Advertisement

Tremor: Phenomenology, Etiology, Diagnosis, and Treatment

  • Arif Dalvi
  • Rajesh Pahwa
  • Kelly E. LyonsEmail author
Chapter

Abstract

Tremor is an involuntary rhythmic, oscillatory movement produced by synchronous or alternating contractions of antagonist muscles. Tremor is the most common movement disorder and one of the most common neurological symptoms. A wide variety of etiologies can cause tremor, with essential tremor being the most common. Parkinson’s disease, multiple sclerosis, and drug-induced tremors are other etiologies commonly seen in clinical practice. The diagnosis of the specific etiology of tremor is based on the clinical characteristics of the tremor as well as associated symptoms. The phenomenology of tremor including whether the tremor is present at rest, with posture or with action, allows classification of tremor. Imaging studies, while not in themselves diagnostic, can help rule out structural etiologies. Dopamine transporter imaging is a recent modality that can help distinguish essential tremor from parkinsonism. The treatment of tremor is based on etiology and, where possible, treatment of the underlying disease state. Deep brain stimulation of the thalamus can be an effective treatment modality in intractable cases of tremor.

Keywords

Tremor disorders Essential tremor Parkinsonian tremor Parkinson’s disease Cerebellar tremor Holmes tremor Psychogenic tremor Pharmacological treatment of tremor Surgical treatment of tremor DBS 

Supplementary material

Video 29.1

Essential tremor. Note postural and intention tremor more prominent in the left hand. Spiral drawing shows marked tremor in the left hand (MP4 89122 kb)

Video 29.2

Cervical dystonia with essential tremor. Note postural tremor more prominent in the left hand and directional component to head tremor most prominent when the patient bends forward (MP4 64153 kb)

Video 29.3

Parkinson’s disease tremor. Note typical rest tremor best seen when the hand is resting on a table. When the hands are outstretched the tremor subsides but a reemergent tremor is seen soon thereafter. There is no significant intention tremor. Slight bradykinesia is noted in the right hand. The rest tremor is also seen when the patient walks (MP4 88938 kb)

Video 29.4

DBS for essential tremor. The patient has a unilateral left VIM DBS implanted. The right hand tremor is well controlled with the left DBS turned on. There is a significant improvement with activities such as eating and drinking with the DBS on, as is seen in the latter part of the video (MP4 78642 kb)

