Journal List > Dement Neurocogn Disord > v.13(1) > 1120722

Dement Neurocogn Disord. 2014 Mar;13(1):16-19. Korean.
Published online Mar 31, 2014.  https://doi.org/10.12779/dnd.2014.13.1.16
© 2014 Korean Dementia Association
Spinocerebellar Ataxia Type 2 with Only Clinical Feature of Memory Impairment: Case Report
JaeJeong Joo, M.D., Sang Wo Han, M.D., Sang Won Ha, M.D., Jeong Ho Han, M.D., Doo Eung Kim, M.D. and YoungSoon Yang, M.D.
Department of Neurology, SVH Medical Center, Seoul, Korea.

Address for correspondence: YoungSoon Yang, M.D. Department of Neurology, SVH Medical Center, 53 Jinhwangdo-ro 61-gil, Gangdong-gu, Seoul 134-791, Korea. Tel: +82-2-2225-4106, Fax: +82-2-2225-4105, Email: astro76@naver.com
Received Mar 18, 2014; Revised Mar 26, 2014; Accepted Mar 26, 2014.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.


Abstract

Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. There are more than 35 different types of spinocerebellar ataxias, each caused by a different genetic mutation. Spinocerebellar ataxia type 2 (SCA2) is a subtype of type I autosomal dominant cerebellar ataxia (ADCA type I) characterized by truncal ataxia, dysarthria, slowed saccades and less commonly ophthalmoparesis and chorea. The age at onset varies from 3 to 79 years (mean 33). Usually, the first symptom of the disease is the gait ataxia, followed by the cerebellar dysarthria. Of late, other clinical manifestations of SCA2 are the cognitive dysfunctions, which include frontal executive impairment, verbal short-term memory deficits as well as reduction of attention and concentration. We report a 56-year old woman identified as spinocerebellar ataxia type 2 (SCA2) with only clinical feature of memory impairment.

Keywords: Spinocerebellar ataxia type 2; Memory impairment

Figures


Fig. 1
Pedigree of the present family.
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Fig. 2
Brain MR image shows no definite abnormal findings.
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Fig. 3
Brain positron emission tomography shows no significant focal metabolic abnormality in the cerebral hemispheres.
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Tables


Table 1
Neuropsychological test
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Table 2
Ataxia Trinucleotide Repeat Analysis Report
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