{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T14:45:32Z","timestamp":1774017932910,"version":"3.50.1"},"reference-count":36,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,10,12]],"date-time":"2021-10-12T00:00:00Z","timestamp":1633996800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Ministry of Education, Culture, Sports, Science, and Technology of Japan","award":["17H03927"],"award-info":[{"award-number":["17H03927"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Veterinary Sciences"],"abstract":"<jats:p>A two-year-and-eleven-month-old male Shikoku Inu was referred for evaluation of progressive gait abnormality that had begun three months prior. Neurological examination revealed ventral flexion of the neck, a wide-based stance in the hindlimb, wide excursions of the head from side to side, tremor in all four limbs, hypermetria in all four limbs, proprioceptive deficits in all four limbs, reduced patellar reflex in both hindlimbs, and postural vertical nystagmus. Later, behavioral and cognitive dysfunction, ataxia, and visual deficits slowly progressed. Magnetic resonance imaging revealed symmetrical progressive atrophy of the whole brain and cervical spinal cord. Bilateral retinal degeneration was observed, and both flush and flicker electroretinograms were bilaterally non-recordable at the age of five years and eight months, and the dog was euthanized. Histopathologically, faint-to-moderate deposition of light-brown pigments was frequently observed in the cytoplasm of neurons throughout the cerebrum, cerebellum, and nuclei of the brainstem. The pigments were positive for Luxol fast blue, periodic acid\u2013Schiff, and Sudan black B, and exhibited autofluorescence. Electron microscopic examination revealed the accumulation of membranous material deposition in the neuronal cytoplasm. Small foci of pigment-containing macrophages were frequently observed around the capillary vessels. Based on these clinical and pathological findings, the animal was diagnosed with adult-onset neuronal ceroid lipofuscinosis.<\/jats:p>","DOI":"10.3390\/vetsci8100227","type":"journal-article","created":{"date-parts":[[2021,10,13]],"date-time":"2021-10-13T21:48:39Z","timestamp":1634161719000},"page":"227","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu"],"prefix":"10.3390","volume":"8","author":[{"given":"Shinji","family":"Tamura","sequence":"first","affiliation":[{"name":"Tamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaya","family":"Tsuboi","sequence":"additional","affiliation":[{"name":"Laboratory of Veterinary Pathology, Graduate School of Agriculture and Life Science, University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naotami","family":"Ueoka","sequence":"additional","affiliation":[{"name":"Ueoka Animal Hospital,  2 Chome-18-11 Yoshijimahigashi, Naka-ku, Hiroshima 730-0822, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shoko","family":"Doi","sequence":"additional","affiliation":[{"name":"Tamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yumiko","family":"Tamura","sequence":"additional","affiliation":[{"name":"Tamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazuyuki","family":"Uchida","sequence":"additional","affiliation":[{"name":"Laboratory of Veterinary Pathology, Graduate School of Agriculture and Life Science, University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akira","family":"Yabuki","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Pathology, Joint Faculty of Veterinary Medicine, Kagoshima University, 1-21-24 Korimoto, Kagoshima 890-0065, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4430-5645","authenticated-orcid":false,"given":"Osamu","family":"Yamato","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical Pathology, Joint Faculty of Veterinary Medicine, Kagoshima University, 1-21-24 Korimoto, Kagoshima 890-0065, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.nbd.2017.08.017","article-title":"Canine neuronal ceroid lipofuscinoses: Promising models for preclinical testing of therapeutic interventions","volume":"108","author":"Katz","year":"2017","journal-title":"Neurobiol. 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