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.
Liotta EM, Jhaveri MD, Fox JC, Venugopal P, Lewis SL. Erdheim-Chester Disease. Arch Neurol. 2012;69(11):1514. doi:10.1001/archneurol.2012.180