Sturge-Weber Syndrome with Diffuse Truncal Lesions: Case Presentation

Abstract

Sturge-Weber syndrome is characterised with central nervous system anomalies and cutaneous angiomatosis. A vascular nevus on one side of the face, ipsilateral meningeal angiomatosis, epileplic seizures, glaucoma and mental retardation are the typical components. The cutaneous lesions may be more diffuse in a relatively small group of patients. Here we report the clinical, radiological and electroencephalographical findings of a thirteen years old girl having diffuse cutaneous lesions on her body except the upper and middle parts of the left side of the face. She had left hemiparesis, more dominant in the upper extremity, glaucoma and secondary optic atrophy of the right eye and mental retardation. Her generalised tonic clonic seizures had increased in frequency. Contrast enhanced cranial MRI showed diffuse right hemispheric leptomeningeal and dural enhancement besides choroidal plexus lesion seen relatively less often. In her interictal EEG bioelectrical disorganisation in the right hemisphere and epileptiform anomalies originating from the right frontal and left parietal regions were detected. Her misbehaviour besides her seizures was remarkable and the seizures were controlled with valproic acid.