Teaching Video NeuroImages: Multisystemic Erdheim-Chester Disease Presenting as a Cerebellar Ataxia

A 41-year-old man was admitted to the neurology ward due to progressive vertigo and unsteadiness for the previous 2 months. Neurologic examination was remarkable for a global cerebellar syndrome. Investigation with brain MRI led to the hypothesis of a histiocytosis due to infiltrative lesions of the pons, cerebellar peduncles, and pituitary. Therefore, investigation progressed with chest/abdomen/pelvis CT, bone scintigraphy, and a tibial biopsy that confirmed the diagnosis of Erdheim-Chester disease (figures 1 and 2, video).

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