Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu
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...
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MDPI AG
2021-10-01
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| Series: | Veterinary Sciences |
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| Online Access: | https://www.mdpi.com/2306-7381/8/10/227 |
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| author | Shinji Tamura Masaya Tsuboi Naotami Ueoka Shoko Doi Yumiko Tamura Kazuyuki Uchida Akira Yabuki Osamu Yamato |
| author_facet | Shinji Tamura Masaya Tsuboi Naotami Ueoka Shoko Doi Yumiko Tamura Kazuyuki Uchida Akira Yabuki Osamu Yamato |
| author_sort | Shinji Tamura |
| collection | DOAJ |
| description | 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–Schiff, 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. |
| first_indexed | 2024-03-10T06:09:13Z |
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| institution | Directory Open Access Journal |
| issn | 2306-7381 |
| language | English |
| last_indexed | 2024-03-10T06:09:13Z |
| publishDate | 2021-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Veterinary Sciences |
| spelling | doaj.art-2e63753f1d714e2f81b6afabc4fd3d6c2023-11-22T20:17:21ZengMDPI AGVeterinary Sciences2306-73812021-10-0181022710.3390/vetsci8100227Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku InuShinji Tamura0Masaya Tsuboi1Naotami Ueoka2Shoko Doi3Yumiko Tamura4Kazuyuki Uchida5Akira Yabuki6Osamu Yamato7Tamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, JapanLaboratory of Veterinary Pathology, Graduate School of Agriculture and Life Science, University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, JapanUeoka Animal Hospital, 2 Chome-18-11 Yoshijimahigashi, Naka-ku, Hiroshima 730-0822, JapanTamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, JapanTamura Animal Clinic, 7-16 Yoshimien, Saeki-ku, Hiroshima 731-5132, JapanLaboratory of Veterinary Pathology, Graduate School of Agriculture and Life Science, University of Tokyo, 1-1-1 Yayoi, Bunkyo-ku, Tokyo 113-8657, JapanLaboratory of Clinical Pathology, Joint Faculty of Veterinary Medicine, Kagoshima University, 1-21-24 Korimoto, Kagoshima 890-0065, JapanLaboratory of Clinical Pathology, Joint Faculty of Veterinary Medicine, Kagoshima University, 1-21-24 Korimoto, Kagoshima 890-0065, JapanA 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–Schiff, 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.https://www.mdpi.com/2306-7381/8/10/227neuronal ceroid lipofuscinosisShikoku InudogMRIretina |
| spellingShingle | Shinji Tamura Masaya Tsuboi Naotami Ueoka Shoko Doi Yumiko Tamura Kazuyuki Uchida Akira Yabuki Osamu Yamato Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu Veterinary Sciences neuronal ceroid lipofuscinosis Shikoku Inu dog MRI retina |
| title | Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
| title_full | Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
| title_fullStr | Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
| title_full_unstemmed | Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
| title_short | Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
| title_sort | adult onset neuronal ceroid lipofuscinosis in a shikoku inu |
| topic | neuronal ceroid lipofuscinosis Shikoku Inu dog MRI retina |
| url | https://www.mdpi.com/2306-7381/8/10/227 |
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