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Four rottweiler pups from two litters developed severe progressive signs of spinal ataxia, cerebellar ataxia and tetraparesis/paralysis. The signs started with ataxia of the pelvic limbs at seven to eight weeks of age and progressed to tetraparesis and paralysis within three to five weeks. Postmortem, a vacuolar neuronal disorder was found in the cerebellum, brainstem and the spinal cord, associated with Wallerian type degeneration in the brainstem, cerebellar peduncles and the medullary cord. Electron microscopy revealed empty membrane-bound vacuoles. Immunohistochemistry for PrPSc was negative. The disorder differs clinically and pathologically from other neurological disorders in the breed and a new (familial) neurological disorder in the rottweiler is suspected.
Vet Rec 1998 Mar 07
PMID:A neuronal vacuolar disorder in young rottweiler dogs. 972 82

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.
Vet Rec 2019 09 28
PMID:Spongiform leucoencephalomyelopathy in border terriers: clinical, electrophysiological and imaging features. 3134 36