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Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia
BACKGROUND: Clinical studies have implicated the cerebellum in the pathogenesis of essential tremor (ET), and recent postmortem studies have identified structural changes in the ET cerebellum. While the basal ganglia have traditionally been implicated in dystonia, cerebellar involvement has been sug...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Columbia University Libraries/Information Services
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3535836/ https://www.ncbi.nlm.nih.gov/pubmed/23439731 |
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author | Ma, Karen Babij, Rachel Cortés, Etty Vonsattel, Jean‐Paul G. Louis, Elan D. |
author_facet | Ma, Karen Babij, Rachel Cortés, Etty Vonsattel, Jean‐Paul G. Louis, Elan D. |
author_sort | Ma, Karen |
collection | PubMed |
description | BACKGROUND: Clinical studies have implicated the cerebellum in the pathogenesis of essential tremor (ET), and recent postmortem studies have identified structural changes in the ET cerebellum. While the basal ganglia have traditionally been implicated in dystonia, cerebellar involvement has been suggested as well, and a recent study showed Purkinje cell (PC) loss. We conducted a detailed postmortem examination of the brain in four individuals with clinical diagnoses of ET and dystonia, and hypothesized that pathological changes in the cerebellum would be greater in these four ET cases than in published ET cases without dystonia. METHODS: After a complete neuropathological assessment, a standard parasagittal neocerebellar tissue block was harvested in each brain. One 7‐µm thick section was stained with luxol fast blue/hematoxylin and eosin, and one section with the Bielschowsky method. We quantified PCs, torpedoes, heterotopic PCs, PC dendritic swellings, and basket cell changes. RESULTS: Two ET+dystonia cases had more microscopic changes in the cerebellum than published ET cases; the other two cases had similar changes to published ET cases. DISCUSSION: This is the first report that uses human autopsy tissue to study patients with both ET and dystonia. The findings were heterogeneous. Additional studies, with larger samples, are needed. |
format | Online Article Text |
id | pubmed-3535836 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Columbia University Libraries/Information Services |
record_format | MEDLINE/PubMed |
spelling | pubmed-35358362013-04-11 Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia Ma, Karen Babij, Rachel Cortés, Etty Vonsattel, Jean‐Paul G. Louis, Elan D. Tremor Other Hyperkinet Mov (N Y) Brief Report BACKGROUND: Clinical studies have implicated the cerebellum in the pathogenesis of essential tremor (ET), and recent postmortem studies have identified structural changes in the ET cerebellum. While the basal ganglia have traditionally been implicated in dystonia, cerebellar involvement has been suggested as well, and a recent study showed Purkinje cell (PC) loss. We conducted a detailed postmortem examination of the brain in four individuals with clinical diagnoses of ET and dystonia, and hypothesized that pathological changes in the cerebellum would be greater in these four ET cases than in published ET cases without dystonia. METHODS: After a complete neuropathological assessment, a standard parasagittal neocerebellar tissue block was harvested in each brain. One 7‐µm thick section was stained with luxol fast blue/hematoxylin and eosin, and one section with the Bielschowsky method. We quantified PCs, torpedoes, heterotopic PCs, PC dendritic swellings, and basket cell changes. RESULTS: Two ET+dystonia cases had more microscopic changes in the cerebellum than published ET cases; the other two cases had similar changes to published ET cases. DISCUSSION: This is the first report that uses human autopsy tissue to study patients with both ET and dystonia. The findings were heterogeneous. Additional studies, with larger samples, are needed. Columbia University Libraries/Information Services 2012-08-06 /pmc/articles/PMC3535836/ /pubmed/23439731 Text en © 2012 Ma et al http://creativecommons.org/licenses/by-nc-nd/3.0/us/ This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommerical–No Derivatives License, which permits the user to copy, distribute, and transmit the work provided that the original author and source are credited; that no commercial use is made of the work; and that the work is not altered or transformed. |
spellingShingle | Brief Report Ma, Karen Babij, Rachel Cortés, Etty Vonsattel, Jean‐Paul G. Louis, Elan D. Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title | Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title_full | Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title_fullStr | Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title_full_unstemmed | Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title_short | Cerebellar Pathology of a Dual Clinical Diagnosis: Patients with Essential Tremor and Dystonia |
title_sort | cerebellar pathology of a dual clinical diagnosis: patients with essential tremor and dystonia |
topic | Brief Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3535836/ https://www.ncbi.nlm.nih.gov/pubmed/23439731 |
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