UNUSUAL MRI AND PATHOLOGICAL FINDINGS OF PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY COMPLICATING ADULT WISKOTT-ALDRICH SYNDROME

We present a long-surviving patient with Wiskott-Aldrich syndrome comp licated by atypical progressive multifocal leukoencephalopathy (PML). MRI showed multiple tiny spots of Gd-DTPA-enhanced lesions on the T-1- weighted image. Pathologic findings for brain biopsy were patchy demye linated vascularized lesions infiltrated by a surprising number of eos inophils. The presence of polyomavirus JC was confirmed by in situ hyb ridization and polymerase chain reaction. PML should be included in th e differential diagnosis when Gd-DTPA-enhanced spotty lesions are pres ent in the white matter, especially in patients who have a mild immuno logic defect.