Ono S, Inoue K, Mannen T, Mitake S, Shirai T, Kanda F, Jinnai K, Takahashi K
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Japan.
Acta Neuropathol. 1989;77(4):350-6. doi: 10.1007/BF00687369.
Intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies have been studied in four patients with myotonic dystrophy (MyD), eight patients with other neurological diseases (control A), and eight patients without neurological diseases (control B). The percentages of the affected cells were calculated by dividing the number of neurons including intracytoplasmic inclusion bodies of the thalamus and the substantia nigra, and Marinesco bodies, by the total cell count in these respective regions. Statistical analyses were performed with regard to the frequency of these bodies by using Student's t test. There was a significantly higher incidence of intracytoplasmic inclusion bodies of the thalamus (13.2% versus 0.7%, P less than 0.001) and the substantia nigra (20.4% versus 2.7%, P less than 0.001), and Marinesco bodies (37.4% versus 4.1%, P less than 0.001) in patients with MyD than in controls A and B. From our observations, it is suggested that the presence with a high frequency, in combination, of these bodies is not an incidental finding but may have an intimate and important relationship with the pathogenesis of MyD, and may be a conspicuous and diagnostically important feature of MyD.
对4例强直性肌营养不良（MyD）患者、8例患有其他神经系统疾病的患者（对照A）和8例无神经系统疾病的患者（对照B）的丘脑和黑质的胞质内包涵体以及马里内斯科小体进行了研究。通过将包含丘脑和黑质的胞质内包涵体以及马里内斯科小体的神经元数量除以这些相应区域的细胞总数来计算受影响细胞的百分比。通过使用学生t检验对这些小体的频率进行统计分析。与对照A和对照B相比，MyD患者丘脑的胞质内包涵体（13.2%对0.7%，P<0.001）、黑质的胞质内包涵体（20.4%对2.7%，P<0.001）以及马里内斯科小体（37.4%对4.1%，P<0.001）的发生率显著更高。根据我们的观察结果，提示这些小体同时高频出现并非偶然发现，而是可能与MyD的发病机制存在密切且重要的关系，并且可能是MyD显著且具有诊断意义的特征。
