Levin M I, Mozaffar T, Al-Lozi M T, Pestronk A
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Neurology. 1998 Mar;50(3):764-7. doi: 10.1212/wnl.50.3.764.
To characterize the clinical features and muscle pathology of paraneoplastic necrotizing myopathy.
Paraneoplastic syndromes involving many levels of the nervous system are well described, but there are only rare case reports of a necrotizing myopathy associated with cancer.
Case series.
We identified four patients with paraneoplastic necrotizing myopathy from muscle biopsies done at Washington University over a 10-year period. The patients (aged 38 to 76 years) presented with subacutely evolving, symmetric, proximal weakness. Tumor types included gastrointestinal adenocarcinoma (2 of 4), transitional cell carcinoma, prostatic carcinoma, and non-small cell lung carcinoma. Two patients died. Two others improved after treatment that included corticosteroids and tumor resection. Muscle pathology showed numerous necrotic fibers (8 to 100%) and intense alkaline phosphatase staining of the muscle connective tissue, but little inflammation.
Paraneoplastic necrotizing myopathy is characterized by a rapidly progressive, symmetric, predominantly proximal weakness that produces severe disability. Muscle pathology demonstrates prominent necrosis with alkaline phosphatase staining of connective tissue and little inflammation. Evaluation for cancer is indicated in patients with these clinical and pathologic findings.
描述副肿瘤性坏死性肌病的临床特征及肌肉病理学表现。
累及神经系统多个层面的副肿瘤综合征已有充分描述，但仅有罕见的与癌症相关的坏死性肌病病例报告。
病例系列研究。
我们从华盛顿大学10年间进行的肌肉活检中确定了4例副肿瘤性坏死性肌病患者。患者年龄在38至76岁之间，表现为亚急性进展的对称性近端肌无力。肿瘤类型包括胃肠道腺癌（4例中的2例）、移行细胞癌、前列腺癌和非小细胞肺癌。2例患者死亡。另外2例患者在接受包括皮质类固醇和肿瘤切除在内的治疗后病情改善。肌肉病理学显示大量坏死纤维（8%至100%）以及肌肉结缔组织碱性磷酸酶染色强烈，但炎症轻微。
副肿瘤性坏死性肌病的特征是快速进展的对称性、主要为近端肌无力，可导致严重残疾。肌肉病理学显示明显坏死，结缔组织碱性磷酸酶染色阳性且炎症轻微。对于有这些临床和病理表现的患者，应进行癌症评估。
