Department of Small Animal Clinical Sciences, Western College of Veterinary Medicine, University of Saskatchewan, 52 Campus Drive, Saskatoon, Saskatchewan S7N 5B4 (Leger, Bater, Paulin, Linn, Taylor); Department of Pathology, School of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, California 92093, USA (Shelton).
Can Vet J. 2024 Oct;65(10):1034-1040.
An 8-year-old neutered male Bengal cat was referred because of a 1-year history of progressive and relapsing generalized muscle weakness and muscle atrophy. Before referral, the cat was treated with immunosuppressive doses of oral prednisolone, intermittently for 6 mo, and had responded well when the immunosuppressive dose was maintained. Generalized paresis, diffuse muscle atrophy, and diminished spinal reflexes were present in all limbs, consistent with a generalized lower motor neuron disease. Histopathologic evaluation of muscle biopsies confirmed a pattern of muscle fiber atrophy consistent with chronic and severe denervation. No specific abnormalities were identified in the nerve biopsy or within intramuscular nerve branches. A presumptive antemortem diagnosis of an adult-onset motor neuron degeneration resembling amyotrophic lateral sclerosis (ALS) or spinal muscle atrophy was suspected. However, given the response to immunosuppressive doses of corticosteroids, an autoimmune process or other degenerative process could not be definitively excluded. Key clinical message: In this case, an adult cat had a chronic, progressive history of lower motor neuron weakness and absent spinal reflexes; biopsies revealed a neurogenic pattern of muscle fiber atrophy and histologically normal peripheral nerve and intramuscular nerve branches. Although reports of motor neuron disease are rare in the veterinary literature, this case report highlights the importance of muscle and nerve biopsies that lead to a presumptive diagnosis of motor neuron degeneration.
一只 8 岁已去势的雄性孟加拉猫因进行性和复发性全身性肌肉无力和肌肉萎缩的 1 年病史而被转介。转院前,这只猫曾接受过免疫抑制剂量的口服泼尼松龙治疗,间歇性治疗了 6 个月,当维持免疫抑制剂量时,它的病情得到了很好的缓解。所有四肢均出现广泛的轻瘫、弥漫性肌肉萎缩和脊髓反射减弱,符合全身性下运动神经元疾病。肌肉活检的组织病理学评估证实了与慢性和严重去神经支配一致的肌肉纤维萎缩模式。神经活检或肌内神经分支内均未发现特定异常。生前疑似诊断为类似于肌萎缩侧索硬化症 (ALS) 或脊髓性肌萎缩的成年起病运动神经元变性。然而,鉴于对免疫抑制剂量的皮质类固醇的反应,不能明确排除自身免疫过程或其他退行性过程。主要临床信息:在本例中,一只成年猫有慢性、进行性的下运动神经元无力和无脊髓反射病史;活检显示神经源性的肌肉纤维萎缩模式和组织学上正常的周围神经和肌内神经分支。尽管兽医文献中很少有运动神经元疾病的报道,但本病例报告强调了肌肉和神经活检的重要性,这有助于做出运动神经元变性的初步诊断。