References

  1. 1.
    Elble RJ. Tremor: clinical features, pathophysiology, and treatment. Neurol Clin. 2009;27:679–95, v–vi.CrossRefPubMedGoogle Scholar
  2. 2.
    Louis ED. Essential tremor: evolving clinicopathological concepts in an era of intensive post-mortem enquiry. Lancet Neurol. 2010;9:613–22.CrossRefPubMedGoogle Scholar
  3. 3.
    Anouti A, Koller WC. Tremor disorders. Diagnosis and management. West J Med. 1995;162:510–3.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Deuschl G, Bain P, Brin M. Consensus statement of the movement disorder society on tremor. Ad Hoc Scientific Committee. Mov Disord. 1998;13 Suppl 3:2–23.PubMedGoogle Scholar
  5. 5.
    Bain PG. Parkinsonism & related disorders. Tremor. Parkinsonism Relat Disord. 2007;13 Suppl 3:S369–74.CrossRefPubMedGoogle Scholar
  6. 6.
    Dalvi A, Premkumar A. Tremor: etiology, phenomenology, and clinical features. Dis Mon. 2011;57:109–26.CrossRefPubMedGoogle Scholar
  7. 7.
    Elble RJ. Physiologic and essential tremor. Neurology. 1986;36:225–31.CrossRefPubMedGoogle Scholar
  8. 8.
    Louis ED, Broussolle E, Goetz CG, et al. Historical underpinnings of the term essential tremor in the late 19th century. Neurology. 2008;71:856–9.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Critchley M. Observations on essential (heredofamilial) tremor. Brain. 1949;72:113–39.CrossRefPubMedGoogle Scholar
  10. 10.
    Schmouth JF, Dion PA, Rouleau GA. Genetics of essential tremor: from phenotype to genes, insights from both human and mouse studies. Prog Neurobiol. 2014;119-120C:1–19.CrossRefGoogle Scholar
  11. 11.
    Louis ED. Essential tremors: a family of neurodegenerative disorders? Arch Neurol. 2009;66:1202–8.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Deuschl G, Elble R. Essential tremor – neurodegenerative or nondegenerative disease towards a working definition of ET. Mov Disord. 2009;24:2033–41.CrossRefPubMedGoogle Scholar
  13. 13.
    Louis ED, Marder K, Cote L, et al. Prevalence of a history of shaking in persons 65 years of age and older: diagnostic and functional correlates. Mov Disord. 1996;11:63–9.CrossRefPubMedGoogle Scholar
  14. 14.
    Deuschl G, Raethjen J, Hellriegel H, Elble R. Treatment of patients with essential tremor. Lancet Neurol. 2011;10:148–61.CrossRefPubMedGoogle Scholar
  15. 15.
    Lou JS, Jankovic J. Essential tremor: clinical correlates in 350 patients. Neurology. 1991;41:234–8.CrossRefPubMedGoogle Scholar
  16. 16.
    Bain PG, Findley LJ, Thompson PD, et al. A study of hereditary essential tremor. Brain. 1994;117(Pt 4):805–24.CrossRefPubMedGoogle Scholar
  17. 17.
    Louis ED. Essential tremor. Lancet Neurol. 2005;4:100–10.CrossRefPubMedGoogle Scholar
  18. 18.
    Pal PK, Samii A, Schulzer M, et al. Head tremor in cervical dystonia. Can J Neurol Sci. 2000;27:137–42.PubMedGoogle Scholar
  19. 19.
    Louis ED, Galecki M, Rao AK. Four essential tremor cases with moderately impaired gait: how impaired can gait be in this disease? From pubmed: Tremor Other Hyperkinet Mov (NY). 2013 Nov 4;3.Google Scholar
  20. 20.
    Shahed J, Jankovic J. Exploring the relationship between essential tremor and Parkinson’s disease. Parkinsonism Relat Disord. 2007;13:67–76.CrossRefPubMedGoogle Scholar
  21. 21.
