Lukas Carsten, Hahn Horst K, Bellenberg Barbara, Hellwig Kerstin, Globas Christoph, Schimrigk Sebastian K, Köster Odo, Schöls Ludger
Dept. of Diagnostic and Interventional Radiology and Nuclear Medicine, St. Josef Hospital Ruhr University Bochum, Gudrunstr. 56, 44791, Bochum, Germany.
J Neurol. 2008 Aug;255(8):1244-9. doi: 10.1007/s00415-008-0907-6. Epub 2008 Jun 5.
To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6.
Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched healthy control subjects (n = 24,) corresponding to the two SCA groups, were studied. Images were post-processed by a semi-automated volumetry method combining a marker based segmentation and an automatic histogram method facilitating highly reliable quantification and morphometry of the upper cervical cord in vivo.
We found a significant reduction of normalized mean crosssectional area of the spinal cord in SCA3 (p < 0.0005), whereas in SCA6 patients normalized mean crosssectional area was in the normal range (p = 0.379). No correlation was found between spinal cord atrophy and disease duration as well as CAG repeat length in both subtypes. In SCA6 a negative dependency between clinical disability, as expressed by the International Cooperative Ataxia Rating Scale as a well established ataxia score, and the mean cross-sectional area was found (p = 0.02). A similar correlation was observed in SCA3 but did not reach statistical significance.
Our results quantify for the first time in vivo spinal cord atrophy as a non-cerebellar neurodegenerative process in SCA3. Our results suggest MR volumetry of the upper cervical cord as a marker of functional importance in SCA3 and SCA6.
量化脊髓小脑共济失调3型（SCA3）和6型（SCA6）患者的脊髓萎缩情况及其对临床残疾的影响。
通过高分辨率T1加权磁共振成像（MRI）评估14例SCA3患者和10例SCA6患者的上颈段脊髓萎缩情况。此外，研究了与两个SCA组年龄和性别匹配的两组健康对照者（每组24例）。图像采用基于标记分割和自动直方图方法相结合的半自动容积测量法进行后处理，有助于在体对上颈段脊髓进行高度可靠的定量分析和形态测量。
我们发现SCA3患者脊髓的标准化平均横截面积显著减小（p < 0.0005），而SCA6患者的标准化平均横截面积在正常范围内（p = 0.379）。在两个亚型中，均未发现脊髓萎缩与疾病持续时间以及CAG重复长度之间存在相关性。在SCA6中，以国际合作共济失调评定量表（一种成熟的共济失调评分）表示的临床残疾与平均横截面积之间存在负相关性（p = 0.02）。在SCA３中也观察到类似的相关性，但未达到统计学意义。
我们的结果首次在体量化了SCA3中作为非小脑神经退行性过程的脊髓萎缩情况。我们的结果表明，上颈段脊髓的磁共振容积测量可作为SCA3和SCA6中功能重要性的标志物。
