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Non-Ataxic Phenotypes of SCA8 Mimicking Amyotrophic Lateral Sclerosis and Parkinson Disease
김지선,손태옥,기창석,조진환 대한신경과학회 2013 Journal of Clinical Neurology Vol.9 No.4
Background Spinocerebellar ataxia (SCA) type 8 (SCA8) is an inherited neurodegenerative disorder caused by the expansion of untranslated CTA/CTG triplet repeats on 13q21. The phenomenology of SCA8 is relatively varied when compared to the other types of SCAs and its spectrum is not well established. Case Report Two newly detected cases of SCA8 with the nonataxic phenotype and unusual clinical manifestations such as dopaminergic-treatment-responsive parkinsonism and amyotrophic lateral sclerosis (ALS) are described herein. Family A expressed good dopaminergic treatment-responsive parkinsonism as an initial manifestation and developed mild cerebellar ataxia with additional movements, including dystonic gait and unusual oscillatory movement of the trunk, during the disease course. The proband of family B presented as probable ALS with cerebellar atrophy on brain MRI, with a positive family history (a brother with typical cerebellar ataxia) and genetic confirmation for SCA8. Conclusions Our findings support that the non-ataxic phenotypes could be caused by a mutation of the SCA8 locus which might affect neurons other than the cerebellum.
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태안 신진도 고가(古家) 수군(水軍) 군적부(軍籍簿) 연구
진호신,박상준,손태옥 국립해양문화재연구소 2020 해양문화재 Vol.13 No.-
The naval forces stationed in Anheungnyang straits served as a support group to prevent Japanese invaders and to support Ganghwa Island in case of emergency from Goryo to Chosun dynasty. In particular, the mission of the naval forces was to prevent accidents and control the cargo vessel passing through the waters off Anheungryang strait. The naval military register of the navy found in the old house of Taean Shinjin Island is important in that the documents managed by the state were found in shinjin Island, a naval base, after the 16th century. Judging from the format and timing of the formation, the purpose of this naval forces register seems to have been to collect hemp or cotton cloth, which is method of imposing military service rather than requisition by the navy. In the text, the contents of the naval military resister were analyzed by name, residence, height, and age, and it was confirmed that the military resister was written in Dangjin-hyeon in the early 19th century. Therefore, this paper intended to analyze the operation and status of Anheungjin naval forces through receiving hemp or cotton cloth in Dangjin area. 안흥량 일대 주둔 수군은 고려부터 조선까지 지속된 왜구의 침입을 막고, 유사시 강도(江都)를 지원하기 위한 후원군 역할을 하였다. 특히 안흥량 일대를 통행하는 조운선의 사고방지와 통제가 중요한 임무였다. 이번 태안 신진도 고가(古家)에서 발견된 수군 군적부는 16세기 이후 수군편성 체계를 실질적으로 확인할 수 있는 문서이며, 국가에서 관리하던 문서가 수군 주둔지역인 신진도에서 발견되었다는 점에서 중요한 의미를 갖는다. 이 군적부의 용도는 작성 형식이나 시기로 미루어 보아 수군의 징발보다는 군역 부과방식인 군포(軍布)의 수취를 주목적으로 하였던 것으로 보인다. 본문에서는 군적부 내용을 이름, 거주지역, 신장, 나이 등에 따라 분석하였고, 이 군적부가 19세기 초반 당진현에서 작성된 문서라는 것을 확인하였다. 따라서 이 논문은 당진지역에서 군포 수취라는 목적을 통해 안흥진 수군의 운영과 실태에 대하여 분석하고자 하였다.
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조은빈,김석재,차지훈,손태옥,김준표,방오영,김경문,정진상,이광호 대한신경과학회 2013 대한신경과학회지 Vol.31 No.4
Primary angiitis of the central nervous system (PACNS) is a poorly understood form of vascular inflammatory disease that is restricted to the brain and spinal cord. A 38-year-old woman presented with severe headache and transient aphasia. Her cerebrospinal fluid exhibited aseptic meningitis, and high-resolution vessel-wall MRI (HRVW-MRI)revealed narrowing of multiple intracranial vessels with concentric wall thickening and diffuse enhancement, suggestive of PACNS. High-dose steroid and azathioprine therapy resulted in a significant improvement in vessel wall thickening,and enhancement was observed on the follow-up HRVW-MRI.
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