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Proteus syndrome with syringohydromyelia and arachnoid cyst
Authors:Yonca Anik  Ihsan Anik  Erdem Gonullu  Nagihan Inan  Ali Demirci
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
Abstract:

Introduction

Proteus 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 methods

We 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.
Keywords:
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