Sturge-Weber Syndrome: A Case Report in a 39 Yr- Old Man with Delayed Diagnosis

Sturge Weber Syndrome is a rare neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal haemangioma), and leptomeningeal angioma most often involving the occipital and posterior parietal lobes. This syndrome consists of a constellation of clinical features such as a facial neavus, seizures, hemiparesis, intracranial calcifications and mental retardation. We report a 39 year old cobbler who presented at the neurology clinic of LAUTECH Teaching Hospital Osogbo Nigeria on account of recurrent generalized seizures and learning difficulties since childhood. On examination, he had a right hyperpigmented periorbital patch with chemosis and complete visual loss in the right eye. Electroencephalography (EEG) revealed generalized epileptiform activity and brain CT scan yielded multiple cerebral calcifications in keeping with Sturge Weber Syndrome. Patient is currently on carbamazepine, Folic acid and Cognitol and seizure control has improved remarkably and is being followed up at our out-patient clinic.