Kararizou Evangelia, Manta Panajota, Kalfakis Nikolaos, Gkiatas Konstantinos, Vassilopoulos Dimitrios
Myopathology Laboratory, Department of Neurology, Athens National University, Eginition Hospital, 74 Vas. Sofias Ave., GR 11528 Athens, Greece.
Acta Histochem. 2006;108(4):265-9. doi: 10.1016/j.acthis.2006.03.020. Epub 2006 May 26.
A study was made of the morphological and morphometrical features of muscle spindles in biopsies of patients with Werdnig-Hoffmann disease (infantile spinal muscular atrophy type I) to investigate the possible involvement of the muscle spindles in the pathological processes of the disease. A total of 57 muscle spindles from 26 cases were studied. The parameters determined were: the diameter and area of spindles, the number, diameter and area of intrafusal fibers, the number and area of nuclei. In addition, the ratio of the area of the intrafusal fibers to the area of nuclei and the ratio of the area of the spindle to the area of the intrafusal fibers were calculated. Statistical evaluation of the data showed significant differences regarding the area of the muscle spindle, the diameter of the intrafusal fibers and the mean area of nuclei of the intrafusal fibers, which were all smaller in patients than in controls (p=0.03, 0.01 and 0.02, respectively), while the thickness of the capsule was greater in patients than in controls (p=0.01). Our results indicate that the muscle spindle participates in the pathological processes of Werdnig-Hoffmann disease.
为研究肌梭是否参与韦尔尼克 - 霍夫曼病（婴儿型脊髓性肌萎缩症I型）的病理过程，对该病患者活检组织中的肌梭形态和形态测量特征进行了研究。共研究了26例患者的57个肌梭。测定的参数包括：梭的直径和面积、梭内纤维的数量、直径和面积、核的数量和面积。此外，还计算了梭内纤维面积与核面积的比值以及梭面积与梭内纤维面积的比值。数据的统计评估显示，患者的肌梭面积、梭内纤维直径和梭内纤维核的平均面积存在显著差异，这些参数均低于对照组（p分别为0.03、0.01和0.02），而患者的囊厚度大于对照组（p = 0.01）。我们的结果表明，肌梭参与了韦尔尼克 - 霍夫曼病的病理过程。
