Clinical signs and neuropathologic abnormalities in working Australian Kelpies with globoid cell leukodystrophy (Krabbe disease)

Abstract

Objective: To characterize the clinical signs of globoid cell leukodystrophy (GLD) in Australian Kelpies from a working line (AWKs) and determine whether an association existed between these signs and degrees of demyelination and inflammatory responses in affected brains. Design: Case-control study. Animals: 4 AWKs with GLD (cases) and 7 unaffected young adult dogs of mixed breeding (controls). Procedures: Clinical records were reviewed for information on signalment, and samples of neurologic tissues underwent histological processing, immunohistochemical staining, and image analysis. Findings were compared between case and control dogs. Results: The 4 affected AWKs had progressive ataxia, tr..