Paroxysmal vascular events in Sturge– Weber syndrome: Role of aspirin

Abstract

Sturge–Weber syndrome (SWS) is a rare, sporadically occurring neurocutaneous disorder with a frequency of approximately 1 per 50,000. The hallmark is an intracranial leptomeningeal vascular angioma in association with a port wine nevus, usually involving ophthalmic or maxillary distribution of trigeminal nerve. Other clinical findings associated with SWS are seizures, glaucoma, hemiparesis and mental retardation. The radiological hallmark is “Tram-line” or “Gyri-form” calcification. 25 to 56% of patients experience recurrent episodes of paroxysmal focal neurological deficits in form of transient hemiparesis, which may be due to vascular ischemia or postictal in origin. EEG helps to differentiate the exact etiology, as it is normal in former. Aspirin prophylaxis in those, due to ischemia decreases their recurrences and improves overall neurological prognosis. We report a 25-month-old child of SWS with recurrent episodes of transient hemiparesis and atypical midline location of facial vascular nevus.

Sturge-Weber syndrome type 3 manifesting as ‘Status migrainosus’

“…Seizures often present in the first year of life as a consequence of the angioma initiating ischaemia, hypoxia and gliosis, which causes cortical irritability 6. In our case, the first seizure manifested at 6 years of age with no further seizures for 3 years despite not being on any treatment.…”

“…SWS is characterised by a port-wine stain, leptomeningeal angiomatosis and ophthalmic abnormalities; these are caused by an abnormal persistence of the embryonic vascular system 6. It is believed that this syndrome's possible cause is the formation of a vascular plexus around the cephalic portion of the neural tube 7.…”

“…Neurological deficits in the form of transient hemiparesis can be present in 65% of patients,13 and these may be postictal or due to vascular ischaemia 6. An EEG can help to differentiate the cause of neurological deficit as it would be normal in vascular ischaemia, and these cases would benefit from stroke prophylaxis in the form of aspirin 14 15.…”

“…Other features of SWS include learning difficulties (40–50%);9 of whom, almost all of them suffer with seizures6 and glaucoma (60%) 16…”

A Rare Cause of Intra Cavernous Internal Carotid Artery Dissection: Sturge Weber Syndrome

“…In normal circumstances, the vascular plexus develops at the sixth gestational week and regresses in the ninth week of gestation. Failure of regression results in residual vascular tissue, such as angiomas on face, leptomeninges and the involvement of the ipsilateral eye (6) .…”