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Phosphorylated trans\u2010activation response DNA\u2010binding protein of 43 kDa (TDP\u201043) inclusion bodies are reportedly a pathological hallmark of sporadic ALS. Here, we present an atypical case of sporadic ALS that progressed very slowly, persisted for 19 years, and clinically appeared to only affect the lower motor neurons; however, upper motor neuron degeneration was detected at autopsy. Furthermore, no inclusion bodies positive for phosphorylated TDP\u201043, ubiquitin, fused in sarcoma, or superoxide dismutase\u20101 were detected in the central nervous system. We performed exome\u2010sequencing data analysis but found no genetic disorders. This was therefore an unusual case of lower motor neuron\u2010predominant ALS without TDP\u201043 pathology or known gene\u2010disease associations. We also reviewed autopsied ALS cases that progressed slowly and had no phosphorylated TDP\u201043 or ubiquitin\u2010positive inclusions and present the clinicopathological features of such cases. Based on these results, there may be a sporadic ALS subgroup that progresses slowly and shows no accumulation of phosphorylated TDP\u201043.<\/jats:p>","DOI":"10.1111\/neup.12523","type":"journal-article","created":{"date-parts":[[2018,12,4]],"date-time":"2018-12-04T03:09:37Z","timestamp":1543892977000},"page":"47-53","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Amyotrophic lateral sclerosis of long clinical course clinically presenting with progressive muscular atrophy"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7559-8284","authenticated-orcid":false,"given":"Tomoyasu","family":"Matsubara","sequence":"first","affiliation":[{"name":"Department of Neurology Mifukai Vihara Hananosato Hospital  Hiroshima Japan"},{"name":"Department of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences  Hiroshima Japan"},{"name":"Department of Neurology and Neuropathology (Brain Bank for Aging Research) Tokyo Metropolitan Geriatric Hospital &amp; Institute of Gerontology  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaya","family":"Oda","sequence":"additional","affiliation":[{"name":"Department of Neurology Mifukai Vihara Hananosato Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuya","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chigusa","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Hiroshima\u2010Nishi Medical Center  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiro","family":"Tachiyama","sequence":"additional","affiliation":[{"name":"Department of Clinical Laboratory National Hospital Organization Hiroshima\u2010Nishi Medical Center  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Morino","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, Research Institute for Radiation Biology and Medicine Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideshi","family":"Kawakami","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, Research Institute for Radiation Biology and Medicine Hiroshima University  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryuji","family":"Kaji","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience, Institute of Biomedical Sciences Tokushima University Graduate School  Tokushima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirofumi","family":"Maruyama","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9709-5242","authenticated-orcid":false,"given":"Shigeo","family":"Murayama","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neuropathology (Brain Bank for Aging Research) Tokyo Metropolitan Geriatric Hospital &amp; 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