The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population.
10.3988/jcn.2018.14.3.374
- Author:
Ji Sun KIM
1
;
Soonwook KWON
;
Chang Seok KI
;
Jinyoung YOUN
;
Jin Whan CHO
Author Information
1. Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. jinwhan.cho@samsung.com
- Publication Type:Original Article
- Keywords:
cerebellar ataxia;
spinocerebellar ataxia;
cerebellar variant of multiple-system atrophy;
sporadic adult-onset ataxia of unknown etiology;
Friedreich's ataxia
- MeSH:
Ataxia;
Atrophy;
Cerebellar Ataxia*;
Counseling;
Diagnosis;
Epidemiologic Studies;
Friedreich Ataxia;
Humans;
Korea;
Medical Records;
Spinocerebellar Ataxias;
Tertiary Care Centers
- From:Journal of Clinical Neurology
2018;14(3):374-380
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
