Cerebral manifestation of Erdheim-Chester disease: Clinical and radiologic findings

被引:45
作者
Bohlega, S
Alwatban, J
Tulbah, A
Bakheet, SM
Powe, J
机构
[1] King Faisal Specialist Hosp & Res Ctr, Dept Neurosci, Neurol Sect, Riyadh 11211, Saudi Arabia
[2] King Faisal Specialist Hosp & Res Ctr, Dept Radiol, Sect Med Imaging, Riyadh 11211, Saudi Arabia
[3] King Faisal Specialist Hosp & Res Ctr, Dept Pathol & Lab Med, Riyadh 11211, Saudi Arabia
关键词
D O I
10.1212/WNL.49.6.1702
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
A 33-year-old woman presented with a 3-year history of progressive numbness in the hand, cerebellar ataxia, limb weakness, nystagmus, and dysarthria. T2-weighted MRI revealed abnormal foci of increased signal intensity mimicking demyelinating plaques in the periventricular white matter, and brain (18)FDG-PET scan showed increased uptake in the pens. Biopsy from a tibial lesion showed aggregates of foamy histiocytes in the intertrabecular spaces replacing the bone marrow, characteristic of Erdheim-Chester disease. The patient was treated with craniospinal radiation. After 6 months, the clinical picture was stable and the MRI was unchanged.
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页码:1702 / 1705
页数:4
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