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Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor- Parkinson’s Disease in a Movement Disorders Clinic Cover

Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor- Parkinson’s Disease in a Movement Disorders Clinic

Open Access
|Mar 2021

Figures & Tables

Table 1

Demographic and historical features.

OVERALLETET PLUSET-PDP (ALL)ADJ. P (ET VS ET PLUS)ADJ. P (ET VS ET-PD)ADJ. P (ET PLUS VS ET-PD)
Tremor Classification
      Number of patients (%)300 (100%)62 (20.7%)160 (53.3%)78 (26.0%)
Gender
      Male157 (52.3%)36 (58.1%)70 (43.8%)51 (65.4%)0.00440.0550.380.0017
      Female143 (47.7%)26 (41.9%)90 (56.3%)27 (34.5%)
Age, years
      Mean age (SD)66.1 (13.6)60.5 (15.7)68.1 (13.6)66.3 (10.2)0.000950.000560.16790.0215
      Median age67.065.57065
Age at Tremor Onset, years
      Mean age at onset (SD)38.3 (21.0)34.3 (19.0)40.5 (22.0)36.9 (19.9)0.130.0530.380.29
      Median age at onset413345
Tremor duration, years
      Mean duration (SD)28.1 (18.8)26.0 (18.2)28.1 (19.1)29.6 (18.9)0.590.470.290.67
      Median duration23222225
Family History
      Tremor215 (71.7%)40 (64.5%)123 (76.9%)52 (66.7%)0.0970.0610.790.94
      PD58 (19.3%)16 (25.8%)23 (14.4%)19 (24.4%)0.0660.0450.840.058
      Dystonia8 (2.7%)2 (3.2%)6 (3.8%)0 (0.0%)0.267510.19430.1814
Clinical Features by History
      Balance disturbance82 (27.3%)10 (16.1%)44 (27.5%)28 (35.9%)0.0330.0760.0090.19
      Depression89 (29.7%)16 (25.8%)49 (30.6%)24 (30.8%)0.760.480.520.98
      Anxiety61 (20.3%)5 (8.1%)32 (20.0%)24 (30.8%)0.0040.030.0010.066
      RBD symptoms17 (5.7%)1 (1.6%)4 (2.5%)12 (15.4%)0.000310.0050.0002

[i] Adj. p = adjusted p (statistically significant cutoff of p < 0.017 by Bonferroni correction). SD = standard deviation, PD = Parkinson’s disease, RBD = rapid-eye movement behavior disorder, ET = essential tremor, ET plus = essential tremor plus, ET-PD = essential tremor-Parkinson’s disease.

tohm-11-1-581-g1.png
Figure 1

Age at Onset of Tremor Histogram. A bimodal distribution is visible in the 2nd and 6th decades of life.

Table 2

Examination features.

OVERALLETET PLUSET-PDP (ALL)ADJ. P (ET VS ET PLUS)ADJ. P (ET VS ET-PD)ADJ. P (ET PLUS VS ET-PD)
Examination features
      Rest tremor130 (43.3%)n/a64 (40.0%)66 (84.6%)n/an/an/a<0.0001
      Postural tremor294 (98.0%)62 (100.0%)157 (98.1%)75 (96.2%)0.350.560.250.40
      Kinetic tremor290 (96.7%)62 (100.0%)156 (97.5%)72 (92.3%)0.0330.5780.0340.084
      Isometric tremor90 (30.0%)25 (40.3%)46 (28.8%)19 (24.4%)0.110.0970.0430.48
Body areas involved
      Head141 (47.0%)20 (32.3%)95 (59.4%)26 (33.3%)<0.00010.000290.890.00016
      Voice74 (24.7%)15 (24.2%)51 (31.9%)8 (10.3%)0.00140.260.0270.00029
      Tongue41 (13.7%)7 (11.3%)27 (16.9%)7 (9.0%)0.210.300.650.10
      Upper limbs300 (100.0%)62 (100.0%)160 (100.0%)78 (100.0%)n/an/an/an/a
      Lower limbs70 (23.3%)13 (21.0%)43 (26.9%)22 (28.2%)0.910.970.710.68
      Trunk51 (17.0%)11 (17.7%)28 (17.5%)12 (15.4%)0.910.970.710.68
Associated features
      Parkinsonism117 (39.0%)n/a39 (24.4%)78 (100.0%)n/an/an/a<0.0001
      Dystonia75 (25.0%)n/a68 (42.5%)7 (9.0%)n/an/an/a<0.0001
      Tandem gait abnormality69 (23.0%)n/a62 (38.8%)7 (9.0%)n/an/an/a<0.0001
      Ataxia8 (2.7%)n/a6 (3.8%)2 (2.6%)n/an/an/a0.63
Cognitive impairment45 (15.0%)n/a26 (16.3%)19 (24.4%)n/an/an/a0.134
      Neuropathy35 (11.7%)3 (4.8%)24 (15.0%)8 (10.3%)0.100.0380.350.31
TETRAS evaluations
      # patients88 (29.3%)
      Mean performance subscore (SD)23.4 (6.75)19.7 (5.2)24.7 (6.5)21.8 (7.9)0.0150.00470.390.15
Mean ADL subscore (SD)21.2 (10.24)17.9 (8.4)24.0 (9.9)12.9 (8.4)0.000360.0220.0880.00037
Average total score (SD)44.8 (15.47)37.8 (12.6)49.4 (15.0)33.6 (12.0)0.000220.000620.360.0005

