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Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens

Three 4-month-old kittens from the same litter were presented, two of which were exhibiting cerebellar signs. Euthanasia was requested. No cerebellum atrophy was disclosed on necropsy. General cerebellar anatomy was normal, including the thickness of the cortical layers, myelination, and neurons of...

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Autores principales: Résibois, Anne, Poncelet, Luc
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer-Verlag 2004
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086572/
https://www.ncbi.nlm.nih.gov/pubmed/15042386
http://dx.doi.org/10.1007/s00401-004-0846-y
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author Résibois, Anne
Poncelet, Luc
author_facet Résibois, Anne
Poncelet, Luc
author_sort Résibois, Anne
collection PubMed
description Three 4-month-old kittens from the same litter were presented, two of which were exhibiting cerebellar signs. Euthanasia was requested. No cerebellum atrophy was disclosed on necropsy. General cerebellar anatomy was normal, including the thickness of the cortical layers, myelination, and neurons of the deep cerebellar nuclei. In the ataxic cat vermis, Purkinje cells were lacking along broad parasagittal bands symmetrically disposed relative to the midline. Many Purkinje cells were also lacking in the hemispheres. The nodulus and the flocculus were normal. Surviving Purkinje cells had frequent main dendrite swellings visible with anti-calbindin and anti-microtubule associated protein. In affected regions, calbindin and phosphorylated neurofilaments immunesera stained numerous axonal torpedoes located in the granular layer and the folial white matter. They were also present in processes of the deep cerebellar nuclei and lateral vestibular nucleus. Loss of synaptic endings onto the neurons of these nuclei was evident. Hypertrophied Purkinje cell recurrent axons and enhanced retrograde synaptic endings were present in the granular layer. Bergmann glia was strongly labeled by anti-GFAP, but no abnormal supplementary fibers were seen. None of these alterations were present in the normal sister. However, abnormal vacuolation of the Purkinje cell main dendrites was evident in all three cats, but not in six unrelated control cats that were 3–6 months old. The inferior olive and pontine nuclei were also normal. The two ataxic cats had a primary Purkinje cell degeneration that shared many common features with the abnormal Purkinje cells of the nervous mutant mouse.
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spelling pubmed-70865722020-03-23 Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens Résibois, Anne Poncelet, Luc Acta Neuropathol Regular Paper Three 4-month-old kittens from the same litter were presented, two of which were exhibiting cerebellar signs. Euthanasia was requested. No cerebellum atrophy was disclosed on necropsy. General cerebellar anatomy was normal, including the thickness of the cortical layers, myelination, and neurons of the deep cerebellar nuclei. In the ataxic cat vermis, Purkinje cells were lacking along broad parasagittal bands symmetrically disposed relative to the midline. Many Purkinje cells were also lacking in the hemispheres. The nodulus and the flocculus were normal. Surviving Purkinje cells had frequent main dendrite swellings visible with anti-calbindin and anti-microtubule associated protein. In affected regions, calbindin and phosphorylated neurofilaments immunesera stained numerous axonal torpedoes located in the granular layer and the folial white matter. They were also present in processes of the deep cerebellar nuclei and lateral vestibular nucleus. Loss of synaptic endings onto the neurons of these nuclei was evident. Hypertrophied Purkinje cell recurrent axons and enhanced retrograde synaptic endings were present in the granular layer. Bergmann glia was strongly labeled by anti-GFAP, but no abnormal supplementary fibers were seen. None of these alterations were present in the normal sister. However, abnormal vacuolation of the Purkinje cell main dendrites was evident in all three cats, but not in six unrelated control cats that were 3–6 months old. The inferior olive and pontine nuclei were also normal. The two ataxic cats had a primary Purkinje cell degeneration that shared many common features with the abnormal Purkinje cells of the nervous mutant mouse. Springer-Verlag 2004-03-20 2004 /pmc/articles/PMC7086572/ /pubmed/15042386 http://dx.doi.org/10.1007/s00401-004-0846-y Text en © Springer-Verlag 2004 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Regular Paper
Résibois, Anne
Poncelet, Luc
Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title_full Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title_fullStr Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title_full_unstemmed Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title_short Purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
title_sort purkinje cell neuroaxonal dystrophy similar to nervous mutant mice phenotype in two sibling kittens
topic Regular Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086572/
https://www.ncbi.nlm.nih.gov/pubmed/15042386
http://dx.doi.org/10.1007/s00401-004-0846-y
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AT ponceletluc purkinjecellneuroaxonaldystrophysimilartonervousmutantmicephenotypeintwosiblingkittens