STURGE-WEBER-SYNDROME IN A 23 YEAR OLD SECONDARY SCHOOL DROP-OUT: A CASE REPORT

*Balarabe S.A., Abubukar M., Jamila M., Akintomide T. and Aliyu A.

ABSTRACT

Sturge-Weber syndrome is a rare sporadic developmental neurocutaneous disorder, of undetermined etiology, that is characterized by unilateral or bilateral port-wine stain (PWS), focal or generalized epileptic seizures, hemiparesis, learning difficulties, mental retardation and ocular features such as glaucoma. It is a rare disorder occurring with a frequency of 1:50,000 live births, there is no racial or sex predilection as both sexes and races are affected equally. Currently, there are clear differences for the minimum diagnostic criteria required for the diagnosis of Sturch-Weber Syndrome, that includes a triad of cutaneous (facial) angioma, leptomeningeal angiomatosis and ocular involvement, or facial angioma and the possibility of glaucoma but with no clear evidence of intracranial disease, or just based on the presence of intracranial leptomeningeal vascular malformation. We present herein, a case of 23 year old secondary school drop-out, who presented with typical clinical and radiological features of Sturge-Weber Syndrome. To the best of our knowledge, this is the first reported case of Sturge-Weber syndrome from North-Western Nigeria.

Keywords: Sturch-Weber Syndrome, Neurocuteneous disease, Epilepsy, Leptomenningial angiomatosis, Port-wine stain.