Diazepam-enhanced beta activity in Sturge Weber syndrome: its diagnostic significance in comparison with MRI.
OBJECTIVE
This study was performed to assess the extent of functional involvement of the affected hemisphere in Sturge Weber syndrome in comparison with the uninvolved hemisphere. To this end beta activity in the electroencephalogram (EEG) was measured, both before and after administration of diazepam intravenously (i.v.).


METHODS
In 9 patients asymmetry in beta band activity was studied before and after diazepam administration. Several beta bands and asymmetry parameters were calculated. beta band asymmetries were compared with structural abnormalities (magnetic resonance imaging, MRI).


RESULTS
Total beta activity was reduced in the involved hemisphere in all patients after diazepam administration. In 3 patients functional abnormalities were found in brain regions that were structurally intact.


CONCLUSIONS
Decreased diazepam-enhanced beta activity in the EEG is a sensitive criterion of functional abnormality. In patients with subtle structural abnormalities diazepam-enhanced EEG may have added value in diagnosing functional involvement and in monitoring disease progression in patients.
Jansen
F E
FE
Department of Child Neurology, University Medical Centre Utrecht, P.O. Box 85500, The Netherlands.
van Huffelen
A C
AC
Witkamp
ThD
T
Couperus
A
A
Teunissen
N
N
Wieneke
G H
GH
van Nieuwenhuizen
O
O
D000293:Adolescent; D000927:Anticonvulsants / Q000008:administration & dosage; D001611:Beta Rhythm / Q000187:drug effects*; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003975:Diazepam / Q000008:administration & dosage; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D007223:Infant; D007275:Injections, Intravenous; D008279:Magnetic Resonance Imaging; D008297:Male; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000473:pathology
D000927:Anticonvulsants; D003975:Diazepam