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    ABSTRACT: Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. It is characterized by osteosclerosis of the metaphyseal regions of long bones and several extraskeletal manifestations. Clinically, it ranges from an asymptomatic focal process to systemic disease with life-threatening visceral involvement. Until now, only two cases of Erdheim-Chester disease with paraparesis have been reported. Herein we report the first case of Erdheim-Chester disease with the clinical manifestation of paraplegia. Our patient also had diabetes insipidus, pleural and pericardial effusion, retro-orbital and cavernous sinus masses, fibrotic changes in the retroperitoneal, perirenal, and periaortic areas, and epidural space-occupying lesions. We want to emphasize that ECD may be a very rare cause of paraplegia.
    European Journal of Internal Medicine 03/2003; 14(1):53-55. DOI:10.1016/S0953-6205(02)00208-X · 2.30 Impact Factor
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    ABSTRACT: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. We present a case of a 56-year-old male with ECD. As time progressed, involvement of the orbital fossa, cranial convexity, spinal cord, brain stem, thyroid, lung, retroperitoneum, lower extremity bones and skin were found. Previously reported cases reveal the frequency of ECD with spinal cord involvement is rare. Although this was a presumed diagnosis based on other lesions, our case is the first in which both intramedullary and epidural masses are present.
    The British journal of radiology 03/2012; 85(1011):e62-4. DOI:10.1259/bjr/65720693 · 1.53 Impact Factor
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    ABSTRACT: There are very few reported cases of Erdheim-Chester disease that document involvement of dura at the level of the spinal cord. Among these reports, we know of no publication that includes detailed MRI findings. To the best of our knowledge, the case presented here is the first published report of this specific manifestation of Erdheim-Chester disease that includes detailed MRI findings in addition to the related history. Spinal manifestations of Erdheim-Chester disease in our patient were at the dorsal and lumbar levels (T1-T6 and T12-T11 respectively). Both epidural and subdural linear large masses were present, causing spinal cord compression at the dorsal level and epidural thickening at the lumbar level.
    Neuroradiology 01/2003; 44(12):1004-7. DOI:10.1007/s00234-002-0853-4 · 2.37 Impact Factor


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May 20, 2014