遗传性痉挛性截瘫5型患者脑白质微结构改变的脑白质纤维束追踪法研究

Changes of White Matter Microstructure in Patients with Hereditary Spasmodic Paraplegia Type 5 via Magnetic Resonance Region of Interest Tracing Method

目的基于磁共振感兴趣区脑白质纤维束追踪法,探讨遗传性痉挛性截瘫5型(SPG5)患者脑白质微结构的变化特点。资料与方法前瞻性收集2019年1月—2020年12月在福建医科大学附属第一医院就诊的SPG5患者17例,通过社会公开招募性别、年龄匹配的健康对照者17名,行全脑磁共振扩散张量成像,追踪受试者双侧大脑的运动传导通路和感觉传导通路的纤维束,计算各向异性分数(FA)和平均扩散系数(MD),进行组间比较,采用Pearson相关性分析SPG5患者扩散张量成像参数与病程、疾病严重程度痉挛性截瘫评价量表及血浆27-羟基胆固醇含量的相关性。结果与对照组比较,SPG5患者在运动及感觉传导通路纤维束上FA值较低,MD值较高,差异均有统计学意义(P<0.05),延髓、放射冠及大脑皮层下白质节段二者FA值(t=-7.05~-2.01)及MD值(t=-0.25~6.50)的差异较脑桥、中脑、内囊节段的FA值(t=-4.84~1.04)及MD值(t=-1.71~4.33)大。SPG5患者扩散张量成像参数与病程、痉挛性截瘫评分量表评分及血浆27-羟基胆固醇含量均无相关性(P均>0.05)。结论SPG5患者在运动及深感觉相关的纤维束脑白质微结构呈节段性损伤趋势而非逆行性改变,白质微结构损伤程度与疾病严重程度、病程及血浆神经毒性物质含量均无相关性。

Purpose To investigate the characteristics of white matter microstructure changes in patients with hereditary spastic paraplegias type 5(SPG5)via magnetic resonance region of interest fiber tractography.Materials and Methods In this prospective study,seventeen patients with SPG5 were enrolled in the First Affiliated Hospital of Fujian Medical University from January 2019 to December 2020,and seventeen age-gender matched healthy controls(HCs)were recruited from the public.Whole brain magnetic resonance diffusion tensor imaging(DTI)was performed,tracking the fiber bundles of bilateral brain motor conduction pathway(CST)and somatosensory pathway(SSP).The differences in fractional anisotropy(FA)and mean diffusivity(MD)values were analyzed between SPG5 patients and HCs.Pearson correlations between clinical data[disease duration,spastic paraplegia rating scale(SPRS score),and plasma 27-hydroxycholesterol(27-OHC)content]and DTI data were evaluated.Results SPG5 patients exhibited significantly lower FA values(t=-7.05-2.01)and significantly higher MD values(t=-0.25-6.50)in the white matter of SSP and CST than those of HCs(all P<0.05).The differences of FA(t=-4.84-1.04)and MD(t=-1.71-4.33)in medulla oblongata,corona radiata,and subcortical white matter segments were significantly greater than those of pons,midbrain,and internal capsule segments in SPG5 group.DTI parameters showed no correlation with disease duration,SPRS score,or 27-OHC content in SPG5 patients,respectively(all P>0.05).Conclusion In SPG5 patients,segmental damage rather than retrograde change is observed in the microstructures of fiber bundles related to motor and deep sensation,and the degree of white matter damage is not correlated with the disease severity,duration,or plasma neurotoxic substance content.