Department of Neurology and Neuroimaging Laboratory, Faculty of Medicine, University of Campinas-UNICAMP, Rua Tessália Vieira de Camargo, 126. Cidade Universitaria "Zeferino Vaz", Campinas, São Paulo, Brazil, 13083-887.
Cerebellum. 2013 Feb;12(1):43-7. doi: 10.1007/s12311-012-0390-6.
Although Friedreich's ataxia is characterized by spinal cord atrophy, it remains to be investigated the possible correlation of such atrophy with clinical disability and genetic parameters. Thirty-three patients with Friedreich's ataxia and 30 healthy controls underwent MRI on a 3 T scanner. We used T1-weighted 3D images to estimate spinal cord area and eccentricity at C2/C3 level based on a semi-automatic image segmentation protocol. We quantified severity of ataxia with the Friedreich ataxia rating scale (FARS). Mean cord area in Friedreich's ataxia was smaller than in controls (38 vs 67.9 mm(2), p < 0.001). In contrast, mean cord eccentricity was significantly higher in Friedreich's ataxia when compared to the controls (0.82 vs 0.76, p < 0.001). There was a significant correlation between cord areas and the FARS scores (r = -0.53, p = 0.002). Cord damage in Friedreich's ataxia results in atrophy combined with flattening. Cord area is associated to clinical disability and might be useful as a biomarker in the disease.
虽然弗里德里希共济失调的特征是脊髓萎缩，但仍需研究这种萎缩与临床残疾和遗传参数的可能相关性。33 名弗里德里希共济失调患者和 30 名健康对照者在 3T 扫描仪上进行了 MRI 检查。我们使用 T1 加权 3D 图像，根据半自动图像分割协议，在 C2/C3 水平估计脊髓面积和偏心率。我们使用弗里德里希共济失调评定量表 (FARS) 量化共济失调的严重程度。弗里德里希共济失调患者的脊髓平均面积小于对照组 (38 对 67.9mm2，p<0.001)。相比之下，弗里德里希共济失调患者的脊髓平均偏心率明显高于对照组 (0.82 对 0.76，p<0.001)。脊髓面积与 FARS 评分之间存在显著相关性 (r=-0.53，p=0.002)。弗里德里希共济失调中的脊髓损伤导致萎缩伴变平。脊髓面积与临床残疾相关，可能作为该疾病的生物标志物有用。
