부산대학교 도서관

Background: Sturge-Weber syndrome (SWS) is an uncommon etiology of hemiplegic migraine-like (HM-like) attacks,associated with epilepsy and mental retardation.Case:We report the case of a 40-year-old woman with SWS who has been suffering from HM-like episodes since she was24, with no history of seizure or mental retardation. Susceptibility weighted imaging (SWI)-MRI and CT scans haveshown bilateral calcifications of the choroidal plexuses, a developmental venous anomaly with dilated transmedullaryveins and a left parieto-occipital leptomeningeal angioma. 18F-Fluorodeoxyglucose (FDG)-PET/CT revealed a diffuse lefthemispherehypometabolism. The comparison between the MRI performed at the age of 24 and the one performed atthe age of 40 highlighted a progressive unilateral fronto-temporo-parietal atrophy. Surprisingly, even now, cognitivefunctions of this patient are relatively preserved. Lamotrigine permitted an improvement of HM-like attacks.Discussion: Explanations for this minimally symptomatic form of SWS may be the absence of seizure, the importance ofher deep venous drainage, the absence of cortical calcification and white matter impairment in the affected hemisphere,and, paradoxically, the severely asymmetric cortical metabolism. Furthermore, this case reinforces the hypothesis thatalteration of cerebral hemodynamics could precipitate the cortical spreading depression giving rise to migraine with aura.Conclusion: We propose to consider SWS as a cause of apparently isolated hemiplegic migraine and lamotrigine as apreventive medication in HM-like attacks. [ABSTRACT FROM AUTHOR]