other inheritance modes? Mov Disord. 2006;21:1368–74.
Pasini E, Busolin G, Nobile C, Michelucci R. Autosomal dominant essential tremor: a novel family with anticipation. Neurol Sci. 2012;[Epub ahead of print].
Yoo YM, Lee CJ, Lee U, Kim YJ. Mitochondrial DNA in patients with essential tremor. Neurosci Lett. 2008;434:29–34.
Zimprich A. Phenocopies in families with essential tremor and restless legs syndrome challenge Mendelian laws. Epigenetics might provide answers. Parkinsonism Relat Disord. 2012;18:711–6.
•• Jiménez-Jiménez FJ, Garrrcia-Martin E, Lorenzo-Betancor O, Pastor P, Alonso-Navarro H, Agundez JA. LINGO1 and risk for essential tremor: results of a meta-analysis of rs9652490 and rs11856808. J Neurol Sci. 2012;317:52–7. This is a recent meta-analysis and review of the data supporting LINGO1 variants as risk factors for essential tremor.
Stefansson H, Steinberg S, Petursson H, et al. Variant in the sequence of the LINGO1 gene confers risk of essential tremor. Nat Genet. 2009;41:277–9.
Wu YW, Rong TY, Li HH, et al. Analysis of Lingo1 variant in sporadic and familial essential tremor among Asians. Acta Neurol Scand. 2011;124:264–8.
Deng H, Gu S, Jankovic J. LINGO1 variants in essential tremor and Parkinson's disease. Acta Neurol Scand. 2012;125:1–7.
Yu M, Ma K, Faust PL, et al. Increased number of Purkinje cell dendritic swellings in essential tremor. Eur J Neurol. 2012;19:625–30.
Thier S, Lorenz D, Nothnagel M, et al. Polymorphisms in the glial glutamate transporter SLC1A2 are associated with essential tremor. Neurology. 2012;79:243–8.
Garcia-Martin E, Martinez C, Alonso-Navarro H, et al. Gamma-aminobutyric acid GABRA4, GABRE, and GABRQ receptor polymorphisms and risk for essential tremor. Pharmacogenet Genomics. 2011;21:436–9.
Garcia-Martin E, Martinez C, Alonso-Navarro H, et al. Gamma-aminobutyric acid (GABA) receptor rho (GABRR) polymorphisms and risk for essential tremor. J Neurol. 2011;258:203–11.
Thier S, Kuhlenbaumer G, Lorenz D, et al. GABA(A) receptor- and GABA transporter polymorphisms and risk for essential tremor. Eur J Neurol. 2011;18:1098–100.
•• Louis ED. Essential tremors: a family of neurodegenerative disorders? Arch Neurol. 2009;66:1202–8. This review summarizes the epidemiologic and pathologic evidence for essential tremor being a heterogeneous group of disorders.
•• Louis ED, Faust PL, Vonsattel JP. Purkinje cell loss is a characteristic of essential tremor. Parkinsonism Relat Disord. 2011;17:406–9. This paper and reference 80 summarize the argument that essential tremor is a neurodegenerative disorder.
Louis ED, Faust PL, Ma KJ, Yu M, Cortes E, Vonsattel JP. Torpedoes in the cerebellar vermis in essential tremor cases vs controls. Cerebellum. 2011;10:812–9.
Louis ED, Ma K, Babij R, et al. Neurofilament protein levels: quantitative analysis in essential tremor cerebellar cortex. Neurosci Lett. 2012;518:49–54.
Louis ED, Mazzoni P, Ma KJ, et al. Essential tremor with ubiquitinated intranuclear inclusions and cerebellar degeneration. Clin Neuropathol. 2012;31:119–26.
•• Rajput AH, Adler CH, Shill HA, Rajput A. Essential tremor is not a neurodegenerative disease. Neurodegener Dis Manag. 2012;2:259–68. This paper summarizes the evidence against the hypothesis that essential tremor is a neurodegenerative disorder.
Rajput AH, Robinson CA, Rajput ML, Robinson SL, Rajput A. Essential tremor is not dependent upon cerebellar Purkinje cell loss. Parkinsonism Relat Disord. 2012;18:626–8.
Louis ED, Faust PL, Vonsattel JP, et al. Torpedoes in Parkinson's disease, Alzheimer's disease, essential tremor, and control brains. Mov Disord. 2009;24:1600–5.
Ma K, Babij R, Cortes E, Vonsattel JP, Louis ED. Cerebellar pathology of a dual clinical diagnosis: patients with essential tremor and dystonia. Tremor and other Hyperkinetic Movements. 2012;2.
Benito-León J, Louis ED, Bermejo-Pareja F. Population-based case–control study of cognitive function in essential tremor. Neurology. 2006;66:69–74.
Bermejo-Pareja F, Louis ED, Benito-Leon J. Risk of incident dementia in essential tremor: a population-based study. Mov Disord. 2007;22:1573–80.
Thawani SP, Schupf N, Louis ED. Essential tremor is associated with dementia: prospective population-based study in New York. Neurology. 2009;73:621–5.
Benito-León J, Bermejo-Pareja F, Morales JM, Vega S, Molina JA. Prevalence of essential tremor in 3 elderly populations of central Spain. Mov Disord. 2003;18:389–94.
Louis ED. Factor analysis of motor and nonmotor signs in essential tremor: are these signs all part of the same underlying pathogenic process? Neuroepidemiology. 2009;33:41–6.
Adler CH, Shill HA, Beach TG. Essential tremor and Parkinson's disease: lack of a link. Mov Disord. 2011;26:372–7.
Elble RJ, Dubinsky RM, Ala T. Alzheimer's disease and essential tremor finally meet. Mov Disord. 2007;22:1525–7.
Shill HA, Adler CH, Beach TG, et al. Brain biochemistry in autopsied patients with essential tremor. Mov Disord. 2012;27:113–7.
Deuschl G. Locus coeruleus dysfunction: a feature of essential or senile tremor? Mov Disord. 2012;27:1–2.
Benito-Leon J. Essential tremor: from a monosymptomatic disorder to a more complex entity. Neuroepidemiology. 2008;31:191–2.
Johnson WG, Hodge SE, Duvoisin R. Twin studies and the genetics of Parkinson's disease—a reappraisal. Mov Disord. 1990;5:187–94.
Fugger L, McVean G, Bell JI. Genomewide association studies and common disease–realizing clinical utility. N Engl J Med. 2012;367:2370–1.
Supported by the Spastic Paralysis Research Foundation of Kiwanis International Illinois-Eastern Iowa District.
Conflict of Interest
Rodger J. Elble has received honoraria from Kinetics Foundation and Movement Disorder Society.
This article is part of the Topical Collection on Movement Disorders
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Elble, R.J. What is Essential Tremor?. Curr Neurol Neurosci Rep 13, 353 (2013). https://doi.org/10.1007/s11910-013-0353-4
- Essential tremor
- Physiologic tremor
- Parkinson disease