Protons syndrome: craniofacial and cerebral MRI

The Proteus syndrome is a rare hamartoneoplastic syndrome that may affect t he brain, skull, and extracranial head and neck. We present a case with sev ere, characteristic findings. Brain abnormalities are not common in Proteus syndrome; when present, hemimegalencephaly and migrational disorders are t ypically seen, commonly with an associated seizure disorder. Maxillary and mandibular dysmorphism may occur, including unilateral condylar hyperplasia , Subcutaneous fatty, fibrous, lymphangiomatous masses commonly seen in thi s syndrome may involve the neck and face, leading to disfigurement and pote ntial airway compromise.