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Images in Neurology |

Erdheim-Chester Disease

Eric M. Liotta, MD; Miral D. Jhaveri, MD; John C. Fox, MD; Parameswaran Venugopal, MD; Steven L. Lewis, MD
Arch Neurol. 2012;69(11):1514. doi:10.1001/archneurol.2012.180.
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A 41-year-old man had a 3-year history of vertigo that began at an amusement park, progressed to occur with head movements, and eventually persisted with associated diplopia and ataxia. He had a 10-year history of diabetes insipidus, hypotestosteronism, and hyperprolactinemia. Neurologic examination demonstrated saccadic pursuit, right gaze nystagmus, left hand dysmetria, and ataxic gait. Inspection of the skin showed several 2-mm to 6-mm firm, well-defined papules in different phases of evolution that had been present for 2 years (Figure 1A-B). High-dose intravenous methylprednisolone sodium succinate had recently been administered for a working diagnosis of neurosarcoidosis and was associated with improvement of symptoms that recurred with tapering of oral prednisone.

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Figure 1. Skin lesions. A, Photograph of the patient's early skin lesion. B, Photograph of a late, resolving lesion. C, Hematoxylin-eosin staining of lesion biopsy demonstrates foamy histiocytes (arrowheads) and multinucleated giant cells (arrow) (original magnification × 40).

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Figure 2. Radiographic findings. Radiographs show T2/fluid-attenuated inversion recovery hyperintense lesions involving the centrum semiovale and dentate nuclei (A and B; arrows). Postcontrast T1 images show nodular enhancement (C and D; arrows). Radiographs show areas of sclerosis in the distal femur (E and F; arrows). G, Radiograph shows multifocal diffuse, patchy uptake in the distal diaphysis of the femora and proximal tibiae (arrows) on technetium 99 bone scan.




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