Clinical characterization of a familial degenerative myelopathy in Pembroke Welsh Corgi dogs

Clinical characterization of a familial degenerative myelopathy in Pembroke Welsh Corgi dogs
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DOI:
10.1892/07-059.1
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发表时间:
2007-11-01
影响因子:
2.6
通讯作者:
Williams, David A.
Williams, David A.
中科院分区:
农林科学2区
文献类型:
--
作者:
Coates, Joan R.;March, Philip A.;Williams, David A.

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背景资料:患有退行性脊髓病(DM)的成年犬具有进行性共济失调和骨盆肢体轻瘫,导致截瘫和安乐死。尽管最常见于德国牧羊犬,但其他品种的疾病患病率也很高。目的:我们的目的是彭布罗克威尔士柯基犬(PWC)家族性退行性脊髓病(FDM)的临床和组织病理学特征。动物:从初步诊断到安乐死,对21只PWC进行了前瞻性研究。方法:进行神经系统检查、血液检查、脑脊液(CSF)分析、电诊断试验和脊柱成像。测量CSF中8-异前列腺素F-2 α(8-异前列腺素)的浓度。常规组织化学用于神经病理学。结果:安乐死前临床体征的中位持续时间为19个月。安乐死时的中位年龄为13岁。所有犬均为不能行走的截瘫或截瘫,15只犬在人道处死时出现胸椎肢体无力。电诊断测试和脊柱成像与非压迫性脊髓病一致。正常和FDM受影响的狗之间的8-异前列烷浓度没有显着差异。脊髓轴突和髓鞘变性在侧索背侧部最为严重。系谱分析表明,一个家族性diseases.Conclusions和临床意义:FDM在PWC犬的临床进展是类似的,在其他品种中观察到的,但其特点是由一个较长的持续时间。脊髓病理主要表现为非炎性轴突变性。氧化应激损伤与8-异前列烷的生产是不参与FDM影响PWC狗的发病机制。怀疑有家族性疾病。
Background: Adult dogs with degenerative myelopathy (DM) have progressive ataxia and paresis of the pelvic limbs, leading to paraplegia and euthanasia. Although most commonly reported in German Shepherd dogs, high disease prevalence exists in other breeds.Objective: Our aim was the clinical and histopathologic characterization of familial degenerative myelopathy (FDM) in Pembroke Welsh Corgi (PWC) dogs.Animals: Twenty-one PWCs were prospectively studied from initial diagnosis until euthanasia.Methods: Neurologic examination, blood tests, cerebrospinal fluid (CSF) analysis, electrodiagnostic testing, and spinal imaging were performed. Concentrations of 8-iso-prostaglandin F-2 alpha (8-isoprostane) were measured in CSF. Routine histochemistry was used for neuropathology. Deoxyribonucleic acid and pedigrees were collected from I 10 dogs.Results: Median duration of clinical signs before euthanasia was 19 months. Median age at euthanasia was 13 years. All dogs were nonambulatory paraparetic or paraplegic, and 15 dogs had thoracic limb weakness at euthanasia. Electrodiagnostic testing and spinal imaging were consistent with noncompressive myelopathy. No significant difference was detected in 8-isoprostane concentrations between normal and FDM-affected dogs. Axonal and myelin degeneration of the spinal cord was most severe in the dorsal portion of the lateral funiculus. Pedigree analysis suggested a familial disease.Conclusions and Clinical Importance: Clinical progression of FDM in PWC dogs was similar to that observed in other breeds but characterized by a longer duration. Spinal cord pathology predominates as noninflammatory axonal degeneration. Oxidative stress injury associated with 8-isoprostane production is not involved in the pathogenesis of FDM-affected PWC dogs. A familial disease is suspected.