From the School of Medical Sciences (K.R.S., F.D.d.L. T.J.R.R., L.P.R., M.C.F.), University of Campinas, Campinas, Brazil.
Seaman Family MR Research Center (R.F.C.), University of Calgary, Calgary, Alberta, Canada.
AJNR Am J Neuroradiol. 2021 Mar;42(3):610-615. doi: 10.3174/ajnr.A7017. Epub 2021 Jan 21.
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.
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 values < .05.
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 (ρ = -0.903, < .001).
Cervical spinal cord atrophy is found in some but not all hereditary spastic paraplegia subtypes. Spinal cord damage in SPG4 and 11 involves both GM and WM.
脊髓损伤是遗传性痉挛性截瘫的标志，但目前仍不清楚该病的特定亚型是否具有独特的脊髓灰质（GM）和白质（WM）受累模式。我们比较了不同遗传性痉挛性截瘫亚型患者的颈椎横断面 GM 和 WM 区域。我们还评估了这些指标是否与临床参数相关。
我们分析了 37 名患者（男 17 名；平均年龄 47.3[标准差 16.5]岁）和 21 名健康对照者（男 7 名；平均年龄 42.3[标准差 13.2]岁）。其中 7 名为痉挛性截瘫 3A 型（SPG3A）患者，12 名为 SPG4 患者，10 名为 SPG7 患者，8 名为 SPG11 患者。图像采集在 3T MR 成像扫描仪上进行，使用脊髓工具箱评估 T2*-加权 2D 图像。使用非参数检验和校正后的错误发现率值<.05 在 SPSS 中进行统计分析。
遗传性痉挛性截瘫组的平均病程为 22.4[标准差 13.8]年，痉挛性截瘫评分量表平均得分为 22.8[标准差 11.0]。我们未能在 SPG3A 和 SPG7 中发现脊髓萎缩。相比之下，我们在 SPG4 和 SPG11 患者中发现了异常。这两种亚型均存在脊髓 GM 和 WM 萎缩。SPG4 的 GM 面积与病程呈强烈负相关（ρ=-0.903，<.001）。
一些遗传性痉挛性截瘫亚型存在颈椎脊髓萎缩，但并非所有亚型均存在。SPG4 和 11 型的脊髓损伤涉及 GM 和 WM。
