Nemaline rods in canine myopathies:: 4 case reports and literature review
Nemaline rods in canine myopathies:: 4 case reports and literature review
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DOI：
10.1111/j.1939-1676.1998.tb02145.x
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发表时间：
1998-11-01
影响因子：
2.6
通讯作者：
Shelton, GD
中科院分区：
文献类型：
--
作者：
Delauche, AJ;Cuddon, PA;Shelton, GD
线状体杆状肌病 (NM) 的诊断基于新鲜冷冻肌肉活检标本中存在大量特征性杆状体。人类先天性 NM 的三种形式已被描述，并且杆状细胞也被发现出现在各种其他情况下。 1986 年，一个猫科动物也出现了类似的肌病。在本报告中，我们描述了一只 10 个月大的边境牧羊犬的先天性 NM 病例、一只 11 岁的 Schipperke 的成年发病 NM 病例，以及两例与甲状腺功能减退和库欣综合征相关的成年犬的获得性线状杆状肌病。常见的临床特征包括运动不耐受、肌电图异常以及近端阑尾肢体肌肉的新鲜、冷冻和戊二醛固定活检中存在线虫样杆。采用改良的 Gomori 三色技术对肌肉活检标本的低温切片进行染色，发现许多杆体通过组织化学三磷酸腺苷酶反应定位于 1 型纤维。电子显微镜也证实了 2 个病例中存在杆状堆积，且 Z 线局部增大和流动。年轻边境牧羊犬中 NM 的记录以及 Schipperke 中成年发病形式的记录提醒临床医生这些品种中存在这种疾病。
The diagnosis of nemaline rod myopathy (NM) is based on the presence of numerous pathognomonic rods within a fresh frozen muscle biopsy specimen. Three forms of congenital NM have been described in humans, and rods have been found to occur in various other conditions. A similar myopathy was described in 1986 in a family of cats. In this report, we describe a case of congenital NM in a 10-month-old Border Collie, an adult-onset NM in an 11-year-old Schipperke, and 2 acquired myopathies with nemaline rods in adult dogs associated with hypothyroidism and Cushing's syndrome. Common clinical features included exercise intolerance, abnormal electromyography, and the presence of nemaline rods in fresh, frozen, and glutaraldehyde-fixed biopsies from proximal appendicular limb muscles. Staining of cryostat sections of muscle biopsy specimens by the modified Gomori trichrome technique disclosed numerous rod bodies that were localized to type 1 fibers by the histochemical adenosine triphosphatase reaction. Accumulation of rods also was demonstrated by electron microscopy in 2 of the cases with localized enlargement and streaming of Z lines. Documentation of NM in a young Border Collie and the adult-onset form in the Schipperke alerts clinicians to the existence of this disorder in these breeds.