Kumar Kishore R, Hon Kenneth, Schultz David, Agzarian Mark J, Jones David Neil, Thyagarajan Dominic
Royal Adelaide Hospital, Adelaide, South Australia, Australia.
Neurologist. 2009 Nov;15(6):351-4. doi: 10.1097/NRL.0b013e3181940244.
Stroke-like episodes associated with Sturge-Weber Syndrome have previously been attributed to thrombosis within the leptomeningeal malformation causing infarction.
A 22-year-old woman with a right-sided port-wine nevus presented with acute left hemiparesis. Brain magnetic resonance imaging (MRI) demonstrated a vascular malformation of the right cerebral hemisphere consistent with Sturge-Weber Syndrome. There was mild edema of the right hemisphere cortex. While in hospital she developed recurrent complex partial seizures. Repeat brain MRI showed a diffusion abnormality affecting the right hemisphere with an increase in the degree of edema. There was no evidence of thrombosis on cerebral angiography. The patient's neurologic deficit and acute MRI changes subsequently resolved.
Although cerebral angiography could potentially demonstrate thrombosis causing large vessel obstruction, it is unlikely to have the resolution to detect stasis at a microvascular level. Microcirculatory stasis with reversible ischemia is the likely pathogenic mechanism in this case.
