Kokubun Norito, Ishihara Tetsuya, Nishibayashi Momoka, Ikeda Syun-ichiro, Nagashima Kazuo, Hirata Koichi
Department of Neurology, Dokkyo University, School of Medicine.
Rinsho Shinkeigaku. 2005 Sep;45(9):663-8.
A 61-year-old man with no history of HIV infection developed a subacutely progressive dementia and left hemiparesis. Brain MRI showed a high intensity lesion in the right frontal lobe on T2 weighted image. There was no contrast enhancement after gadolinium-DTPA administration. 1H MRS revealed a marked decrease in the n-acetyl aspartate/creatine ratios and an increase in the choline/creatine ratio. A lactate peak also was present. A low-grade glioma was suspected and he was admitted to our hospital. On examination, there was a mild dementia and left hemiparesis. A peripheral blood count revealed lymphocytopenia (426/mm3) with a CD4/CD8 ratio of 0.28. No evidence of HIV infection, malignancies or collagen disease was found. A brain biopsy revealed no tumor cells but instead demyelinated brain tissue with large nucleated cells. JC virus antigen was detected in the cells of the demyelinated lesions. A diagnosis of PML associated with idiopathic CD4 positive lymphocytopenia was made. There are only a few reports concerning 1H-MRS findings in patients with PML and the present case illustrates the difficulty of making a differential diagnosis between PML and glioma.
一名61岁无HIV感染史的男性出现亚急性进行性痴呆和左侧偏瘫。脑部MRI在T2加权图像上显示右额叶有高强度病变。给予钆喷酸葡胺后无强化。氢质子磁共振波谱(1H MRS)显示N-乙酰天门冬氨酸/肌酸比值明显降低,胆碱/肌酸比值升高。还出现了乳酸峰。怀疑为低级别胶质瘤,遂收入我院。检查发现有轻度痴呆和左侧偏瘫。外周血细胞计数显示淋巴细胞减少(426/mm³),CD4/CD8比值为0.28。未发现HIV感染、恶性肿瘤或胶原病的证据。脑活检未发现肿瘤细胞,而是发现脱髓鞘脑组织伴有大核细胞。在脱髓鞘病变的细胞中检测到JC病毒抗原。诊断为与特发性CD4阳性淋巴细胞减少相关的进行性多灶性白质脑病(PML)。关于PML患者的1H MRS表现的报道很少,本病例说明了PML与胶质瘤进行鉴别诊断的困难。