非酮症性高血糖偏侧舞蹈症脑部CT及MRI表现

目的分析非酮症性高血糖偏侧舞蹈症患者脑部CT及MRI影像学特点。方法回顾性分析11例经临床确诊的非酮症性高血糖偏身舞蹈症患者的脑部CT及MRI表现。11例患者均接受至少一次CT及MRI平扫,其中2例行MRI增强检查,4例加做DWI序列,6例加做MRS序列,2例加做DTI序列,3例复查MRI平扫,1例复查CT平扫。结果 9例CT见豆状核和/或尾状核头部片状高密度影,2例CT阴性;T_1WI 9例高信号,2例阴性;DWI 2例高低混杂信号,1例低信号,1例阴性;MRS检查:NAA峰6例均降低,Cho峰6例均升高,NAA/Cr比值6例均降低,Cho/Cr比值6例均升高;DTI 1例显示左侧内囊前肢纤维束受损,FA值减低,1例阴性。结论非酮症性高血糖偏侧舞蹈症脑部CT及MRI均具有特征性影像表现,DWI、MRS及DTI等序列对确定病变性质、评估神经元丢失或损伤、纤维束受损等有价值。

Cranial CT and MR imaging findings in non-ketotic hyperglycemia hemichorea

Objective To analyze the brain CT and MR imaging manifestations of non-ketotic hyperglycemia hemichorea. Methods Eleven patients with clinically-proved non-ketotic hyperglycemia chorea underwent unenhanced CT and MRI (11), contrast-enhanced MRI (2), diffusion-weighted MRI (4), MR spectroscopy (6), diffusion tensor imaging (2), follow-up MRI (3) and CT (1). Results Patchy hyperdense lesions in the basal ganglia were seen on CT and T1WI of 9 patients, while the other 2 patients had no positive findings on CT and MRI. The lesions were heterogeneous in 2/4, hypointense in 1/4, and isointense in 1/4 patients who underwent diffusion-weighted MRI. MR spectroscopy showed diminished NAA and elevated Cho , NAA/Cr and Cho/Cr in all 6 patients. Diffusion tensor imaging was normal in 1 patient, showed damage in the anterior limb of the left internal capsule fiber bundle with reduced FA in the other patient. Conclusion There are characteristic features on CT, MRI, diffusion-weighted MRI , MR spectroscopy and diffusion tensor imaging for assessment of brain insult in non-ketotic hyperglycemia hemichorea.