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Paul Cullis, MD
Chief, Department of Neurology St. John Health System
Tremor Defined
An involuntary, rhythmic, oscillatory, sinusoidal movement of a part or parts of the body Produced by contractions of alternating or synchronous antagonist muscles
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Classification of Tremor
Rest tremor Action tremor
Postural tremor
Reemergent tremor
Kinetic tremor
Initial tremor Dynamic tremor Terminal tremor
Internal tremor
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Deuschl G, Bain P, Brin M, and an Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on tremor. Mov Disord 1998;13 (suppl 3):2-23.
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Deuschl G, Bain P, Brin M, and an Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on tremor. Mov Disord 1998;13 (suppl 3):2-23.
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Deuschl G, Bain P, Brin M, and an Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on tremor. Mov Disord 1998;13 (suppl 3):2-23.
www.wemove.org
Deuschl G, Bain P, Brin M, and an Ad Hoc Scientific Committee. Consensus statement of the Movement Disorder Society on tremor. Mov Disord 1998;13 (suppl 3):2-23.
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Red Flags
1.Unilateral tremor, focal tremor, leg tremor, gait disturbance, rigidity, bradykinesia, rest tremor 2.Sudden or rapid onset 3.Current drug treatment that may cause or exacerbate tremor 4.Isolated head tremor with abnormal posture (head tilt or turning)
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Epidemiology: Case Ascertainment, (contd) Tremor frequently misdiagnosed: If mild ==> anxiety or neurosis If severe ==> Parkinsons disease Larson: 1 in 210 individuals contacted the hospital for treatment Rautakorpi: 1 in 10 individuals sought treatment
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Challenge to determine true frequency of ET in general population due to... Variation in the clinical criteria Lack of consensus on the definition of ET Difficulty in differentiating between mild forms of ET and exaggerated physiologic tremor (EPT) Difficulties in classifying tremor when it is associated with other conditions (e.g., dystonia and parkinsonism)
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Rajput: Incidence of ET
90 80 70 60 50 40 30 20 10 0 0-19 20-39 40-59 50-59 60-69 70-79 Age at Inciden
Brin M, Koller W. Epidemiology and genetics of essential tremor. Mov Disord 1998;13 (Suppl 3):55-63.
Number pe 100,000
80+
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Salemi: Prevalence of ET
6000 5000 4000 Prevalence per 3000 100,000 2000 1000 0 0-19 20-39 40-59 60-69 70-79 Age at Incidenc 80-89 90+
Salemi G, Savettieri G, Rocca WA, et al. Prevalence of essential tremor: a door-to-door survey in Terrasini, Sicily. Neurology. 1994;44:61-64.
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Genetics of ET
Familial nature noted by many including:
Most (1836) Dana (1887) Critchley (1949) Louis (1998) Findley (2000)
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ET as a Central Tremor
Arises from a central oscillatory activity within a central network or cell group that enters an oscillatory mode
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Hallet M. Overview of human tremor physiology. Mov Disord 1998;13 (suppl 3):43-48.
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ET: Pathophysiology
C15-labeled O2 PET demonstrated marked increased blood flow to both cerebellar hemispheres and midbrain activation in region of red nucleus No established structural lesion in ET fMRI confirmed increased activation of the cerebellum and red nucleus Abnormalities in tandem gait suggestive of cerebellar dysfunction
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Peripheral Contributors
Beta-adrenergic blockers attenuate ET and postural tremor, possibly via beta2 skeletal muscle adrenoreceptors (betablockers may also affect central pathways) Peripheral injuries may induce or precipitate tremor IV and intra-arterial epinephrine enhance PT via peripheral forearm beta-adrenoreceptors; blocked by propranolol
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Diagnosis of ET
Clinical history/family history Physical examination (including QOL/disability, etc.) Absence of abnormal laboratory findings (normal TFT, serum ceruloplasm, etc.) Current medications Effect of ETOH Archimedes spiral; handwriting test; water test; peg board; CT scan (when warranted)
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Archimedes Spiral
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Accelerometry for ET
Autospectra obtained by accelerometry from a patient with ET (frequency plotted against root-mean-square [rms] acceleration [g x 10-3])
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Clinical Characteristics of ET
Lou JS, Jankovic J. Essential tremor: clinical correlates in 350 patients. Neurology. 1991;41(Pt 1):234-238.
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Differential Diagnosis
Parkinsons disease (PD) Pallidonigral degeneration Multiple system atrophy (MSA) Olivopontocerebellar atrophy (OPCA) Striatonigral degeneration Progressive pallidal atrophy Huntingtons disease (HD) Benign hereditary chorea
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Peripheral neuropathies
Same as controls:
Duvoisin et al. (1969) Martilla and Rinne (1976) Roy et al. (1983) Cleeves et al. (1988) Martilla and Rinne (1988)
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PD and ET
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PD and ET
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