Dalakas M C
NINCDS, Bethesda, MD 20892.
Neurology. 1988 Jan;38(1):99-104. doi: 10.1212/wnl.38.1.99.
Thirty-five muscle biopsies were performed on 27 patients with postpoliomyelitis progressive muscular atrophy (PPMA) (8 patients had two biopsies) and 5 asymptomatic postpolio patients in an attempt to define diagnostic criteria for the newly weakening muscles and to provide insights into the mechanism of the disease. PPMA muscles that had been left weak since the original illness showed a mixture of myopathy with new and old denervation including group atrophy and nuclear clumps. Fully recovered or originally spared PPMA muscles showed signs of reinnervation and recent denervation. Perivascular or interstitial inflammatory cells (predominantly lymphocytes unrelated to phagocytosis) were noted in 40% of all the PPMA biopsies. It is concluded that (1) postpolio muscle biopsies show a spectrum of morphologic changes that depend on whether the biopsied muscle was originally affected and had fully or partially recovered, (2) the newly weakened muscles show signs of recent denervation, and (3) the presence of reinnervation in the asymptomatic muscles and the patterns of recent and old denervation in PPMA muscles provide information regarding the degree of compensation of the surviving motor neurons and their apparent failure for further reinnervation via axonal sprouting.
对27例脊髓灰质炎后进行性肌肉萎缩（PPMA）患者（8例患者接受了两次活检）和5例无症状脊髓灰质炎后患者进行了35次肌肉活检，以确定新出现无力的肌肉的诊断标准，并深入了解该疾病的发病机制。自最初发病以来一直无力的PPMA肌肉表现出肌病与新、旧去神经改变的混合，包括群组性萎缩和核团。完全恢复或最初未受累的PPMA肌肉表现出再支配和近期去神经的迹象。在所有PPMA活检中，40%的样本发现血管周围或间质炎症细胞（主要是与吞噬作用无关的淋巴细胞）。得出以下结论：（1）脊髓灰质炎后肌肉活检显示一系列形态学变化，这些变化取决于活检的肌肉最初是否受累以及是否已完全或部分恢复；（2）新出现无力的肌肉显示近期去神经的迹象；（3）无症状肌肉中再支配的存在以及PPMA肌肉中近期和旧的去神经模式提供了有关存活运动神经元的代偿程度及其通过轴突发芽进行进一步再支配明显失败的信息。
