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KMID : 0359720180360010035
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Journal of the Korean Neurological Association
2018 Volume.36 No. 1 p.35 ~ p.39
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An Autopsy Confirmed Case of Semantic Variant Primary Progressive Aphasia with Frontotemporal Lobar Degeneration-TDP type C
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Jung Na-Yeon
Lee Myung-Jun
Lee Jae-Hyeok
Shin Jin-Hong
Lee Young-Min
Shin Myung-Jun
Park Kyoung-June
Hwang Chung-Su
Ahn Jae-Woo
Sung Suk
Choi Kyung-Un
Huh Gi-Yeong
Kim Eun-Joo
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Abstract
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A 62-year-old man presented with a one-year history of word finding difficulty, impaired single word comprehension and personality changes including aggression, apathy and eating change. Brain MRIs showed severe atrophy in the left anterior temporal lobe. The clinical syndromic diagnosis was semantic variant primary progressive aphasia. He died at age 70 of pneumonia. At autopsy, transactive response DNA-binding protein (TDP) immunoreactive long dystrophic neurites were predominantly found in the cerebral cortices, which were compatible with frontotemporal lobar degeneration-TDP type C pathology.
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KEYWORD
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Semantic dementia, Frontotemporal lobar degeneration, TDP-43 proteinopathies
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