Spinal Cord Gray and White Matter Damage in Different Hereditary Spastic Paraplegia Subtypes
Spinal Cord Gray and White Matter Damage in Different Hereditary Spastic Paraplegia Subtypes
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DOI：
10.3174/ajnr.a7017
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发表时间：
2021-03-01
影响因子：
3.5
通讯作者：
Franca Jr, M. C.
中科院分区：
文献类型：
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作者：
Servelhere, K. R.;Casseb, R. F.;Franca Jr, M. C.
背景和目的：脊髓损伤是遗传性痉挛性截瘫的一个标志，但目前尚不清楚这种疾病的特定亚型是否具有脊髓灰质（GM）和白色（WM）受累的独特模式。我们比较了不同遗传性痉挛性截瘫亚型患者的颈部横截面GM和WM区域。我们还评估了这些指标是否与临床parameters.MATERIALS和METHODS相关：我们分析了37例患者（17名男性，平均年龄47.3 [SD，16.5]？21例健康对照组（7例男性，平均年龄42.3 [SD，13.2]？年）。痉挛性截瘫3A型（SPG3A）7例，SPG4型12例，SPG7型10例，SPG11型8例。图像采集采用3T磁共振成像仪，T2* 加权二维图像采用Spinal Cord MRI评估。统计分析进行了SPSS使用非参数检验和错误发现率？校正P值？结果：遗传性痉挛性截瘫组的平均病程为22.4 [SD，13.8]？年，平均痉挛性截瘫评定量表评分为22.8 [SD，11.0]。我们未能在SPG3A和SPG7中识别脊髓萎缩。相反，我们在SPG4和SPG11患者中发现了异常。两种亚型均有脊髓GM和WM萎缩。SPG4表现出很强的反相关GM面积和病程（？= ? 0.903，P？
BACKGROUND AND PURPOSE:Spinal cord damage is a hallmark of hereditary spastic paraplegias, but it is still not clear whether specific subtypes of the disease have distinctive patterns of spinal cord gray (GM) and white (WM) matter involvement. We compared cervical cross-sectional GM and WM areas in patients with distinct hereditary spastic paraplegia subtypes. We also assessed whether these metrics correlated with clinical parameters.MATERIALS AND METHODS:We analyzed 37 patients (17 men; mean age, 47.3 [SD, 16.5]?years) and 21 healthy controls (7 men; mean age, 42.3 [SD, 13.2]?years). There were 7 patients with spastic paraplegia type 3A (SPG3A), 12 with SPG4, 10 with SPG7, and 8 with SPG11. Image acquisition was performed on a 3T MR imaging scanner, and T2*-weighted 2D images were assessed by the Spinal Cord Toolbox. Statistical analyses were performed in SPSS using nonparametric tests and false discovery rate?corrected P values?< .05.RESULTS:The mean disease duration for the hereditary spastic paraplegia group was 22.4 [SD, 13.8]?years and the mean Spastic Paraplegia Rating Scale score was 22.8 [SD, 11.0]. We failed to identify spinal cord atrophy in SPG3A and SPG7. In contrast, we found abnormalities in patients with SPG4 and SPG11. Both subtypes had spinal cord GM and WM atrophy. SPG4 showed a strong inverse correlation between GM area and disease duration (? = ?0.903, P?