TY - JOUR T1 - The Proteus syndrome: CNS manifestations. JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 987 LP - 990 VL - 19 IS - 5 AU - R B Dietrich AU - D E Glidden AU - G M Roth AU - R A Martin AU - D S Demo Y1 - 1998/05/01 UR - http://www.ajnr.org/content/19/5/987.abstract N2 - Proteus syndrome is a complex hamartomatous disorder characterized by multiple, diverse, somatic manifestations. We present a case in which severe, evolving CNS abnormalities were also exhibited. Imaging findings at presentation included hemimegalencephaly, subependymal calcified nodules, and periventricular cysts. Subsequently, dural sinus thrombosis developed. Eight previously reported patients may also have had hemimegalencephaly. When neuroimaging studies show hemimegalencephaly in a child with pigmented skin lesions, Proteus syndrome should be considered in the differential diagnosis. ER -