{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T14:34:15Z","timestamp":1756996455752},"reference-count":50,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,3,27]],"date-time":"2014-03-27T00:00:00Z","timestamp":1395878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:p>Basophilic inclusions (<jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content>) and neuronal intermediate filament inclusions (<jats:styled-content style=\"fixed-case\">NIFIs<\/jats:styled-content>) are key structures of basophilic inclusion body disease and neuronal intermediate filament inclusion disease (<jats:styled-content style=\"fixed-case\">NIFID<\/jats:styled-content>), respectively. <jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content> are sharply\u2010defined, oval or crescent neuronal intracytoplasmic inclusions that appear pale blue\u2010gray in color with <jats:styled-content style=\"fixed-case\">HE<\/jats:styled-content> staining and purple in color with <jats:styled-content style=\"fixed-case\">N<\/jats:styled-content>issl but are stained poorly with silver impregnation techniques. Immunohistochemically <jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content> are negative for tau, trans\u2010activation response <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> 43 (<jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043), \u03b1\u2010synuclein, neurofilament (<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>) and \u03b1\u2010internexin, positive for p62, and variably ubiquitinated. Noticeably, <jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content> are consistently fused in sarcoma (<jats:styled-content style=\"fixed-case\">FUS<\/jats:styled-content>) positive. <jats:styled-content style=\"fixed-case\">NIFIs<\/jats:styled-content> are by definition immuno\u2010positive for class <jats:styled-content style=\"fixed-case\">IV IFs<\/jats:styled-content> including three <jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content> triplet subunit proteins and \u03b1\u2010internexin but negative for tau, <jats:styled-content style=\"fixed-case\">TDP<\/jats:styled-content>\u201043, and \u03b1\u2010synuclein. In <jats:styled-content style=\"fixed-case\">NIFID<\/jats:styled-content> cases several types of inclusions have been identified. Among them, hyaline conglomerate\u2010like inclusions are the only type that meets the above immunohistochemical features of <jats:styled-content style=\"fixed-case\">NIFIs<\/jats:styled-content>. This type of inclusion appears upon <jats:styled-content style=\"fixed-case\">HE<\/jats:styled-content> staining as multilobulated, faintly eosinophilic or pale amphophilic spherical masses with a glassy appearance. These hyaline conglomerates appear strongly argyrophilic, and robustly and consistently immuno\u2010positive for <jats:styled-content style=\"fixed-case\">IFs<\/jats:styled-content>. In contrast, this type of inclusion shows no or only occasional dot\u2010like <jats:styled-content style=\"fixed-case\">FUS<\/jats:styled-content> immunoreactivity. Therefore, <jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">NIFIs<\/jats:styled-content> are distinct from each other in terms of morphological, tinctorial and immunohistochemical features. However, basophilic inclusion body disease (<jats:styled-content style=\"fixed-case\">BIBD<\/jats:styled-content>) and <jats:styled-content style=\"fixed-case\">NIFID<\/jats:styled-content> are difficult to differentiate clinically. Moreover, <jats:styled-content style=\"fixed-case\">P<\/jats:styled-content>ick body\u2010like inclusions, the predominant type of inclusions seen in <jats:styled-content style=\"fixed-case\">NIFID<\/jats:styled-content>, are considerably similar to the <jats:styled-content style=\"fixed-case\">BIs<\/jats:styled-content> of <jats:styled-content style=\"fixed-case\">BIBD<\/jats:styled-content> in that this type of inclusion is basophilic, poorly argyrophilic, negative for <jats:styled-content style=\"fixed-case\">IFs<\/jats:styled-content> and intensely immuno\u2010positive for <jats:styled-content style=\"fixed-case\">FUS<\/jats:styled-content>. As <jats:styled-content style=\"fixed-case\">BIBD<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">NIFID<\/jats:styled-content> share <jats:styled-content style=\"fixed-case\">FUS<\/jats:styled-content> accumulation as the most prominent molecular pathology, whether these two diseases are discrete entities or represent a pathological continuum remains a question to be answered.<\/jats:p>","DOI":"10.1111\/neup.12119","type":"journal-article","created":{"date-parts":[[2014,3,27]],"date-time":"2014-03-27T07:12:46Z","timestamp":1395904366000},"page":"589-595","source":"Crossref","is-referenced-by-count":7,"title":["Basophilic inclusions and neuronal intermediate filament inclusions in amyotrophic lateral sclerosis and frontotemporal lobar degeneration"],"prefix":"10.1111","volume":"34","author":[{"given":"Hidefumi","family":"Ito","sequence":"first","affiliation":[{"name":"Department of Neurology Wakayama Medical University  Wakayama 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