Proteus syndrome with syringohydromyelia and arachnoid cyst |
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Authors: | Yonca Anik Ihsan Anik Erdem Gonullu Nagihan Inan Ali Demirci |
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Affiliation: | (1) School of Medicine, Department of Radiology, University of Kocaeli, 41380 Umuttepe, Kocaeli, Turkey;(2) School of Medicine, Department of Neurosurgery, University of Kocaeli, Umuttepe, Kocaeli, Turkey;(3) School of Medicine, Department of Pediatry, University of Kocaeli, Umuttepe, Kocaeli, Turkey |
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Abstract: | IntroductionProteus syndrome (PS) is a rarely seen, sporadic disorder with a wide range of abnormalities including asymmetrical overgrowth, skin lesions, dysregulated adipose tissue, and vascular malformations. Brain and spinal malformations are extremely rare; syringohydromyelia and arachnoid cyst have not been reported previously.Materials and methodsWe present a 5-year-old girl with PS having severe central nervous system (CNS) abnormalities demonstrated by magnetic resonance imaging (MRI) including craniocutaneous lipomatosis, hemimegalencephaly, arachnoid cyst, and syringohydromyelia. |
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