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Research article - Peer-reviewed, 2016

Degenerative Encephalopathy in Nova Scotia Duck Tolling Retrievers Presenting with a Rapid Eye Movement Sleep Behavior Disorder

Barker, E. N.; Dawson, L. J.; Rose, J. H.; Van Meervenne, S.; Frykman, O.; Rohdin, C.; Leijon, A.; Soerensen, K. E.; Jarnegren, J.; Johnson, G. C.; O'Brien, D. P.; Granger, N.

Abstract

BackgroundNeurodegenerative diseases are a heterogeneous group of disorders characterized by loss of neurons and are commonly associated with a genetic mutation.Hypothesis/ObjectivesTo characterize the clinical and histopathological features of a novel degenerative neurological disease affecting the brain of young adult Nova Scotia Duck Tolling Retrievers (NSDTRs).AnimalsNine, young adult, related NSDTRs were evaluated for neurological dysfunction and rapid eye movement sleep behavior disorder.MethodsCase series review.ResultsClinical signs of neurological dysfunction began between 2 months and 5 years of age and were progressive in nature. They were characterized by episodes of marked movements during sleep, increased anxiety, noise phobia, and gait abnormalities. Magnetic resonance imaging documented symmetrical, progressively increasing, T2-weighted image intensity, predominantly within the caudate nuclei, consistent with necrosis secondary to gray matter degeneration. Abnormalities were not detected on clinicopathological analysis of blood and cerebrospinal fluid, infectious disease screening or urine metabolite screening in most cases. Postmortem examination of brain tissue identified symmetrical malacia of the caudate nuclei and axonal dystrophy within the brainstem and spinal cord. Genealogical analysis supports an autosomal recessive mode of inheritance.Conclusions and Clinical ImportanceA degenerative encephalopathy was identified in young adult NSDTRs consistent with a hereditary disease. The prognosis is guarded due to the progressive nature of the disease, which is minimally responsive to empirical treatment.

Keywords

Axonopathy; Basal nuclei; Behavioral changes; Familial

Published in

Journal of Veterinary Internal Medicine
2016, Volume: 30, number: 5, pages: 1681-1689
Publisher: WILEY-BLACKWELL

      SLU Authors

    • UKÄ Subject classification

      Pathobiology

      Publication identifier

      DOI: https://doi.org/10.1111/jvim.14575

      Permanent link to this page (URI)

      https://res.slu.se/id/publ/82873