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.