{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:45:53Z","timestamp":1747187153962,"version":"3.40.5"},"reference-count":36,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,6,30]],"date-time":"2022-06-30T00:00:00Z","timestamp":1656547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["20K16592"],"award-info":[{"award-number":["20K16592"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:p>We report the case of a Japanese woman with sporadic amyotrophic lateral sclerosis (ALS) of 28\u2009months\u2019 duration who died at the age of 66\u2009years. Postmortem examination revealed moderate loss of neurons and phosphorylated TDP\u201043 (p\u2010TDP\u201043)\u2010immunoreactive neuronal and glial cytoplasmic inclusions in the upper and lower motor neurons. Additionally, marked neuronal loss was observed in the neostriatum, globus pallidum, subthalamic nucleus, and substantia nigra. p\u2010TDP\u201043\u2010immunoreactive inclusions were frequently found in these areas. Neuronal loss and TDP\u201043 pathology in the motor, striatonigral, and pallidoluysian systems were predominant on the right side. Moreover, p\u2010TDP\u201043\u2010immunoreactive cat's\u2010eye\u2010shaped neuronal nuclear inclusions (NNIs) were observed in the affected lesions. NNIs in the striatonigral system were also positive for valosin\u2010containing protein (VCP). We diagnosed the patient as having ALS with striatonigral and pallidoluysian degeneration. Patients with ALS rarely experience pallido\u2010nigro\u2010luysian degeneration. To our best knowledge, only one case of ALS combined with striatonigral and pallidoluysian degeneration has been reported. Neuronal loss in the striatonigral and\/or pallidoluysian systems has also been reported in patients with ALS with multisystem degeneration accompanied by long\u2010term use of an artificial respirator. Based on these findings, a possibility of an extremely rare subtype of ALS demonstrating selective loss of neurons in the striatonigral and pallidoluysian systems exists; another possibility is that this type could be an early stage or <jats:italic>forme fruste<\/jats:italic> of ALS with multisystem degeneration. Although VCP\u2010positive cat's\u2010eye\u2010shaped NNIs have been reported in spinocerebellar ataxia type\u20102 cases, our case report presents VCP\u2010positive NNIs in a patient with ALS for the first time.<\/jats:p>","DOI":"10.1111\/neup.12843","type":"journal-article","created":{"date-parts":[[2022,7,1]],"date-time":"2022-07-01T06:25:06Z","timestamp":1656656706000},"page":"329-338","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An autopsy case of amyotrophic lateral sclerosis with striatonigral and pallidoluysian degeneration and cat's\u2010eye\u2010shaped neuronal nuclear inclusions"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6072-3954","authenticated-orcid":false,"given":"Tomoya","family":"Kon","sequence":"first","affiliation":[{"name":"Department of Neurology, Institute of Brain Science Hirosaki University Graduate School of Medicine  Hirosaki 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