A 37-year-old man developed encephalopathy with prominent eosinophilia
. Magnetic resonance imaging (MRI) revealed multiple T2-weighted high
signal intensity lesions with dimeglumine gadopentetate (Gd-DTPA) enha
ncement on T1-weighted images, which were distributed in the cerebral
cortex, thalamus, deep white matter and cerebellum. He was diagnosed a
s having systemic mastocytosis on the basis of proliferating mast cell
s in the bone marrow and peripheral eosinophilia. Following steroid ad
ministration, there was a rapid improvement of his symptoms and labora
tory data. To our knowledge, this was the first reported case of syste
mic mastocytosis provoking encephalopathy with serial MRI findings.