title: Hereditary encephalomyelopathy and polyneuropathy in an Alaskan husky.
abstract: An Alaskan husky puppy was examined for a neurologic disease which began at six weeks of age with generalised paresis that progressed resulting in recumbency by 18 weeks. Thoracic limbs primarily exhibited lower motor neuron signs that included distal muscle atrophy and persistent elbow and carpal flexion that resisted manual extension. Pelvic limb signs primarily exhibited upper motor neuron and general proprioceptive deficits, but also included lower motor neuron signs. Abnormal vocalisation suggested a laryngeal paresis. Histopathologic lesions included a diffuse axonopathy and secondary demyelination in the nerves of the limbs and larynx and a similar bilaterally symmetrical degeneration in the spinal cord white matter suggestive of a dying back axonopathy. In addition, a degenerative process was present in nuclei in the brain stem and cerebellum. Recognition of this disease through clinical and pathologic examination in other related Alaskan Huskies suggested an autosomal recessive inherited disorder.
authors: Department of Clinical Sciences, Cornell University, College of Veterinary Medicine, Ithaca, NY 14853-6401, USA.
authors: J J
authors: JJ
authors: Wakshlag
authors: A
authors: A
authors: de Lahunta
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002490:Central Nervous System / Q000473:pathology* / Q000503:physiopathology; D004283:Dog Diseases / Q000151:congenital* / Q000235:genetics; D004285:Dogs; D017809:Fatal Outcome; D005808:Genes, Recessive; D020022:Genetic Predisposition to Disease; D007178:Inbreeding; D008297:Male; D011115:Polyneuropathies / Q000151:congenital / Q000235:genetics / Q000662:veterinary*; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000151:congenital / Q000235:genetics / Q000662:veterinary*