    Minen MT, Louis ED. Emergence of Parkinson’s disease in essential tremor: a study of the clinical correlates in 53 patients. Mov Disord. 2008;23:1602–5.CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Louis ED, Faust PL, Vonsattel JP, et al. Neuropathological changes in essential tremor: 33 cases compared with 21 controls. Brain. 2007;130:3297–307.CrossRefPubMedGoogle Scholar
  23. 23.
    Adler CH, Shill HA, Beach TG. Essential tremor and Parkinson’s disease: lack of a link. Mov Disord. 2011;26:372–7.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Rajput AH, Adler CH, Shill HA, Rajput A. Essential tremor is not a neurodegenerative disease. Neurodegener Dis Manag. 2012;2:259–68.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Bucher SF, Seelos KC, Dodel RC, et al. Activation mapping in essential tremor with functional magnetic resonance imaging. Ann Neurol. 1997;41:32–40.CrossRefPubMedGoogle Scholar
  26. 26.
    Louis ED, Shungu DC, Chan S, et al. Metabolic abnormality in the cerebellum in patients with essential tremor: a proton magnetic resonance spectroscopic imaging study. Neurosci Lett. 2002;333:17–20.CrossRefPubMedGoogle Scholar
  27. 27.
    Benabid AL, Pollak P, Gervason C, et al. Long-term suppression of tremor by chronic stimulation of the ventral intermediate thalamic nucleus. Lancet. 1991;337:403–6.CrossRefPubMedGoogle Scholar
  28. 28.
    Klein JC, Barbe MT, Seifried C, et al. The tremor network targeted by successful VIM deep brain stimulation in humans. Neurology. 2012;78:787–95.CrossRefPubMedGoogle Scholar
  29. 29.
    Deuschl G, Papengut F, Hellriegel H. The phenomenology of parkinsonian tremor. Parkinsonism Relat Disord. 2012;18 Suppl 1:S87–9.CrossRefPubMedGoogle Scholar
  30. 30.
    Jankovic J, Schwartz KS, Ondo W. Re-emergent tremor of Parkinson’s disease. J Neurol Neurosurg Psychiatry. 1999;67:646–50.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Josephs KA, Matsumoto JY, Ahlskog JE. Benign tremulous parkinsonism. Arch Neurol. 2006;63:354–7.CrossRefPubMedGoogle Scholar
  32. 32.
    Hughes AJ, Daniel SE, Blankson S, Lees AJ. A clinicopathologic study of 100 cases of Parkinson’s disease. Arch Neurol. 1993;50:140–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Pahwa R, Lyons KE. Early diagnosis of Parkinson’s disease: recommendations from diagnostic clinical guidelines. Am J Manag Care. 2010;16(Suppl Implications):S94–9.PubMedGoogle Scholar
  34. 34.
    Vingerhoets FJ, Schulzer M, Calne DB, Snow BJ. Which clinical sign of Parkinson’s disease best reflects the nigrostriatal lesion? Ann Neurol. 1997;41:58–64.CrossRefPubMedGoogle Scholar
  35. 35.
    Paulus W, Jellinger K. The neuropathologic basis of different clinical subgroups of Parkinson’s disease. J Neuropathol Exp Neurol. 1991;50:743–55.CrossRefPubMedGoogle Scholar
  36. 36.
    Jellinger KA. Post mortem studies in Parkinson’s disease – is it possible to detect brain areas for specific symptoms? J Neural Transm Suppl. 1999;56:1–29.CrossRefPubMedGoogle Scholar
  37. 37.
    Bergman H, Deuschl G. Pathophysiology of Parkinson’s disease: from clinical neurology to basic neuroscience and back. Mov Disord. 2002;17 Suppl 3:S28–40.CrossRefPubMedGoogle Scholar
  38. 38.
    Hallett M. Tremor: pathophysiology. Parkinsonism Relat Disord. 2014;20 Suppl 1:S118–22.CrossRefPubMedGoogle Scholar
  39. 39.
    Brodkey JA, Tasker RR, Hamani C, et al. Tremor cells in the human thalamus: differences among neurological disorders. J Neurosurg. 2004;101:43–7.CrossRefPubMedGoogle Scholar
  40. 40.
