Choreo-Athetosis and Ataxia as Leading Features in a Case of Erdheim-Chester Disease

Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis that presents with multifocal osteosclerotic bone lesions; although tissue infiltration can occur in almost every organ, skeleton, retroperitoneum (mainly the kidney and aorta) and orbit involvement is common.1 Being a multiorgan disease accounts for the protean clinical manifestations, of which bone pain is the most frequently reported, whereas neurological symp- toms are rare and include ponto-cerebellar dysfunction associated with hypopituitarism and seizures.1 The described brain magnetic resonance imaging abnormalities consist of proliferative tissue invasions of the hypophysis, cerebellum, and brainstem.2 Here we report an atypical ECD presentation manifesting with movement disorders.