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KMID : 0359720220400020164
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Journal of the Korean Neurological Association
2022 Volume.40 No. 2 p.164 ~ p.167
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An Autopsy Confirmed Case of Amyotrophic Lateral Sclerosis with TDP Pathology
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Je Yu-Ri
Kim Soo-Yeon
Sung Jung-Joon
Lee Myung-Jun
Jung Na-Yeon
Lee Jae-Hyeok
Shin Jin-Hong
Lee Young-Min
Yoon Jin-A
Pak Kyoung-June
Ko Jun-Kyeung
Lee Jae-Meen
Hwang Cheong-Su
Ahn Jae-Woo
Sung Suk
Choi Kyung-Un
Huh Gi-Yeong
Kim Eun-Joo
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Abstract
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The phosphorylated 43-kDa transactive response DNA-binding protein (TDP-43) was identified as a major disease protein in sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. We present a case with progressive muscle weakness who was diagnosed with sporadic ALS. On postmortem examination, TDP-43 immunoreactive neuronal cytoplasmic inclusions were noted in motor cortex, hippocampus and anterior horns of spinal cord, which was compatible with ALS-TDP, stage 4. This is the first documented autopsy-confirmed ALS case with ALS-TDP pathology in Korea.
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KEYWORD
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Amyotrophic lateral sclerosis, TDP, Autopsy
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