Leigh综合征头颅质子磁共振波谱影像表现探讨

摘要

目的 探讨Leigh综合征头颅质子磁共振波谱(1H-MRS)的影像特点及诊断价值.方法 对我院收治的临床诊断为Leigh综合征10例的头颅MRI及1H-MRS表现进行回顾性分析.结果 本组10例双侧基底节区及中脑导水管周围灰质区、3例脑干背侧、1例小脑常规MRI检查均可见对称性长T1、长T2信号,FLAIR像上为高信号.弥散加权成像(DWI)7例显示高信号,扩散系数(ADC)图呈低信号；3例表现为等及低信号.10例灌注加权成像(PWI)示双侧豆状核、尾状核病灶均为高灌注,但其他部位病灶表现为低灌注.本组病变区域1H-MRS表现为胆碱峰升高,N-乙酰天门冬氨酸峰降低；病变区域、脑脊液及常规MRI图像表现正常的区域均可见巨大乳酸双峰.结论 对Leigh综合征1H-MRS可提供直接反映疾病代谢异常的信息,联合应用常规MRI、DWI、PWI有利于该病的诊断和鉴别诊断.%Objective To explore the characteristics and value of spectrum on brain proton magnetic resonance spectroscopy ( 1 H-MRS) and its value in diagnosis of Leigh syndrome. Methods 1 H-MRS and MRI of 10 patients with Leigh syndrome in our hospital were retrospectively analyzed. Results Hyperintensity on T2WI/FLAIR and hypointensity on T1WI were presented in 10 patients'bilateral basal ganglia and periaqueductal gray matter (PAG) , in 3 patients'dorsal brain stem and in 1 patient's cerebellar nuclei. 7 patients showed hyperintense on diffuse weighing imaging (DWI) and hypointensity on diffusion coefficient (ADC); 3 patients showed equal hypointensity. Perfusion weighted imaging (PWI) of 10 patients showed hypertransfusion on bilateral lenticular nucleus and caudate nucleus lesions, and hypoperfusion on the other positions of lesions. 1H-MRS of the Leigh syndrome cases showed elevated choline (Cho) peak and decreased N acetylaspartate (NAA) peak; huge two-humped lactates were presented in the diseased region, cerebrospinal fluid (CSF) and normal areas by routine brain MRI imaging. Conclusion H-MRS may directly reflect the metabolism changes of Leigh syndrome, and may be helpful in diagnosis and differential diagnosis of the disease when combined with routine MRI, DWI and PWI.