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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...

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Autores principales: Tamura, Shinji, Tsuboi, Masaya, Ueoka, Naotami, Doi, Shoko, Tamura, Yumiko, Uchida, Kazuyuki, Yabuki, Akira, Yamato, Osamu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
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.
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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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