[i] Adj. p = adjusted p (statistically significant cutoff of p < 0.017 by Bonferroni correction). SD = standard deviation, ET = essential tremor, ET plus = essential tremor plus, ET-PD = essential tremor-Parkinson’s disease. TETRAS = The Essential Tremor Rating Assessment Scale, ADL = activities of daily living. Cognitive Impairment included MCI or dementia by neuropsychological testing, or MoCA score of ≤ 25. Some TETRAS scores had only ADL or performance subscores available, and were not included in the total score average.

Table 3

Treatment history.

OVERALLETET PLUSET-PDP (ALL)ADJ. P (ET VS ET PLUS)ADJ. P (ET VS ET-PD)ADJ. P (ET PLUS VS ET-PD)
Medication History
      Average # of current oral medications (SD)1.92 (1.2)1.4 (1.0)1.8 (1.1)2.5 (1.3)<0.00010.013<0.00010.00046
      Median # of current oral medications2122
      Average # of prior oral medications (SD)3.81 (1.9)3.1 (1.5)3.6 (1.8)4.7 (2.0)<0.00010.058<0.0001<0.0001
Median # of prior oral medications4345
      At least partially responsive213 (71.0%)43 (69.4%)105 (65.5%)65 (83.3%)0.0180.600.0500.005
      Improved tremor with alcohol79 (26.3%)16 (25.8%)44 (27.5%)19 (24.4%)0.870.800.840.61
BoNT Injections
      Received BoNT injections123 (41.0%)26 (41.9%)81 (50.6%)16 (20.5%)<0.00010.250.006<0.0001
      BoNT effective91/123 (74.0%)21 (80.8%)59 (72.8%)11 (68.8%)0.640.420.460.76
DBS
      Underwent DBS placement38 (12.7%)11 (17.7%)17 (10.6%)10 (12.8%)0.360.150.420.62
      DBS effective35/38 (92.1%)11 (100.0%)15 (88.2%)9 (90.0%)0.6010.510.481

[i] Adj. p = adjusted p (statistically significant cutoff of p < 0.017 by Bonferroni correction). ET = essential tremor, ET plus = essential tremor plus, ET-PD = essential tremor-Parkinson’s disease, SD = standard deviation, BoNT = botulinum toxin, DBS = deep brain stimulation.

Table 4

Essential tremor plus associated features.

FEATURENUMBER OF PATIENTS (%), TOTAL N = 160
Rest tremor64 (40.0%)
Other parkinsonian features39 (24.4%)
Dystonia68 (42.5%)
Ataxia6 (3.8%)
Abnormal tandem gait62 (38.8%)
Cognitive impairment26 (16.3%)
DOI: https://doi.org/10.5334/tohm.581 | Journal eISSN: 2160-8288
Language: English
Submitted on: Oct 28, 2020
Accepted on: Dec 23, 2020
Published on: Mar 29, 2021
Published by: Ubiquity Press
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2021 Steven T. Bellows, Joseph Jankovic, published by Ubiquity Press
This work is licensed under the Creative Commons Attribution 4.0 License.