title: Sturge-Weber syndrome: clinical and radiological correlates in 86 patients.
abstract: OBJECTIVE
  To correlate the extent of the leptomeningeal angiomatosis with clinical features in Sturge-Weber syndrome (SWS).
  
  
  METHODS
  The study group consisted of 86 consecutive patients aged two months to 56 (mean 7.9 +/- 10.3) years with SWS and epilepsy. Clinical and MRI data were analyzed.
  
  
  RESULTS
  Based on the extent of leptomeningeal angiomatosis, patients were divided into two subgroups: 43 patients had hemispheric angiomatosis and atrophy, whereas, another 43 had focal involvement. Nine of the 43 hemispherial patients (10%) showed bilateral involvement: all of these bilateral cases demonstrated dominance in a single side with hemispheric leptomeningeal angiomatosis and contralateral focal extension. Hemispheric and focal subgroups were clinically different. Patients with hemispheric SWS were younger at the age of epilepsy onset (p < 0.001) and age at MRI examination (p < 0.05). Neither gender, lateralization, duration of epilepsy, appearance of secondarily generalized seizures, nor seizure frequency revealed a significant difference between subgroups.
  
  
  CONCLUSIONS
  Bilateral involvement is frequent and occurs in cases with a hemisperic involvement on one side. The age of epilepsy onset is related to the extent of leptomeningeal angiomatosis. Patients with hemispheric form of SWS presented with earlier age of seizure onset. Focal pial angiomatoses do not tend to progress (a longer duration is not associated with more frequent hemispheric involvement). Other variables including seizure frequency and secondary generalized tonic-clonic seizures are not associated with the extent of angiomatosis.
authors.affiliation: Epilepsy Center, Bethesda Children's Hospital, Budapest, Hungary. fogarasi@bethesda.hu
authors.forename: András
authors.initials: A
authors.lastname: Fogarasi
authors.forename: Tobias
authors.initials: T
authors.lastname: Loddenkemper
authors.forename: Cecilia
authors.initials: C
authors.lastname: Mellado
authors.forename: Ingrid
authors.initials: I
authors.lastname: Tuxhorn
authors.forename: Georg
authors.initials: G
authors.lastname: Evers
authors.forename: Dean
authors.initials: D
authors.lastname: Sarco
authors.forename: Richard C
authors.initials: RC
authors.lastname: Burgess
authors.forename: Péter
authors.initials: P
authors.lastname: Halász
authors.forename: Péter
authors.initials: P
authors.lastname: Barsis
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Gyorsok
authors.forename: Csilla
authors.initials: C
authors.lastname: Gyimesi
authors.forename: Jeno
authors.initials: J
authors.lastname: Kóbor
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Siegler
authors.forename: József
authors.initials: J
authors.lastname: Janszky
authors.forename: Rita
authors.initials: R
authors.lastname: Jakus
authors.forename: György
authors.initials: G
authors.lastname: Rásonyi
authors.forename: Alois
authors.initials: A
authors.lastname: Ebner
authors.forename: Friedrich G
authors.initials: FG
authors.lastname: Woermann
authors.forename: Mustafa
authors.initials: M
authors.lastname: Sahin
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000798:Angiomatosis / Q000175:diagnosis*; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012640:Seizures / Q000209:etiology*; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology