Vanhaesebrouck An E, Couturier Jérôme, Cauzinille Laurent, Mizisin Andrew P, Shelton G Diane, Granger Nicolas
National Veterinary School of Alfort, Maisons-Alfort, France.
J Neurol Sci. 2008 Dec 15;275(1-2):100-5. doi: 10.1016/j.jns.2008.07.031. Epub 2008 Sep 21.
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.
对两只患有自发性脱髓鞘性多发性神经病的幼年迷你雪纳瑞犬进行了临床、电生理和组织病理学特征分析。这两只犬有亲缘关系，同一家族的第三只犬也有类似临床症状。就诊时，临床症状局限于呼吸功能障碍。电生理测试显示运动和感觉神经传导速度均显著降低。对周围神经活检组织进行显微镜检查（光学显微镜和电子显微镜检查、 teased神经纤维检查）发现，这种神经病的特征是节段性脱髓鞘和局灶性髓鞘折叠。人类已描述了与周围神经髓鞘瘤或局灶性增厚相关的各种临床综合征，并证明是由影响髓鞘蛋白的基因突变引起的，如遗传性压力易感性麻痹或脱髓鞘型夏科-马里-图斯病。在动物中，牛和鸡曾报道过髓鞘瘤性神经病，但肉食动物中未见报道。本文报道了两只迷你雪纳瑞犬患有伴有髓鞘瘤的脱髓鞘性周围神经病。