    Elble RJ. What is essential tremor? Curr Neurol Neurosci Rep. 2013;13:353.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Krauss JK, Wakhloo AK, Nobbe F, et al. Lesion of dentatothalamic pathways in severe post-traumatic tremor. Neurol Res. 1995;17:409–16.PubMedGoogle Scholar
  42. 42.
    Manto MU. The wide spectrum of spinocerebellar ataxias (SCAs). Cerebellum. 2005;4:2–6.CrossRefPubMedGoogle Scholar
  43. 43.
    Mehta SH, Morgan JC, Sethi KD. Paraneoplastic movement disorders. Curr Neurol Neurosci Rep. 2009;9:285–91.CrossRefPubMedGoogle Scholar
  44. 44.
    Elble RJ. Central mechanisms of tremor. J Clin Neurophysiol. 1996;13:133–44.CrossRefPubMedGoogle Scholar
  45. 45.
    Dalvi A. Holmes tremor. In: Lyons KE, Pahwa R, editors. Handbook of essential tremor and other tremor disorders. Boca Raton: Taylor and Francis; 2005. p. 243–50.CrossRefGoogle Scholar
  46. 46.
    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.CrossRefPubMedGoogle Scholar
  47. 47.
    Deuschl G. Dystonic tremor. Rev Neurol (Paris). 2003;159:900–5.Google Scholar
  48. 48.
    Deuschl G, Bergman H. Pathophysiology of nonparkinsonian tremors. Mov Disord. 2002;17 Suppl 3:S41–8.CrossRefPubMedGoogle Scholar
  49. 49.
    Bain PG, Findley LJ, Britton TC, et al. Primary writing tremor. Brain. 1995;118(Pt 6):1461–72.CrossRefPubMedGoogle Scholar
  50. 50.
    Lee A, Chadde M, Altenmüller E, Schoonderwaldt E. Characteristics of task-specific tremor in string instrument players. Tremor Other Hyperkinetic Mov (NY). 2014;4:198.Google Scholar
  51. 51.
    Yaltho TC, Ondo WG. Orthostatic tremor: a review of 45 cases. Parkinsonism Relat Disord. 2014;20:723–5.CrossRefPubMedGoogle Scholar
  52. 52.
    Heilman KM. Orthostatic tremor. Arch Neurol. 1984;41:880–1.CrossRefPubMedGoogle Scholar
  53. 53.
    Littmann L. Fact or artifact? The electrocardiographic diagnosis of orthostatic tremor. J Electrocardiol. 2010;43:270–3.CrossRefPubMedGoogle Scholar
  54. 54.
    Boroojerdi B, Ferbert A, Foltys H, et al. Evidence for a non-orthostatic origin of orthostatic tremor. J Neurol Neurosurg Psychiatry. 1999;66:284–8.CrossRefPubMedPubMedCentralGoogle Scholar
  55. 55.
    Glass GA, Ahlskog JE, Matsumoto JY. Orthostatic myoclonus: a contributor to gait decline in selected elderly. Neurology. 2007;68:1826–30.CrossRefPubMedGoogle Scholar
  56. 56.
    Krauss JK, Jankovic J. Head injury and posttraumatic movement disorders. Neurosurgery. 2002;50:927–39; discussion 939–40.PubMedGoogle Scholar
  57. 57.
    Jankovic J. Peripherally induced movement disorders. Neurol Clin. 2009;27:821–32, vii.CrossRefPubMedGoogle Scholar
  58. 58.
    Johnson SL, Hall DM. Post-traumatic tremor in head injured children. Arch Dis Child. 1992;67:227–8.CrossRefPubMedPubMedCentralGoogle Scholar
  59. 59.
    Leehey MA. Fragile X-associated tremor/ataxia syndrome: clinical phenotype, diagnosis, and treatment. J Investig Med. 2009;57:830–6.CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Cardoso FE, Jankovic J. Hereditary motor-sensory neuropathy and movement disorders. Muscle Nerve. 1993;16:904–10.CrossRefPubMedGoogle Scholar
  61. 61.
    Aggarwal A, Bhatt M. Update on Wilson disease. Int Rev Neurobiol. 2013;110:313–48.CrossRefPubMedGoogle Scholar
  62. 62.
    Huster D. Wilson disease. Best Pract Res Clin Gastroenterol. 2010;24:531–9.CrossRefPubMedGoogle Scholar
  63. 63.
    Kim YJ, Pakiam AS, Lang AE. Historical and clinical features of psychogenic tremor: a review of 70 cases. Can J Neurol Sci. 1999;26:190–5.CrossRefPubMedGoogle Scholar
