A Case of Headache and Hemiparesis in a Woman with a Port-wine Stain
A 22 year old woman with a right sided port-wine stain developed a severe headache, followed by left arm weakness which developed after 4 hours. 4 days later she presented to our institution with a dense left hemiparesis. A CT head was initially reported as showing a right sided arteriovenous malformation (AVM) with subtle oedema of the right cerebral hemisphere. An MRI subsequently demonstrated pial angiomatosis with abnormal collateral cerebral veins commonly seen in Sturge-Weber Syndrome (SWS) rather than a true AVM. Her headache and neurological deficit gradually improved until the 5th day of her admission when she developed complex partial seizures. A repeat MRI showed a subtle increase in the degree of midline shift and evidence of ischemia of the right cerebral hemisphere which was not apparent on the previous study. Digital subtraction angiography was performed but no evidence of thrombosis was found. A PET scan showed a mild reduction in FDG uptake in the right cerebral hemisphere. Recurrent thrombosis with infarction is postulated to be the cause of stroke like episodes in SWS, however no thrombosis was found despite extensive imaging. This patient developed seizures much later in life than would be expected. These seizures were most likely triggered by ischemia, with corresponding ischaemic changes on MRI.