title: A case of Sturge-Weber syndrome with symptomatic hemiplegic migraine: clinical and multimodality imaging data during a prolonged attack.
abstract: Hemiplegic migraine (HM) in the setting of Sturge-Weber syndrome (SWS) has been previously described. Here, we report clinical and multimodal imaging data on a 21-year-old man with SWS and HM, who presented during an acute HM attack with a dense left-hemispheric syndrome (expressive aphasia and right sensorimotor hemiplegia), lasting for more than 10 days. Repeated EEGs were without evidence of status epilepticus. Consistent with previous findings in prolonged migraine aura, perfusion computed tomography demonstrated left-hemispheric hyperperfusion on day 7. 18F-FDG positron emission tomography (day 7) revealed left-hemispheric hypermetabolism. After 14 days, the patient was symptom-free and discharged home. Follow-up after 30 days showed normal neurological status. Our observation confirms and reinforces the comorbidity of SWS and HM and shows that prolonged HM attacks are associated with complex changes of both cerebral perfusion and glucose metabolism. A pathophysiological model explaining both the association between SWS/HM and the observed imaging changes is presented.
authors.affiliation: Department of Neurology, Ludwig-Maximilians-University, Munich, Germany. tobias.freilinger@med.uni-muenchen.de
authors.forename: Tobias
authors.initials: T
authors.lastname: Freilinger
authors.forename: Nils
authors.initials: N
authors.lastname: Peters
authors.forename: Jan
authors.initials: J
authors.lastname: Rémi
authors.forename: Jennifer
authors.initials: J
authors.lastname: Linn
authors.forename: Marcus
authors.initials: M
authors.lastname: Hacker
authors.forename: Andreas
authors.initials: A
authors.lastname: Straube
authors.forename: Thomas
authors.initials: T
authors.lastname: Pfefferkorn
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism* / Q000503:physiopathology; D002560:Cerebrovascular Circulation / Q000502:physiology; D015897:Comorbidity; D003937:Diagnosis, Differential; D004734:Energy Metabolism / Q000502:physiology; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism; D006801:Humans; D008297:Male; D020325:Migraine with Aura / Q000150:complications* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008959:Models, Neurological; D049268:Positron-Emission Tomography; D013341:Sturge-Weber Syndrome / Q000150:complications* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D019788:Fluorodeoxyglucose F18; D005947:Glucose