  64. 64.
    Hallett M, Weiner WJ, Kompoliti K. Psychogenic movement disorders. Parkinsonism Relat Disord. 2012;18 Suppl 1:S155–7.CrossRefPubMedGoogle Scholar
  65. 65.
    Ricciardi L, Edwards MJ. Treatment of functional (psychogenic) movement disorders. Neurotherapeutics. 2014;11:201–7.CrossRefPubMedGoogle Scholar
  66. 66.
    Paviour DC, Jäger HR, Wilkinson L, et al. Holmes tremor: application of modern neuroimaging techniques. Mov Disord. 2006;21:2260–2.CrossRefPubMedGoogle Scholar
  67. 67.
    Holtbernd F, Eidelberg D. The utility of neuroimaging in the differential diagnosis of parkinsonian syndromes. Semin Neurol. 2014;34:202–9.CrossRefPubMedPubMedCentralGoogle Scholar
  68. 68.
    Savoiardo M. Differential diagnosis of Parkinson’s disease and atypical parkinsonian disorders by magnetic resonance imaging. Neurol Sci. 2003;24 Suppl 1:S35–7.CrossRefPubMedGoogle Scholar
  69. 69.
    Massano J, Costa F, Nadais G. Teaching neuroImage: MRI in multiple system atrophy: “hot cross bun” sign and hyperintense rim bordering the putamina. Neurology. 2008;71:e38.CrossRefPubMedGoogle Scholar
  70. 70.
    Berry-Kravis E, Abrams L, Coffey SM, et al. Fragile X-associated tremor/ataxia syndrome: clinical features, genetics, and testing guidelines. Mov Disord. 2007;22:2018–30, quiz 2140.CrossRefPubMedGoogle Scholar
  71. 71.
    Cummings JL, Henchcliffe C, Schaier S, et al. The role of dopaminergic imaging in patients with symptoms of dopaminergic system neurodegeneration. Brain. 2011;134:3146–66.CrossRefPubMedGoogle Scholar
  72. 72.
    Zesiewicz TA, Elble RJ, Louis ED, et al. Evidence-based guideline update: treatment of essential tremor: report of the quality standards subcommittee of the American academy of neurology. Neurology. 2011;77:1752–5.CrossRefPubMedPubMedCentralGoogle Scholar
  73. 73.
    Winkler GF, Young RR. Efficacy of chronic propranolol therapy in action tremors of the familial, senile or essential varieties. N Engl J Med. 1974;290:984–8.CrossRefPubMedGoogle Scholar
  74. 74.
    Calzetti S, Findley LJ, Perucca E, Richens A. The response of essential tremor to propranolol: evaluation of clinical variables governing its efficacy on prolonged administration. J Neurol Neurosurg Psychiatry. 1983;46:393–8.CrossRefPubMedPubMedCentralGoogle Scholar
  75. 75.
    Koller WC. Long-acting propranolol in essential tremor. Neurology. 1985;35:108–10.CrossRefPubMedGoogle Scholar
  76. 76.
    Ferreira JJ, Sampaio C. Essential tremor. BMJ Clin Evid. 2007 May 1;2007.Google Scholar
  77. 77.
    Koller WC, Royse VL. Efficacy of primidone in essential tremor. Neurology. 1986;36:121–4.CrossRefPubMedGoogle Scholar
  78. 78.
    Sasso E, Perucca E, Calzetti S. Double-blind comparison of primidone and phenobarbital in essential tremor. Neurology. 1988;38:808–10.CrossRefPubMedGoogle Scholar
  79. 79.
    Lyons KE, Pahwa R, Comella CL, et al. Benefits and risks of pharmacological treatments for essential tremor. Drug Saf. 2003;26:461–81.CrossRefPubMedGoogle Scholar
  80. 80.
    Gironell A, Kulisevsky J, Barbanoj M, et al. A randomized placebo-controlled comparative trial of gabapentin and propranolol in essential tremor. Arch Neurol. 1999;56:475–80.CrossRefPubMedGoogle Scholar
  81. 81.
    Pahwa R, Lyons K, Hubble JP, et al. Double-blind controlled trial of gabapentin in essential tremor. Mov Disord. 1998;13:465–7.CrossRefPubMedGoogle Scholar
