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Abstract

The Proteus syndrome: CNS manifestations.

R B Dietrich, D E Glidden, G M Roth, R A Martin and D S Demo
American Journal of Neuroradiology May 1998, 19 (5) 987-990;
R B Dietrich
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D E Glidden
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G M Roth
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R A Martin
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D S Demo
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Abstract

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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American Journal of Neuroradiology
Vol. 19, Issue 5
1 May 1998
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The Proteus syndrome: CNS manifestations.
R B Dietrich, D E Glidden, G M Roth, R A Martin, D S Demo
American Journal of Neuroradiology May 1998, 19 (5) 987-990;

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The Proteus syndrome: CNS manifestations.
R B Dietrich, D E Glidden, G M Roth, R A Martin, D S Demo
American Journal of Neuroradiology May 1998, 19 (5) 987-990;
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