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Peer-reviewed veterinary case report

Brain damage in Staffordshire bull terrier with l-2-hydroxyglutaric

By Scurrell, E et al.·Published in Journal of comparative pathology·2008·Department of Pathology and Infectious Diseases, United Kingdom·View original on PubMed

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Original publication title: Neuropathological findings in a Staffordshire bull terrier with l-2-hydroxyglutaric aciduria.

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Plain-English summary

An 11-month-old Staffordshire bull terrier was showing signs of a serious neurological condition, which led to a diagnosis of l-2-hydroxyglutaric aciduria, a rare genetic disorder. The dog's symptoms and MRI results raised concerns, and further testing confirmed the disease. Unfortunately, due to the severity of the condition and the brain damage observed, the dog was humanely euthanized. Post-mortem examinations revealed significant brain damage, particularly in areas responsible for movement and coordination.

People also search for: Staffordshire bull terrier neurological symptoms · l-2-hydroxyglutaric aciduria in dogs · dog brain disease signs

Abstract

l-2-Hydroxyglutaric aciduria (l-2-HGA) is a hereditary neurometabolic disorder reported in human beings and dogs. An 11-month-old Staffordshire bull terrier was suspected to have the disease, on the basis of clinical signs and magnetic resonance imaging findings. l-2-HGA was confirmed by urinary organic analysis and DNA testing and the dog was humanely destroyed. Post-mortem findings consisted only of microscopical lesions in the brain, characterized by marked spongiform changes and predominantly affecting the grey matter of the cerebral cortex, thalamus, cerebellum and brainstem. The spongiform changes were characterized by well-demarcated, clear vacuoles located at perineuronal and perivascular sites. Immunohistochemical and ultrastructural examination confirmed that the affected cells were astrocytes.

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Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/18295785/