Baba Usman Ahmadu
Sturge-Weber syndrome is a rare neurocutaneous syndrome that includes a facial port-wine stain and associated leptomeningeal angiomatosis. The defect in Sturge-Weber syndrome occurs early during lamination, when the connection between cortical veins and superior sagittal sinus is impaired. The result of which are poor cerebral metabolism, dysfunctional leptomeningeal vascular malformation, progressive atrophy and calcification. We report a case of a three months old infant who was diagnosed with clinical Sturge-Weber syndrome based on focal tonic clonic convulsions associated with weakness of the right side of her body. Physical examination revealed port wine nevi, localized on the left half of her face along the distribution of trigeminal nerve with right sided hemiparesis even though tram-line sign was not visible on her skull radiograph. Computerized tomography and MRI of the brain were desired but facilities for these were lacking at our health center. The patient was placed on Phenobarbital and diazepam for break through seizures. She improved remarkably and was discharged on physiotherapy; follow up visits in addition to seeing the ophthalmologist
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