A hypomyelinating leukodystrophy in German Shepherd dogs

Abstract Background Shaking puppy syndrome is commonly attributed to abnormal myelination of the central nervous system. Hypothesis/Objectives To report the long‐term clinical course and the imaging characteristics of hypomyelinating leukodystrophy in German Shepherd dogs. Animals and Methods Three...

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Autores principales:	Pia R. Quitt, Andreas Brühschwein, Kaspar Matiasek, Franziska Wielaender, Veera Karkamo, Marjo K. Hytönen, Andrea Meyer‐Lindenberg, Berett Dengler, Tosso Leeb, Hannes Lohi, Andrea Fischer
Formato:	article
Lenguaje:	EN
Publicado:	Wiley 2021
Materias:	animal model brain maturation development dysmyelination genetic hypomyelination Veterinary medicine SF600-1100
Acceso en línea:	https://doaj.org/article/c4992caca70d476589f46380694667a0
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spelling	oai:doaj.org-article:c4992caca70d476589f46380694667a02021-11-30T17:01:04ZA hypomyelinating leukodystrophy in German Shepherd dogs1939-16760891-664010.1111/jvim.16085https://doaj.org/article/c4992caca70d476589f46380694667a02021-05-01T00:00:00Zhttps://doi.org/10.1111/jvim.16085https://doaj.org/toc/0891-6640https://doaj.org/toc/1939-1676Abstract Background Shaking puppy syndrome is commonly attributed to abnormal myelination of the central nervous system. Hypothesis/Objectives To report the long‐term clinical course and the imaging characteristics of hypomyelinating leukodystrophy in German Shepherd dogs. Animals and Methods Three related litters with 11 affected dogs. Results The 11 affected dogs experienced coarse, side‐to‐side tremors of the head and trunk, which interfered with normal goal‐oriented movements and disappeared at rest. Signs were noticed shortly after birth. Nine dogs were euthanized, 3 dogs underwent pathological examination, and 2 littermates were raised by their breeder. Tremors improved gradually until 6 to 7 months of age. Adult dogs walked with severe residual pelvic limb ataxia. One dog developed epilepsy with tonic‐clonic seizures at 15 months of age. Conventional magnetic resonance imaging (MRI) disclosed homogenous hyperintense signal of the entire subcortical white matter in 3 affected 7‐week‐old dogs and a hypointense signal in a presumably unaffected littermate. Subcortical white matter appeared isointense to gray matter at 15 and 27 weeks of age on repeated MRI. Abnormal white matter signal with failure to display normal gray‐white matter contrast persisted into adulthood. Cerebellar arbor vitae was not visible at any time point. Clinical signs, MRI findings, and pathological examinations were indicative of a hypomyelinating leukodystrophy. All parents of the affected litters shared a common ancestor and relatedness of the puppies suggested an autosomal recessive mode of inheritance. Conclusion We describe a novel hypomyelinating leukodystrophy in German Shepherd dogs with a suspected inherited origin.Pia R. QuittAndreas BrühschweinKaspar MatiasekFranziska WielaenderVeera KarkamoMarjo K. HytönenAndrea Meyer‐LindenbergBerett DenglerTosso LeebHannes LohiAndrea FischerWileyarticleanimal modelbrain maturationdevelopmentdysmyelinationgenetichypomyelinationVeterinary medicineSF600-1100ENJournal of Veterinary Internal Medicine, Vol 35, Iss 3, Pp 1455-1465 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	animal model brain maturation development dysmyelination genetic hypomyelination Veterinary medicine SF600-1100
spellingShingle	animal model brain maturation development dysmyelination genetic hypomyelination Veterinary medicine SF600-1100 Pia R. Quitt Andreas Brühschwein Kaspar Matiasek Franziska Wielaender Veera Karkamo Marjo K. Hytönen Andrea Meyer‐Lindenberg Berett Dengler Tosso Leeb Hannes Lohi Andrea Fischer A hypomyelinating leukodystrophy in German Shepherd dogs
description	Abstract Background Shaking puppy syndrome is commonly attributed to abnormal myelination of the central nervous system. Hypothesis/Objectives To report the long‐term clinical course and the imaging characteristics of hypomyelinating leukodystrophy in German Shepherd dogs. Animals and Methods Three related litters with 11 affected dogs. Results The 11 affected dogs experienced coarse, side‐to‐side tremors of the head and trunk, which interfered with normal goal‐oriented movements and disappeared at rest. Signs were noticed shortly after birth. Nine dogs were euthanized, 3 dogs underwent pathological examination, and 2 littermates were raised by their breeder. Tremors improved gradually until 6 to 7 months of age. Adult dogs walked with severe residual pelvic limb ataxia. One dog developed epilepsy with tonic‐clonic seizures at 15 months of age. Conventional magnetic resonance imaging (MRI) disclosed homogenous hyperintense signal of the entire subcortical white matter in 3 affected 7‐week‐old dogs and a hypointense signal in a presumably unaffected littermate. Subcortical white matter appeared isointense to gray matter at 15 and 27 weeks of age on repeated MRI. Abnormal white matter signal with failure to display normal gray‐white matter contrast persisted into adulthood. Cerebellar arbor vitae was not visible at any time point. Clinical signs, MRI findings, and pathological examinations were indicative of a hypomyelinating leukodystrophy. All parents of the affected litters shared a common ancestor and relatedness of the puppies suggested an autosomal recessive mode of inheritance. Conclusion We describe a novel hypomyelinating leukodystrophy in German Shepherd dogs with a suspected inherited origin.
format	article
author	Pia R. Quitt Andreas Brühschwein Kaspar Matiasek Franziska Wielaender Veera Karkamo Marjo K. Hytönen Andrea Meyer‐Lindenberg Berett Dengler Tosso Leeb Hannes Lohi Andrea Fischer
author_facet	Pia R. Quitt Andreas Brühschwein Kaspar Matiasek Franziska Wielaender Veera Karkamo Marjo K. Hytönen Andrea Meyer‐Lindenberg Berett Dengler Tosso Leeb Hannes Lohi Andrea Fischer
author_sort	Pia R. Quitt
title	A hypomyelinating leukodystrophy in German Shepherd dogs
title_short	A hypomyelinating leukodystrophy in German Shepherd dogs
title_full	A hypomyelinating leukodystrophy in German Shepherd dogs
title_fullStr	A hypomyelinating leukodystrophy in German Shepherd dogs
title_full_unstemmed	A hypomyelinating leukodystrophy in German Shepherd dogs
title_sort	hypomyelinating leukodystrophy in german shepherd dogs
publisher	Wiley
publishDate	2021
url	https://doaj.org/article/c4992caca70d476589f46380694667a0
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A hypomyelinating leukodystrophy in German Shepherd dogs

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