CEREBRAL MANIFESTATION OF ERDHEIM-CHESTER DISEASE - CLINICAL AND RADIOLOGIC FINDINGS

A 33-year-old woman presented with a 3-year history of progressive num bness in the hand, cerebellar ataxia, limb weakness, nystagmus, and dy sarthria. T2-weighted MRI revealed abnormal foci of increased signal i ntensity mimicking demyelinating plaques in the periventricular white matter, and brain (18)FDG-PET scan showed increased uptake in the pens . Biopsy from a tibial lesion showed aggregates of foamy histiocytes i n the intertrabecular spaces replacing the bone marrow, characteristic of Erdheim-Chester disease. The patient was treated with craniospinal radiation. After 6 months, the clinical picture was stable and the MR I was unchanged.