Peer-reviewed veterinary case report
Trembling and brain white matter disease in German Shepherd puppies
By De Miguel, Ricardo et al.·Published in Journal of veterinary internal medicine·2024·AnaPath Services GmbH·View original on PubMed →
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Original publication title: Congenital spongiform leukodystrophy in 2 female littermate German shepherd puppies.
- Species:
- dog
Plain-English summary
Two 9-week-old female German Shepherd puppies were brought to the vet because they were shaking uncontrollably, especially when they moved. Tests revealed severe damage to their brain and spinal cord, which is a rare condition called congenital spongiform leukodystrophy. Unfortunately, this condition is likely due to a genetic issue that isn't linked to a known gene mutation. Sadly, there is no cure for this condition, and the prognosis for these puppies is poor.
People also search for: German Shepherd puppy shaking · congenital spongiform leukodystrophy in dogs · puppy neurological problems
Abstract
Two 9-week-old female littermate German Shepherd puppies showed severe high-frequency low-amplitude trembling that worsened with movement. The white matter (WM) of the central nervous system (CNS) showed bilateral diffuse severe spongiosis in the cerebellum, brainstem, spinal cord, and the neuropil of the oculomotor and red nuclei. The cortical corona radiata was less severely affected. Rare necrotic or apoptotic glia-like cells also were identified in the WM. Luxol fast blue staining disclosed severe diffuse myelin loss in the entire CNS; peripheral nerves were spared. Glial fibrillary acidic protein immunohistochemistry showed diffuse astrogliosis and astrocytosis in the WM. Genetic analyses of the littermates excluded the aspartoacylase (ASPA) gene as a candidate for this condition in dogs. In conclusion, this description of a rare congenital spongiform leukodystrophy in the German Shepherd breed, closely resembling to Canavan disease in humans, is likely caused by a genetic alteration unrelated to the ASPA gene.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/38544400/