Spongiform leucoencephalomyelopathy in border terriers: clinical, electrophysiological and imaging features

Gutierrez-Quintana, R. , McLaughlin, M. , Grau Roma, L., Hammond, G., Gray, A. and Lowrie, M. (2019) Spongiform leucoencephalomyelopathy in border terriers: clinical, electrophysiological and imaging features. Veterinary Record, 185, 375. (doi: 10.1136/vr.105240) (PMID:31346136)

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Abstract

A novel spongiform leucoencephalomyelopathy was reported in border terrier puppies in 2012 causing a shaking puppy phenotype, but no information regarding clinical progression, imaging or electrophysiological findings were available. The aim of the present study was to describe the clinical, electrophysiological and MRI features of this disease in seven dogs and compare them with human white matter disorders. All cases presented with cerebellar ataxia and severe generalised coarse body tremors, which started at three weeks of age. The three cases that were not euthanased showed slow but progressive improvement over several months. Brainstem auditory evoked response demonstrated a normal wave I, reduced amplitude of wave II and an absence of waves III-VII. MRI revealed bilateral and symmetrical T2-weighted hyperintensities affecting the brainstem and cerebellar white matter. Histological examination of the brain and spinal cord showed spongiform change affecting the white matter of the cerebellum, brainstem and spinal cord with decreased myelin content. In summary, this leucoencephalomyelopathy has a pathognomonic clinical presentation with defining MRI and electrophysiological characteristics, and it is the first report to describe a long-term improvement of this condition.

Item Type:Articles
Keywords:Dogs, leukodystrophy, magnetic resonance imaging (MRI), white matter disorder.
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:McLaughlin, Dr Mark and Gutierrez Quintana, Mr Rodrigo and Gray, Mr Alexander
Authors: Gutierrez-Quintana, R., McLaughlin, M., Grau Roma, L., Hammond, G., Gray, A., and Lowrie, M.
College/School:College of Medical Veterinary and Life Sciences > School of Veterinary Medicine
Journal Name:Veterinary Record
Publisher:BMJ Publishing Group
ISSN:0042-4900
ISSN (Online):2042-7670
Published Online:25 July 2019
Copyright Holders:Copyright © 2019 British Veterinary Association
First Published:First published in Veterinary Record 185:375
Publisher Policy:Reproduced in accordance with the copyright policy of the publisher

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