RT Journal Article
SR Electronic
T1 Spongiform leucoencephalomyelopathy in border terriers: clinical, electrophysiological and imaging features
JF Veterinary Record
JO Vet Rec
FD BMJ Publishing Group Limited
SP 375
OP 375
DO 10.1136/vr.105240
VO 185
IS 12
A1 Gutierrez-Quintana, Rodrigo
A1 McLaughlin, Mark
A1 Grau Roma, Llorenc
A1 Hammond, Gawain
A1 Gray, Alexander
A1 Lowrie, Mark
YR 2019
UL http://veterinaryrecord.bmj.com/content/185/12/375.abstract
AB 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.