Evidente V G, Adler C H, Giannini C, Conley C R, Parisi J E, Fletcher G P
Department of Neurology, Mayo Clinic Scottsdale, Arizona 85259, USA.
Mov Disord. 1998 May;13(3):576-81. doi: 10.1002/mds.870130335.
We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7-mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid-laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.
我们报告了一例罕见的厄尔德海姆-切斯特病（ECD），表现为进行性小脑综合征和尿崩症。在磁共振成像上，可见一个7毫米的轴外强化肿块包绕右椎动脉，尸检证实为含有脂质的泡沫状组织细胞构成的外膜黄色瘤。小脑综合征很可能是由于脑桥广泛的组织细胞浸润所致，尤其是脑桥基底部和小脑中脚。
