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KMID : 1130620180140030374
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Journal of Clinical Neurology
2018 Volume.14 No. 3 p.374 ~ p.380
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The Etiologies of Chronic Progressive Cerebellar Ataxia in a Korean Population
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Kim Ji-Sun
Kwon Soon-Wook
Ki Chang-Seok
Youn Jin-Young
Cho Jin-Whan
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Abstract
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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.
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KEYWORD
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cerebellar ataxia, spinocerebellar ataxia, cerebellar variant of multiple-system atrophy, sporadic adult-onset ataxia of unknown etiology, Friedreich's ataxia
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