Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature

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S. Derrey

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...  

2018 ◽  
Vol 110 ◽  
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Akash J. Patel

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2021 ◽  
Vol 15 (1) ◽  
pp. 77-82
Author(s):  
Aleksandar Popovic ◽  
Christopher Curtiss ◽  
Timothy A. Damron

Background: Erdheim-chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, which typically presents with bilateral symmetric osteosclerosis and multi-organ involvement. Lesions may be intraosseous or extraosseous and involve the heart, pulmonary system, CNS, and skin in order of decreasing likelihood. Objective: The objective of this study is to discuss a case of erdheim-chester disease and conduct a review of the literature. Case: We describe a rare case of erdheim-chester in an asymptomatic 37-year-old male who was diagnosed after suffering a right ulnar injury. Subsequent evaluation revealed a solitary radiolucent ulnar lesion without multi-system involvement. Results & Conclusion: The case is unique in its solitary distribution, lytic radiographic appearance, and asymptomatic presentation preceding pathologic fracture. This presentation may simulate multiple other bone lesions.


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