Demyelinating polyneuropathy with focally folded myelin sheaths in a family of Miniature Schnauzer dogs

• Published in: Journal of the neurological sciences 2008-12, Vol.275 (1), p.100-105
• Main Authors: Vanhaesebrouck, An E, Couturier, Jérôme, Cauzinille, Laurent, Mizisin, Andrew P, Shelton, G. Diane, Granger, Nicolas
• Format: Article
• Language: English
• Subjects: Animal model; Animals; Biological and medical sciences; Demyelinating Diseases - genetics; Demyelinating Diseases - pathology; Demyelinating Diseases - physiopathology; Demyelinating Diseases - veterinary; Demyelination; Dog; Dog Diseases; Dogs - genetics; Male; Medical sciences; Microscopy, Electron, Transmission; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Myelin Sheath - genetics; Myelin Sheath - pathology; Myelin Sheath - ultrastructure; Neural Conduction - genetics; Neural Conduction - physiology; Neurology; Peripheral Nerves - pathology; Peripheral Nerves - physiopathology; Peripheral Nerves - ultrastructure; Peripheral neuropathy; Tomacula
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2008.07.031

Abstract A spontaneous demyelinating polyneuropathy in two young Miniature Schnauzer dogs was characterized clinically, electrophysiologically and histopathologically. Both dogs were related and a third dog, belonging to the same family, had similar clinical signs. On presentation, clinical signs were restricted to respiratory dysfunction. Electrophysiological tests showed a dramatic decrease in both motor and sensory nerve conduction velocities. Microscopic examination of peripheral nerve biopsies (light and electron microscopy, teased nerve fibers), showed that this neuropathy was characterized by segmental demyelination and focally folded myelin sheaths. Various clinical syndromes associated with tomacula or focal thickening of the myelin sheath of the peripheral nerves have been described in humans and shown to be caused by gene mutations affecting the myelin proteins, such as the hereditary neuropathy with liability to pressure palsies or the demyelinating forms of Charcot–Marie–Tooth disease. In animals, a tomaculous neuropathy has been reported in cattle and chickens but not in carnivores. Here we report a demyelinating peripheral neuropathy with tomacula in two Miniature Schnauzer dogs.