伴钙化及囊变的脑白质病影像学表现

Imaging Features of Leukoencephalopathy with Cerebral Calcifications and Cysts

目的:探讨伴钙化及囊变的脑白质病(LCC)的影像学特征及相关临床、病理学表现,以提高对该疾病的认识。方法:回顾性分析3例经病理确诊为LCC患者的临床表现、病理学资料及CT、MRI、PET-CT和PET-MR等影像学资料。结果:3例患者均为女性,年龄21~47岁。CT及MRI平扫显示3例均见脑深部核团及白质的钙化、多发囊性灶及弥漫脑白质病变。MRI增强扫描后囊性病灶均见囊壁不规则环形或结节状轻度强化。PET-CT或PET-MRI表现为囊性病灶代谢减低。组织病理学可见新鲜或陈旧性出血、含铁血黄素沉积、钙化、肉芽组织、薄壁血管聚集及脑组织胶质增生。结论:LCC具有较典型的钙化、囊变及弥漫脑白质病变"三联征"影像学表现,病灶PET低代谢有一定的特征。微血管病变及脱髓鞘共同参与疾病的发展,微血管闭塞性病变是其影像学表现的病理基础。

Purpose： To investigate the clinical, imaging and pathological features of leukoencephalopathy with cerebral calcifications and cysts （LCC） and to improve the diagnosis and understanding of the disease. Methods： The clinical, pathological and imaging data of 3 patients with LCC confirmed by pathology results were analyzed retrospectively. Results： All the 3 patients were female, age ranged from 21 to 47 years old. Plain CT and MRI scan showed calcification, multiple cystic lesions in the deep brain nuclei and white matter, and diffuse leukoencephalopathy in 3 cases. Irregular surrounding rim or nodular enhancement of cystic wall were seen in cystic lesions on MRI enhancement scan. PET-CT or PET-MRI showed decreased metabolism of cystic lesions. Histopathology exam showed hemorrhage, hemosiderin deposition, calcification, granulation tissue, thin-walled vessels aggregation and brain gliosis. Conclusion： LCC has typical imaging ＂triad syndrome＂, including calcification, cystic degeneration and diffuse leukoencephalopathy. Low metabolism of the cystic lesion on PET-CT or PET-MRI has some characteristic. Microvascular lesions and demyelination are involved in the development of this disease. Obliterative microangiopathy was found to be the basic abnormality in histopathology leading to the characteristic imaging findings.