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目的探讨单纯型遗传性痉挛性截瘫(purehereditary spastic paraplegia,PHSP)SPG6型患者的中枢神经系统MRI表现,提高对该病的认识。资料与方法分析一个家系6例PHSPSPG6型患者中枢神经系统的MRI表现,6例均行颅脑和脊髓MRI,分别测量C3、C7、T2、T4、T7脊椎水平脊髓前后径、横径和横断面积。另外选取60例在本院行颈椎和胸椎(各30例)MRI的患者作为对照组,测量方法同患者组,计算脊髓前后径、横径和横断面积的均值,并与患者组的均值进行比较。结果 6例中,5例颅脑MRI表现未见明显异常,1例表现为老年性脑改变;其颈髓及胸髓呈不同程度变细,灰白质均受累,蛛网膜下腔扩大;在变细明显的脊髓节段灰白质分界显示清楚,横轴位T2WI上灰质呈边界清楚、左右对称的点状或点片状高信号,矢状位上表现为连续纵行的条状高信号。6例患者的C3、C7、T2、T4脊椎水平的脊髓横断面积、前后径及横径明显小于对照组,二者之间的差异有统计学意义,T7脊椎水平的脊髓横断面积、前后径及横径与对照组之间的差异无统计学意义。结论 PHSPSPG6型患者的颅脑MRI表现可为正常。颈髓及上、中胸髓在MRI上表现为变细,灰白质分界清楚,下胸髓受累较轻,其MRI表现有一定的特征性。
Objective To investigate the MRI features of central nervous system in patients with pure hereditary spastic paraplegia (PHSP) type SPG6 to improve their understanding of the disease. Materials and Methods MRI findings of central nervous system in 6 PHSPSPG6 patients were analyzed in one pedigree. Six patients underwent MRI of cranial brain and spinal cord. The anteroposterior diameter, transverse diameter and cross-sectional area of spinal cord at C3, C7, T2, T4 and T7 . In addition, 60 patients with MRI of the cervical vertebra and thoracic vertebrae (30 in each) were selected as the control group. The mean of anteroposterior diameter, transverse diameter and cross-sectional area of the spinal cord were calculated and compared with those of the patient group . Results Among the 6 cases, MRI findings of 5 cases showed no significant abnormalities in brain and 1 case showed senile brain changes. The cervical and thoracic spinal cord were thinner and more deformable in both the gray matter and the subarachnoid space. The obvious gray-white boundary of the spinal cord segment clearly shows that the gray matter on the horizontal axis T2WI has a clear boundary, a symmetrical punctate or punctate high signal, and the sagittal plane shows a continuous high stripe signal. The spinal cord cross-sectional area, anteroposterior diameter and transverse diameter of C3, C7, T2, T4 spine of 6 patients were significantly less than those of control group. The difference between the two groups was statistically significant. The spinal cord cross-sectional area, anteroposterior diameter The difference between the diameter and the control group was not statistically significant. Conclusions MRI manifestations of brain in PHSPSPG6 patients may be normal. Cervical pulp and the upper and middle thoracic plexuses showed thinning on MRI, clear demarcation of the gray matter, lower involvement of the lower thoracic cavity, and its MRI findings have certain characteristics.