A clinician's dilemma: Sturge-Weber syndrome ‘without facial nevus’!!
An 8-year-old boy presented with recurrent seizures since 5 years of age. He had normal birth and developmental history but poor scholastic performance. He had complex partial seizures (CPS) of extra temporal of left hemispheric origin with frequency of 3–4/month. He did not have any neurocutaneous markers, had impaired visual acuity (6/9 both eyes) with tubular field defect without evidence of glaucoma, left hemiatrophy of body, and left hemiparesis. His MRI showed right hemispheric atrophy with cortical T1/T2 hypointensity and intense blooming on susceptibility weighted images (SWI). Gadolinium enhanced T1 weighted images showed right temporo-occipital sulcal enhancement consistent with pial angiomatosis [Figures –]. A diagnosis of type III SWS was considered. On electroencephalography (EEG), right hemisphere showed voltage suppression with right posterior head region (PHR) spike wave discharges. He underwent right temporo-occipital lesionectomy in view of the refractory seizures and is seizure-free at 4 year follow-up. Histopathology showed vascular malformation with capillary telangectasia with extensive calcification supporting the diagnosis of SWS.