  82. 82.
    Connor GS, Edwards K, Tarsy D. Topiramate in essential tremor: findings from double-blind, placebo-controlled, crossover trials. Clin Neuropharmacol. 2008;31:97–103.CrossRefPubMedGoogle Scholar
  83. 83.
    Ondo WG, Jankovic J, Connor GS, et al. Topiramate in essential tremor: a double-blind, placebo-controlled trial. Neurology. 2006;66:672–7.CrossRefPubMedGoogle Scholar
  84. 84.
    Huber SJ, Paulson GW. Efficacy of alprazolam for essential tremor. Neurology. 1988;38:241–3.CrossRefPubMedGoogle Scholar
  85. 85.
    Pahwa R, Busenbark K, Swanson-Hyland EF, et al. Botulinum toxin treatment of essential head tremor. Neurology. 1995;45:822–4.CrossRefPubMedGoogle Scholar
  86. 86.
    Jankovic J, Schwartz K, Clemence W, et al. A randomized, double-blind, placebo-controlled study to evaluate botulinum toxin type A in essential hand tremor. Mov Disord. 1996;11:250–6.CrossRefPubMedGoogle Scholar
  87. 87.
    Diaz NL, Waters CH. Current strategies in the treatment of Parkinson’s disease and a personalized approach to management. Expert Rev Neurother. 2009;9:1781–9.CrossRefPubMedGoogle Scholar
  88. 88.
    Koch M, Mostert J, Heersema D, De Keyser J. Tremor in multiple sclerosis. J Neurol. 2007;254:133–45.CrossRefPubMedPubMedCentralGoogle Scholar
  89. 89.
    Papapetropoulos S, Singer C. Botulinum toxin in movement disorders. Semin Neurol. 2007;27:183–94.CrossRefPubMedGoogle Scholar
  90. 90.
    Ramtahal J, Larner AJ. Shaky legs? Think POT! Age Ageing. 2009;38:352–3.CrossRefPubMedGoogle Scholar
  91. 91.
    Schuurman PR, Bosch DA, Merkus MP, Speelman JD. Long-term follow-up of thalamic stimulation versus thalamotomy for tremor suppression. Mov Disord. 2008;23:1146–53.CrossRefPubMedGoogle Scholar
  92. 92.
    Novak KE, Dalvi A, Nenonene EK, Bernstein LP. Surgical treatment of tremor. Dis Mon. 2011;57:142–59.CrossRefPubMedGoogle Scholar
  93. 93.
    Lyons KE, Pahwa R. Deep brain stimulation and tremor. Neurotherapeutics. 2008;5:331–8.CrossRefPubMedPubMedCentralGoogle Scholar
  94. 94.
    Videnovic A, Metman LV. Deep brain stimulation for Parkinson’s disease: prevalence of adverse events and need for standardized reporting. Mov Disord. 2008;23:343–9.CrossRefPubMedGoogle Scholar
  95. 95.
    Lim DA, Khandhar SM, Heath S, et al. Multiple target deep brain stimulation for multiple sclerosis related and poststroke Holmes’ tremor. Stereotact Funct Neurosurg. 2007;85:144–9.CrossRefPubMedGoogle Scholar
  96. 96.
    Kobayashi K, Katayama Y, Oshima H, et al. Multitarget, dual-electrode deep brain stimulation of the thalamus and subthalamic area for treatment of Holmes’ tremor. J Neurosurg. 2014;120:1025–32.CrossRefPubMedGoogle Scholar
  97. 97.
    Volkmann J, Mueller J, Deuschl G, et al. Pallidal neurostimulation in patients with medication-refractory cervical dystonia: a randomised, sham-controlled trial. Lancet Neurol. 2014;13:875–84.CrossRefPubMedGoogle Scholar
  98. 98.
    Larson PS. Deep brain stimulation for movement disorders. Neurotherapeutics. 2014;11:465–74.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag Wien 2017

Authors and Affiliations

  1. 1.Movement Disorders ProgramPalm Beach Neuroscience InstituteWest Palm BeachUSA
  2. 2.Department of Neurology, Parkinson’s Disease and Movement Disorder CenterUniversity of Kansas Medical CenterKansas CityUSA

Personalised recommendations