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American Journal of Neuroradiology, Vol 19, Issue 5 987-990, Copyright © 1998 by American Society of Neuroradiology

REVIEWS

The Proteus syndrome: CNS manifestations

RB Dietrich, DE Glidden, GM Roth, RA Martin and DS Demo
Department of Radiological Sciences, University of California, Irvine, USA.

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.


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[Abstract] [PDF]


Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X