Cargando…
The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population
BACKGROUND AND PURPOSE: The etiologies and frequencies of cerebellar ataxias vary between countries. Our primary aim was to determine the frequency of each diagnostic group of cerebellar ataxia patients in a Korean population. METHODS: We reviewed the medical records of patients who were being follo...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Neurological Association
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6032000/ https://www.ncbi.nlm.nih.gov/pubmed/29971977 http://dx.doi.org/10.3988/jcn.2018.14.3.374 |
_version_ | 1783337424785506304 |
---|---|
author | Kim, Ji Sun Kwon, Soonwook Ki, Chang-Seok Youn, Jinyoung Cho, Jin Whan |
author_facet | Kim, Ji Sun Kwon, Soonwook Ki, Chang-Seok Youn, Jinyoung Cho, Jin Whan |
author_sort | Kim, Ji Sun |
collection | PubMed |
description | BACKGROUND AND PURPOSE: The etiologies and frequencies of cerebellar ataxias vary between countries. Our primary aim was to determine the frequency of each diagnostic group of cerebellar ataxia patients in a Korean population. METHODS: We reviewed the medical records of patients who were being followed up between November 1994 and February 2016. We divided patients with cerebellar ataxias into familial and non-familial groups and analyzed the frequency of each etiology. Finally, we categorized patients into genetic, sporadic, secondary, and suspected genetic, but undetermined ataxia. RESULTS: A total of 820 patients were included in the study, among whom 136 (16.6%) familial patients and 684 (83.4%) non-familial cases were identified. Genetic diagnoses confirmed 98/136 (72%) familial and 72/684 (11%) nonfamilial patients. The overall etiologies of progressive ataxias comprised 170 (20.7%) genetic, 516 (62.9%) sporadic, 43 (5.2%) secondary, and 91 (11.1%) undetermined ataxia. The most common cause of ataxia was multiple-system atrophy (57.3%). In the genetic group, the most common etiology was spinocerebellar ataxia (152/170, 89.4%) and the most common subtype was spinocerebellar ataxia-3.38 of 136 familial and 53 of 684 sporadic cases (91/820, 11.1%) were undetermined ataxia. CONCLUSIONS: This is the largest epidemiological study to analyze the frequencies of various cerebellar ataxias in a Korean population based on the large database of a tertiary hospital movement-disorders clinic in South Korea. These data would be helpful for clinicians in constructing diagnostic strategies and counseling for patients with cerebellar ataxias. |
format | Online Article Text |
id | pubmed-6032000 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Korean Neurological Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-60320002018-07-06 The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population Kim, Ji Sun Kwon, Soonwook Ki, Chang-Seok Youn, Jinyoung Cho, Jin Whan J Clin Neurol Original Article BACKGROUND AND PURPOSE: The etiologies and frequencies of cerebellar ataxias vary between countries. Our primary aim was to determine the frequency of each diagnostic group of cerebellar ataxia patients in a Korean population. METHODS: We reviewed the medical records of patients who were being followed up between November 1994 and February 2016. We divided patients with cerebellar ataxias into familial and non-familial groups and analyzed the frequency of each etiology. Finally, we categorized patients into genetic, sporadic, secondary, and suspected genetic, but undetermined ataxia. RESULTS: A total of 820 patients were included in the study, among whom 136 (16.6%) familial patients and 684 (83.4%) non-familial cases were identified. Genetic diagnoses confirmed 98/136 (72%) familial and 72/684 (11%) nonfamilial patients. The overall etiologies of progressive ataxias comprised 170 (20.7%) genetic, 516 (62.9%) sporadic, 43 (5.2%) secondary, and 91 (11.1%) undetermined ataxia. The most common cause of ataxia was multiple-system atrophy (57.3%). In the genetic group, the most common etiology was spinocerebellar ataxia (152/170, 89.4%) and the most common subtype was spinocerebellar ataxia-3.38 of 136 familial and 53 of 684 sporadic cases (91/820, 11.1%) were undetermined ataxia. CONCLUSIONS: This is the largest epidemiological study to analyze the frequencies of various cerebellar ataxias in a Korean population based on the large database of a tertiary hospital movement-disorders clinic in South Korea. These data would be helpful for clinicians in constructing diagnostic strategies and counseling for patients with cerebellar ataxias. Korean Neurological Association 2018-07 2018-06-29 /pmc/articles/PMC6032000/ /pubmed/29971977 http://dx.doi.org/10.3988/jcn.2018.14.3.374 Text en Copyright © 2018 Korean Neurological Association http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Kim, Ji Sun Kwon, Soonwook Ki, Chang-Seok Youn, Jinyoung Cho, Jin Whan The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title | The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title_full | The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title_fullStr | The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title_full_unstemmed | The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title_short | The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population |
title_sort | etiologies of chronic progressive cerebellar ataxia in a korean population |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6032000/ https://www.ncbi.nlm.nih.gov/pubmed/29971977 http://dx.doi.org/10.3988/jcn.2018.14.3.374 |
work_keys_str_mv | AT kimjisun theetiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT kwonsoonwook theetiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT kichangseok theetiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT younjinyoung theetiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT chojinwhan theetiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT kimjisun etiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT kwonsoonwook etiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT kichangseok etiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT younjinyoung etiologiesofchronicprogressivecerebellarataxiainakoreanpopulation AT chojinwhan etiologiesofchronicprogressivecerebellarataxiainakoreanpopulation |