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We report an autopsy case of lower motor neuron\u2010predominant ALS in a 47\u2010year\u2010old Japanese man who committed suicide 5\u2009months after onset. Histopathologically, neuronal loss was restricted to the anterior horn of the spinal cord, and no obvious neuronal loss was noted in the motor cortex or brainstem motor nuclei. Bunina bodies were found in the spinal anterior horn cells and the facial and hypoglossal nuclei. Immunohistochemically, p\u2010TDP\u201043\u2010immunoreactive neuronal, but not glial, cytoplasmic inclusions were frequently found in the spinal anterior horn and facial and hypoglossal nuclei, and rarely in the motor cortex. We considered the present case to be an example of lower motor neuron\u2010predominant ALS. p\u2010TDP\u201043\u2010immunoreactive aggregates in neurons, but not in glial cells, may be an early\u2010stage pathology of ALS.<\/jats:p>","DOI":"10.1111\/neup.12554","type":"journal-article","created":{"date-parts":[[2019,4,25]],"date-time":"2019-04-25T04:41:20Z","timestamp":1556167280000},"page":"224-230","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["An autopsy case of early\u2010stage amyotrophic lateral sclerosis with TDP\u201043 immunoreactive neuronal, but not glial, inclusions"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6072-3954","authenticated-orcid":false,"given":"Tomoya","family":"Kon","sequence":"first","affiliation":[{"name":"Department of Neuropathology Hirosaki University Graduate School of Medicine  Hirosaki 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