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Ataxia Prevalence in Primary Orthostatic Tremor

BACKGROUND: The exact pathophysiology of primary Orthostatic Tremor (OT) is unknown. A central oscillator is assumed, and previous imaging studies show involvement of cerebellar pathways. However, the presence of ataxia on clinical exam is disputed. We set out to study ataxia in OT prospectively. ME...

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Autores principales: Thompson, Rebecca, Bhatti, Danish E., Hellman, Amy, Doss, Sarah J., Malgireddy, Kalyan, Shou, James, Srikanth-Mysore, Channaiah, Bendi, Sunil, Bertoni, John M., Torres-Russotto, Diego
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Ubiquity Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7747757/
https://www.ncbi.nlm.nih.gov/pubmed/33362948
http://dx.doi.org/10.5334/tohm.570
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author Thompson, Rebecca
Bhatti, Danish E.
Hellman, Amy
Doss, Sarah J.
Malgireddy, Kalyan
Shou, James
Srikanth-Mysore, Channaiah
Bendi, Sunil
Bertoni, John M.
Torres-Russotto, Diego
author_facet Thompson, Rebecca
Bhatti, Danish E.
Hellman, Amy
Doss, Sarah J.
Malgireddy, Kalyan
Shou, James
Srikanth-Mysore, Channaiah
Bendi, Sunil
Bertoni, John M.
Torres-Russotto, Diego
author_sort Thompson, Rebecca
collection PubMed
description BACKGROUND: The exact pathophysiology of primary Orthostatic Tremor (OT) is unknown. A central oscillator is assumed, and previous imaging studies show involvement of cerebellar pathways. However, the presence of ataxia on clinical exam is disputed. We set out to study ataxia in OT prospectively. METHODS: EMG-confirmed primary OT subjects and spousal controls received a neurological exam with additional semiquantitative evaluations of ataxia as part of a multinational, prospective study. These included detailed limb coordination (DLC), detailed stance and gait evaluation (DS), and the Brief Ataxia Rating Scale (BARS). Intra- and inter-rater reliability were assessed and satisfactory. RESULTS: 34 OT subjects (mean age = 67 years, 88% female) and 21 controls (mean age = 66 years, 65% male) were enrolled. Average disease duration was 18 years (range 4–44). BARS items were abnormal in 88% of OT patients. The OT subjects were more likely to have appendicular and truncal ataxia with significant differences in DLC, DS and BARS. Ocular ataxia and dysarthria were not statistically different between the groups. DISCUSSION: Mild-to-moderate ataxia could be more common in OT than previously thought. This is supportive of cerebellar involvement in the pathophysiology of OT. We discuss possible implications for clinical care and future research. HIGHLIGHTS: Previous studies of Primary Orthostatic Tremor (OT) have proposed pathophysiologic involvement of the cerebellar pathways. However, presence of ataxia has not been systematically studied in OT. This is a prospective comprehensive ataxia assessment in OT compared to controls. Mild-to-moderate appendiculo-truncal ataxia was found to be common in OT.
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spelling pubmed-77477572020-12-24 Ataxia Prevalence in Primary Orthostatic Tremor Thompson, Rebecca Bhatti, Danish E. Hellman, Amy Doss, Sarah J. Malgireddy, Kalyan Shou, James Srikanth-Mysore, Channaiah Bendi, Sunil Bertoni, John M. Torres-Russotto, Diego Tremor Other Hyperkinet Mov (N Y) Brief Report BACKGROUND: The exact pathophysiology of primary Orthostatic Tremor (OT) is unknown. A central oscillator is assumed, and previous imaging studies show involvement of cerebellar pathways. However, the presence of ataxia on clinical exam is disputed. We set out to study ataxia in OT prospectively. METHODS: EMG-confirmed primary OT subjects and spousal controls received a neurological exam with additional semiquantitative evaluations of ataxia as part of a multinational, prospective study. These included detailed limb coordination (DLC), detailed stance and gait evaluation (DS), and the Brief Ataxia Rating Scale (BARS). Intra- and inter-rater reliability were assessed and satisfactory. RESULTS: 34 OT subjects (mean age = 67 years, 88% female) and 21 controls (mean age = 66 years, 65% male) were enrolled. Average disease duration was 18 years (range 4–44). BARS items were abnormal in 88% of OT patients. The OT subjects were more likely to have appendicular and truncal ataxia with significant differences in DLC, DS and BARS. Ocular ataxia and dysarthria were not statistically different between the groups. DISCUSSION: Mild-to-moderate ataxia could be more common in OT than previously thought. This is supportive of cerebellar involvement in the pathophysiology of OT. We discuss possible implications for clinical care and future research. HIGHLIGHTS: Previous studies of Primary Orthostatic Tremor (OT) have proposed pathophysiologic involvement of the cerebellar pathways. However, presence of ataxia has not been systematically studied in OT. This is a prospective comprehensive ataxia assessment in OT compared to controls. Mild-to-moderate appendiculo-truncal ataxia was found to be common in OT. Ubiquity Press 2020-12-16 /pmc/articles/PMC7747757/ /pubmed/33362948 http://dx.doi.org/10.5334/tohm.570 Text en Copyright: © 2020 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC-BY 4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. See http://creativecommons.org/licenses/by/4.0/.
spellingShingle Brief Report
Thompson, Rebecca
Bhatti, Danish E.
Hellman, Amy
Doss, Sarah J.
Malgireddy, Kalyan
Shou, James
Srikanth-Mysore, Channaiah
Bendi, Sunil
Bertoni, John M.
Torres-Russotto, Diego
Ataxia Prevalence in Primary Orthostatic Tremor
title Ataxia Prevalence in Primary Orthostatic Tremor
title_full Ataxia Prevalence in Primary Orthostatic Tremor
title_fullStr Ataxia Prevalence in Primary Orthostatic Tremor
title_full_unstemmed Ataxia Prevalence in Primary Orthostatic Tremor
title_short Ataxia Prevalence in Primary Orthostatic Tremor
title_sort ataxia prevalence in primary orthostatic tremor
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7747757/
https://www.ncbi.nlm.nih.gov/pubmed/33362948
http://dx.doi.org/10.5334/tohm.570
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