目的　总结同心圆硬化临床、影像及病理特点 ,并与多发性硬化比较。方法　总结临床确诊的同心圆硬化 4例 ,均行头颅影像、腰穿、神经电生理检查 ,2例行脑活检。结果　 4例发病平均年龄 33岁 ,均以淡漠、反应迟钝、少语等症状起病 ,临床表现主要是性格和行为改变 ,如交往困难、淡漠寡言 ,均有疲乏感 ,3例有强笑强哭 ,2例偏身肌力减退。激素有一定疗效 ,4例随访无复发。头颅影像除 1例外均为多病灶 ,病灶早期可见“煎鸡蛋”样改变 ,CT上强化不明显 ,而MRI上呈明显环形或半环形强化。同心圆影像改变多需数月追踪始现。病灶常累及皮质及皮质下区域 ,虽多且大 ,但症候较轻。 2例活检证实脱髓鞘且有明显同心层改变及血管周围淋巴细胞“套袖”样浸润。结论　同心圆硬化亚急性或慢性起病 ,以精神障碍和行为异常为主要临床表现 ,存在“影像与症候分离”现象 ,且多数同心圆改变需追踪可见。该病从起病、临床症候、影像特点等方面与多发性硬化有较大区别。 Objective To summarize the clinical, imaging and pathological features of concentric sclerosis and to compare with multiple sclerosis. Methods The clinical diagnosis of concentric sclerosis in 4 cases were performed cranial imaging, lumbar puncture, nerve electrophysiological examination, 2 cases of brain biopsy. Results The average age of 4 cases was 33 years old. All of them were onset of symptoms such as indifference, unresponsiveness and few words. The main clinical manifestations were personality and behavioral changes, such as difficulty in communication, indifference, fatigue, and strong laughter in 3 cases Cry, 2 cases of partial muscle weakness. Hormones have a certain effect, 4 cases no recurrence. All cases of craniocerebral lesions were multifocal except for the case of “fried egg” in the early stages of the lesion. The enhancement on the CT was not obvious, and on the MRI, obvious circular or semi-annular enhancement was observed. Concentric image changes take months to track. Lesions often involve the cortex and subcortical regions, although many and large, but the symptoms are mild. Two cases of biopsy confirmed demyelination and significant concentric changes and perivascular lymphocytes “sleeve” -like infiltration. Conclusion Concentric sclerosis subacute or chronic onset, with mental disorders and behavioral disorders as the main clinical manifestations, there is “the phenomenon of image and symptom separation”, and most of the concentric changes to be traced. The disease from onset, clinical symptoms, imaging features and other aspects of multiple sclerosis are quite different.