Radiologic Manifestations of Proteus Syndrome

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The Proteus syndrome: CNS manifestations.

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 patien...

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Craniofacial and dental manifestations of Proteus syndrome: a case report.

The Proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report we describe the craniofacial clinicopathological development in a girl with Proteus syndrome from age 6 to 20 years. The patient had pro...

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Cutaneous manifestations of proteus syndrome: correlations with general clinical severity.

BACKGROUND Proteus syndrome is a rare congenital disorder with progressive asymetric overgrowth of multiple tissues. OBJECTIVES To determine the range of cutaneous findings in Proteus syndrome and to correlate cutaneous findings with overall disease severity. DESIGN A prospective cohort study was performed at the National Institutes of Health, a tertiary referral center. PATIENTS Twenty-f...

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Proteus Syndrome with Neurological Manifestations: A Rare Presentation

Proteus syndrome (PS) is an extremely rare and complex disorder. Approximately 200 cases have been reported, and it seems to affect people of all ethnic and racial groups. PS is characterized by segmental overgrowth of multiple tissues and organs including vascular malformations, lipomatous overgrowth, hyperpigmentation, and various types of nevi. We hereby present a 7-year-old boy who presente...

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ژورنال

عنوان ژورنال: RadioGraphics

سال: 2004

ISSN: 0271-5333,1527-1323

DOI: 10.1148/rg.244035726