[Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease].

Introduction: Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases.

Methods: We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis.

Results: Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone.

Conclusion: Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.