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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8538799/ https://www.ncbi.nlm.nih.gov/pubmed/34679057 http://dx.doi.org/10.3390/vetsci8100227 |
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author | Tamura, Shinji Tsuboi, Masaya Ueoka, Naotami Doi, Shoko Tamura, Yumiko Uchida, Kazuyuki Yabuki, Akira Yamato, Osamu |
author_facet | Tamura, Shinji Tsuboi, Masaya Ueoka, Naotami Doi, Shoko Tamura, Yumiko Uchida, Kazuyuki Yabuki, Akira Yamato, Osamu |
author_sort | Tamura, Shinji |
collection | PubMed |
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. |
format | Online Article Text |
id | pubmed-8538799 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-85387992021-10-24 Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu Tamura, Shinji Tsuboi, Masaya Ueoka, Naotami Doi, Shoko Tamura, Yumiko Uchida, Kazuyuki Yabuki, Akira Yamato, Osamu Vet Sci Case Report 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. MDPI 2021-10-12 /pmc/articles/PMC8538799/ /pubmed/34679057 http://dx.doi.org/10.3390/vetsci8100227 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Tamura, Shinji Tsuboi, Masaya Ueoka, Naotami Doi, Shoko Tamura, Yumiko Uchida, Kazuyuki Yabuki, Akira Yamato, Osamu Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu |
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 | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8538799/ https://www.ncbi.nlm.nih.gov/pubmed/34679057 http://dx.doi.org/10.3390/vetsci8100227 |
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