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Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor

BACKGROUND: Action tremor is the hallmark feature of essential tremor (ET). While the tremor typically is mildly asymmetric, in some patients, it is markedly asymmetric. There are few data on factors that influence this asymmetry. ET is also a highly familial disease. Whether the tremor asymmetry pr...

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Autores principales: Louis, Elan D., Hernandez, Nora, Chen, Karen P., Naranjo, Kelly V., Park, Jemin, Clark, Lorraine N., Ottman, Ruth
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395646/
https://www.ncbi.nlm.nih.gov/pubmed/28469594
http://dx.doi.org/10.3389/fneur.2017.00148
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author Louis, Elan D.
Hernandez, Nora
Chen, Karen P.
Naranjo, Kelly V.
Park, Jemin
Clark, Lorraine N.
Ottman, Ruth
author_facet Louis, Elan D.
Hernandez, Nora
Chen, Karen P.
Naranjo, Kelly V.
Park, Jemin
Clark, Lorraine N.
Ottman, Ruth
author_sort Louis, Elan D.
collection PubMed
description BACKGROUND: Action tremor is the hallmark feature of essential tremor (ET). While the tremor typically is mildly asymmetric, in some patients, it is markedly asymmetric. There are few data on factors that influence this asymmetry. ET is also a highly familial disease. Whether the tremor asymmetry profile (i.e., differential expression of tremor in each arm in a given patient) is similar across family members is not known. The alternative possibility is that this feature is not heritable. There are no published data addressing this issue. The aim of this study was to determine whether the extent of action tremor asymmetry ran in ET families. METHODS: ET probands and relatives were enrolled in a genetic study at Yale and Columbia Universities. An in-person evaluation included a videotaped neurological examination, including a detailed assessment of tremors. A senior movement disorders neurologist reviewed all videotaped examinations, and the severity of postural and kinetic arm tremors was rated on 12 examination items using a reliable rating scale. The tremor asymmetry index = right arm tremor score − left arm tremor score. We used a bivariate linear regression model to assess the predictors of the tremor asymmetry index in relatives; this model used the tremor asymmetry index in the proband as a primary predictor of interest. In an analysis of variance (ANOVA), we tested for heterogeneity across families in the tremor asymmetry index (i.e., to see whether there was a significant family effect). RESULTS: There were 187 enrollees (59 probands, 128 affected relatives). In a bivariate linear regression model, the tremor asymmetry index in the proband was not a predictor of the tremor asymmetry index in their relatives (p = 0.66). In an ANOVA, family grouping did not explain a significant proportion of the total variance in the tremor asymmetry index (p = 0.56). CONCLUSION: Tremor asymmetry did not aggregate in families with ET. Therefore, this does not seem to be a disease feature that is heritable. These data will provide added value to the clinical dialog, giving patients one more piece of information about the way the disease manifests within families.
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spelling pubmed-53956462017-05-03 Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor Louis, Elan D. Hernandez, Nora Chen, Karen P. Naranjo, Kelly V. Park, Jemin Clark, Lorraine N. Ottman, Ruth Front Neurol Neuroscience BACKGROUND: Action tremor is the hallmark feature of essential tremor (ET). While the tremor typically is mildly asymmetric, in some patients, it is markedly asymmetric. There are few data on factors that influence this asymmetry. ET is also a highly familial disease. Whether the tremor asymmetry profile (i.e., differential expression of tremor in each arm in a given patient) is similar across family members is not known. The alternative possibility is that this feature is not heritable. There are no published data addressing this issue. The aim of this study was to determine whether the extent of action tremor asymmetry ran in ET families. METHODS: ET probands and relatives were enrolled in a genetic study at Yale and Columbia Universities. An in-person evaluation included a videotaped neurological examination, including a detailed assessment of tremors. A senior movement disorders neurologist reviewed all videotaped examinations, and the severity of postural and kinetic arm tremors was rated on 12 examination items using a reliable rating scale. The tremor asymmetry index = right arm tremor score − left arm tremor score. We used a bivariate linear regression model to assess the predictors of the tremor asymmetry index in relatives; this model used the tremor asymmetry index in the proband as a primary predictor of interest. In an analysis of variance (ANOVA), we tested for heterogeneity across families in the tremor asymmetry index (i.e., to see whether there was a significant family effect). RESULTS: There were 187 enrollees (59 probands, 128 affected relatives). In a bivariate linear regression model, the tremor asymmetry index in the proband was not a predictor of the tremor asymmetry index in their relatives (p = 0.66). In an ANOVA, family grouping did not explain a significant proportion of the total variance in the tremor asymmetry index (p = 0.56). CONCLUSION: Tremor asymmetry did not aggregate in families with ET. Therefore, this does not seem to be a disease feature that is heritable. These data will provide added value to the clinical dialog, giving patients one more piece of information about the way the disease manifests within families. Frontiers Media S.A. 2017-04-19 /pmc/articles/PMC5395646/ /pubmed/28469594 http://dx.doi.org/10.3389/fneur.2017.00148 Text en Copyright © 2017 Louis, Hernandez, Chen, Naranjo, Park, Clark and Ottman. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Louis, Elan D.
Hernandez, Nora
Chen, Karen P.
Naranjo, Kelly V.
Park, Jemin
Clark, Lorraine N.
Ottman, Ruth
Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title_full Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title_fullStr Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title_full_unstemmed Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title_short Action Tremor Asymmetry Profile Does Not Aggregate in Families with Essential Tremor
title_sort action tremor asymmetry profile does not aggregate in families with essential tremor
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395646/
https://www.ncbi.nlm.nih.gov/pubmed/28469594
http://dx.doi.org/10.3389/fneur.2017.00148
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