脑厄尔海姆-切斯特病：两例伴有磁共振成像显示齿状核异常的进行性小脑综合征病例报告。
Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging.
作者信息
Pautas E, Chérin P, Pelletier S, Vidailhet M, Herson S
机构信息
Department of Internal Medicine, Hôpital de la Pitié-Salpétrière, Paris, France.
出版信息
J Neurol Neurosurg Psychiatry. 1998 Oct;65(4):597-9. doi: 10.1136/jnnp.65.4.597.
Abstract
Two patients with Erdheim-Chester disease with progressive cerebellar dysfunction and pyramidal signs are reported on. Cerebral MRI showed bilateral increased signal intensity in peridentatal regions on T2 weighted sequences. Both patients had kidney and bone involvement, established on bone biopsy for one. One patient improved with steroid therapy. This contrasts with previous reports, which describe rare neurological manifestations and the failure of different therapeutic approaches.
摘要
报告了两名患有厄尔德海姆-切斯特病且伴有进行性小脑功能障碍和锥体束征的患者。脑部磁共振成像（MRI）显示，在T2加权序列上，齿状核周围区域双侧信号强度增加。两名患者均有肾脏和骨骼受累，其中一名通过骨活检得以确诊。一名患者接受类固醇治疗后病情好转。这与之前的报告形成对比，之前的报告描述了罕见的神经学表现以及不同治疗方法的无效性。
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