Small Animal Medicine and Clinical Biology, Faculty of Veterinary Medicine, Ghent University, Salisburylaan 133, 9820 Merelbeke, Belgium.
Vet J. 2011 Sep;189(3):284-8. doi: 10.1016/j.tvjl.2010.07.011. Epub 2010 Aug 17.
The clinical and clinicopathological characteristics, treatment and outcome of vermicular muscle contractions (myokymia) and generalized muscle stiffness (neuromyotonia) in 37 Jack Russell terriers were evaluated retrospectively. Thirty dogs were affected by both disorders, whereas seven were presented with myokymia and never developed neuromyotonia. Clinical signs started at the mean age of 8 months. Except for signs of myokymia and neuromyotonia, clinical and neurological examination was normal in all dogs. Thirty dogs demonstrated typical signs of hereditary ataxia. Changes in serum chemistry included increased creatine kinase, aspartate aminotransferase and alanine aminotransferase concentrations. Electromyographic abnormalities, especially in muscles showing macroscopically visible myokymia, consisted of semirhythmic bursts of doublet, triplet, or multiplet discharges of a single motor unit. The amplitudes varied between 80 μV and 1 mV and occurred with an interburst frequency between 10 and 40 Hz and an intraburst frequency between 150 and 280 Hz. Most dogs were treated with a sodium channel blocker with variable results. Seven dogs died (most likely because of hyperthermia) or were euthanased during a neuromyotonic attack; 15 dogs were euthanased due to worsening of clinical signs, or lack of or no long-lasting effect of medication, and three were euthanased for unknown or unrelated reasons. Nine dogs were lost to follow-up and three were still alive 5-10.5 years after the start of clinical signs. In conclusion, young Jack Russell terriers with myokymia and neuromyotonia should undergo a complete blood and electrophysiological examination. Long-term prognosis is not favourable.
回顾性评估了 37 只杰克罗素梗犬的蠕动性肌肉收缩(肌纤维震颤)和全身肌肉僵硬(肌强直)的临床和临床病理特征、治疗方法和结果。30 只狗同时患有这两种疾病,而 7 只狗只患有肌纤维震颤,从未出现过肌强直。临床症状平均在 8 个月大时开始出现。除了肌纤维震颤和肌强直的迹象外,所有狗的临床和神经检查均正常。30 只狗表现出典型的遗传性共济失调迹象。血清化学变化包括肌酸激酶、天门冬氨酸转氨酶和丙氨酸转氨酶浓度升高。肌电图异常,特别是在肉眼可见肌纤维震颤的肌肉中,表现为单个运动单位的半节律性爆发双、三或多脉冲放电。振幅在 80 μV 至 1 mV 之间,爆发间隔频率在 10 至 40 Hz 之间,爆发内频率在 150 至 280 Hz 之间。大多数狗用钠离子通道阻滞剂治疗,效果不一。7 只狗在肌强直发作时死亡(很可能是因为体温过高)或被安乐死;15 只狗因临床症状恶化、药物无效或无长期效果而被安乐死,3 只狗因未知或无关原因被安乐死。9 只狗失访,3 只狗在临床症状开始后 5-10.5 年内仍存活。总之,患有肌纤维震颤和肌强直的年轻杰克罗素梗犬应进行全面的血液和电生理检查。长期预后不佳。