“ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases

Background: A recent consensus statement introduced the term “ET plus”. Although investigators have quantified the prevalence of ET plus in cross-sectional studies, patients with ET plus have not been tracked longitudinally; hence, there is no understanding of its stability over time.Methods: We pre...

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Main Authors: Daniella Iglesias-Hernandez, Nikki Delgado, Margaret McGurn, Edward D. Huey, Stephanie Cosentino, Elan D. Louis
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-12-01
Series:Frontiers in Neurology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fneur.2021.782694/full
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author Daniella Iglesias-Hernandez
Nikki Delgado
Margaret McGurn
Edward D. Huey
Edward D. Huey
Edward D. Huey
Stephanie Cosentino
Stephanie Cosentino
Elan D. Louis
author_facet Daniella Iglesias-Hernandez
Nikki Delgado
Margaret McGurn
Edward D. Huey
Edward D. Huey
Edward D. Huey
Stephanie Cosentino
Stephanie Cosentino
Elan D. Louis
author_sort Daniella Iglesias-Hernandez
collection DOAJ
description Background: A recent consensus statement introduced the term “ET plus”. Although investigators have quantified the prevalence of ET plus in cross-sectional studies, patients with ET plus have not been tracked longitudinally; hence, there is no understanding of its stability over time.Methods: We present prospective, longitudinal phenotypic data on an ET cohort that was followed regularly at 18-month intervals (T1, T2, T3, T4) for up to 64 months. We assigned an ET or ET plus diagnosis to each case at each time interval.Results: There were 201 participants at baseline. The proportion with ET plus increased from 58.7% at baseline to 72.1% at T4 (p = 0.046). Of 172 (85.6%) who received a diagnosis of ET plus at one or more time intervals, the diagnosis was unstable (e.g., with reversion) in 62 (36.0%). We also assessed the stability of the clinical features of ET plus. Rest tremor was the most unstable clinical feature of ET plus; it was present in 59 participants, among whom it reverted from present to absent in 23 (39.0%). By contrast, for “memory impairment” (i.e., either mild cognitive impairment or dementia), the proportion who reverted from present to absent was only 21.3%.Conclusion: These data support our two a priori hypotheses: (1) the prevalence of ET plus would increase progressively, as it likely represents a more advanced stage of ET, and (2) the ET plus diagnosis would not be stable over time, as cases would fluctuate with respect to their phenotypic features and their assigned diagnoses.
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spelling doaj.art-2f023820c0984efe8a82233a3b1afa542022-12-21T18:11:56ZengFrontiers Media S.A.Frontiers in Neurology1664-22952021-12-011210.3389/fneur.2021.782694782694“ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor CasesDaniella Iglesias-Hernandez0Nikki Delgado1Margaret McGurn2Edward D. Huey3Edward D. Huey4Edward D. Huey5Stephanie Cosentino6Stephanie Cosentino7Elan D. Louis8Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, United StatesDepartment of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, United StatesDepartment of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, United StatesDepartment of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, United StatesTaub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, NY, United StatesDepartment of Psychiatry, College of Physicians and Surgeons, Columbia University, New York, NY, United StatesDepartment of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, United StatesTaub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, New York, NY, United StatesDepartment of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, United StatesBackground: A recent consensus statement introduced the term “ET plus”. Although investigators have quantified the prevalence of ET plus in cross-sectional studies, patients with ET plus have not been tracked longitudinally; hence, there is no understanding of its stability over time.Methods: We present prospective, longitudinal phenotypic data on an ET cohort that was followed regularly at 18-month intervals (T1, T2, T3, T4) for up to 64 months. We assigned an ET or ET plus diagnosis to each case at each time interval.Results: There were 201 participants at baseline. The proportion with ET plus increased from 58.7% at baseline to 72.1% at T4 (p = 0.046). Of 172 (85.6%) who received a diagnosis of ET plus at one or more time intervals, the diagnosis was unstable (e.g., with reversion) in 62 (36.0%). We also assessed the stability of the clinical features of ET plus. Rest tremor was the most unstable clinical feature of ET plus; it was present in 59 participants, among whom it reverted from present to absent in 23 (39.0%). By contrast, for “memory impairment” (i.e., either mild cognitive impairment or dementia), the proportion who reverted from present to absent was only 21.3%.Conclusion: These data support our two a priori hypotheses: (1) the prevalence of ET plus would increase progressively, as it likely represents a more advanced stage of ET, and (2) the ET plus diagnosis would not be stable over time, as cases would fluctuate with respect to their phenotypic features and their assigned diagnoses.https://www.frontiersin.org/articles/10.3389/fneur.2021.782694/fullessential tremorET plusclassificationdiagnosisclinical
spellingShingle Daniella Iglesias-Hernandez
Nikki Delgado
Margaret McGurn
Edward D. Huey
Edward D. Huey
Edward D. Huey
Stephanie Cosentino
Stephanie Cosentino
Elan D. Louis
“ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
Frontiers in Neurology
essential tremor
ET plus
classification
diagnosis
clinical
title “ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
title_full “ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
title_fullStr “ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
title_full_unstemmed “ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
title_short “ET Plus”: Instability of the Diagnosis During Prospective Longitudinal Follow-up of Essential Tremor Cases
title_sort et plus instability of the diagnosis during prospective longitudinal follow up of essential tremor cases
topic essential tremor
ET plus
classification
diagnosis
clinical
url https://www.frontiersin.org/articles/10.3389/fneur.2021.782694/full
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