Peer-reviewed veterinary case report
Neuronal ceroid lipofuscinosis causing limb weakness in American
By Evans, Jason et al.·Published in Journal of veterinary internal medicine·2005·Veterinary Neurological Center, United States·View original on PubMed →
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Original publication title: A variant form of neuronal ceroid lipofuscinosis in American bulldogs.
- Species:
- dog
Plain-English summary
A group of American Bulldogs developed serious movement problems, including difficulty walking and coordination issues, starting between 1 and 3 years of age. As the disease progressed, some dogs became unable to walk at all. Blood tests ruled out other diseases, and sadly, four affected dogs were euthanized for humane reasons. Examination of their brains revealed abnormal accumulations of certain substances, confirming a rare inherited neurodegenerative condition known as neuronal ceroid lipofuscinosis (NCL). This is the first time this condition has been reported in American Bulldogs, indicating a unique variant of the disease.
People also search for: American Bulldog movement problems · dog neurodegenerative disease · NCL in dogs · why is my dog having trouble walking
Abstract
Neuronal ceroid lipofuscinoses (NCLs) are inherited neurodegenerative diseases characterized by accumulations of autofluorescent lipopigments within cells of the nervous system. Nine related American Bulldogs demonstrated dysmetria in all limbs and paraparesis. Nonambulatory tetraparesis was observed only in the later stages of the disease. The clinical signs developed between 1 and 3 years of age and were slowly progressive over several years, which is inconsistent with most reports in other breeds. Results from blood tests for 8 different lysosomal storage diseases on 4 affected and 6 related but unaffected dogs were negative. Four affected dogs were euthanized and histopathologic examinations showed diffuse accumulations of periodic acid-Schiff-positive inclusions in neurons and axonal spheroids along the entire neuraxis and retinae. The most severe lesions were in the brainstem proprioceptive nuclei and spinal cord, consistent with clinical signs. The storage material was autofluorescent and immunohistochemically positive for products of lipid peroxidation. Ultrastructural analysis was consistent with NCL. Pedigree analysis supports an autosomal-recessive mode of inheritance. NCL has not been previously reported in the American Bulldog and these findings suggest a variant form of the canine disease.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/15715047/