Peer-reviewed veterinary case report
Dog with brain and muscle disease linked to fatty acid oxidation
By Biegen, Vanessa R et al.·Published in Frontiers in veterinary science·2015·The Animal Medical Center, United States·View original on PubMed →
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Original publication title: Metabolic Encephalopathy and Lipid Storage Myopathy Associated with a Presumptive Mitochondrial Fatty Acid Oxidation Defect in a Dog.
- Species:
- dog
Plain-English summary
A 1-year-old spayed female Shih Tzu was brought to the vet because she was having episodes of unusual posture and confusion. After a neurological exam and MRI, the vet found serious brain lesions and signs of metabolic issues, including high lactate and low blood sugar. Unfortunately, treatment did not help, and the dog was euthanized. A post-mortem exam revealed severe damage in her brain and fat buildup in various organs, indicating a fatty acid oxidation defect, a rare metabolic disorder in dogs. This case highlights the importance of recognizing and diagnosing such conditions early for potential treatment options.
People also search for: dog confusion posture problems · Shih Tzu neurological issues · fatty acid oxidation disorder treatment in dogs
Abstract
A 1-year-old spayed female Shih Tzu presented for episodic abnormalities of posture and mentation. Neurological examination was consistent with a bilaterally symmetric multifocal encephalopathy. The dog had a waxing-and-waning hyperlactemia and hypoglycemia. Magnetic resonance imaging revealed bilaterally symmetric cavitated lesions of the caudate nuclei with less severe abnormalities in the cerebellar nuclei. Empirical therapy was unsuccessful, and the patient was euthanized. Post-mortem histopathology revealed bilaterally symmetric necrotic lesions of the caudate and cerebellar nuclei and multi-organ lipid accumulation, including a lipid storage myopathy. Malonic aciduria and ketonuria were found on urinary organic acid screen. Plasma acylcarnitine analysis suggested a fatty acid oxidation defect. Fatty acid oxidation disorders are inborn errors of metabolism documented in humans, but poorly described in dogs. Although neurological signs have been described in humans with this group of diseases, descriptions of advanced imaging, and histopathology are severely lacking. This report suggests that abnormalities of fatty acid metabolism may cause severe, bilateral gray matter necrosis, and lipid accumulation in multiple organs including the skeletal muscles, liver, and kidneys. Veterinarians should be aware that fatty acid oxidation disorders, although potentially fatal, may be treatable. A timely definitive diagnosis is essential in guiding therapy.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/26664991/