Pouget J, Pellissier J F, Azulay J P, Vallat J M, Visy J M, Serratrice G
Clinique des Maladies du Système Nerveux et de l'Appareil Locomoteur, Hôpital Universitaire de La Timone, Marseille.
Rev Neurol (Paris). 1992;148(3):200-6.
The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent. Muscle biopsy showed endo- and perimysial amyloid deposits but also inflammatory infiltrates. Inflammatory cells typing was studied by immunocytochemical methods and revealed a predominant T-helper cell infiltration. Free kappa light chains were present in serum and urine. Serum immunoglobulin levels were reduced. Bone marrow examination revealed mild plasmocytosis without abnormal cells. Immunofluorescence and immunoperoxidase techniques for identification of the type of amyloid fibrils showed positivity with antisera to kappa light chains. A 4-year follow-up revealed a progressive worsening of muscle weakness despite immunosuppressive treatment. No malignant plasmocytosis occurred. The unusual inflammatory muscle infiltration observed in this case may suggest an associated polymyositis.
报告了一例41岁患有淀粉样变肌病的女性病例。临床症状包括肩胛带肌肌无力、轻度肩胛肌萎缩和吞咽困难。与已发表的病例不同，未出现肌肉异常坚硬、假肥大和巨舌。肌肉活检显示肌内膜和肌束膜有淀粉样沉积物，但也有炎症浸润。通过免疫细胞化学方法研究炎症细胞类型，发现主要是辅助性T细胞浸润。血清和尿液中存在游离κ轻链。血清免疫球蛋白水平降低。骨髓检查显示轻度浆细胞增多，但无异常细胞。用于鉴定淀粉样纤维类型的免疫荧光和免疫过氧化物酶技术显示，抗κ轻链抗血清呈阳性。4年随访显示，尽管进行了免疫抑制治疗，肌无力仍逐渐加重。未发生恶性浆细胞增多症。该病例中观察到的不寻常的炎症性肌肉浸润可能提示合并了多发性肌炎。
