EEG-fMRI in myoclonic astatic epilepsy (Doose syndrome).
OBJECTIVE
To identify neuronal networks underlying generalized spike and wave discharges (GSW) in myoclonic astatic epilepsy (MAE).


METHODS
Simultaneous EEG-fMRI recordings were performed in 13 children with MAE. Individual GSW-associated blood oxygenation level-dependent (BOLD) signal changes were analyzed in every patient. A group analysis was performed to determine common syndrome-specific hemodynamic changes across all patients.


RESULTS
GSW were recorded in 11 patients, all showing GSW-associated BOLD signal changes. Activation was detected in the thalamus (all patients), premotor cortex (6 patients), and putamen (6 patients). Deactivation was found in the default mode areas (7 patients). The group analysis confirmed activations in the thalamus, premotor cortex, putamen, and cerebellum and deactivations in the default mode network.


CONCLUSIONS
In addition to the thalamocortical network, which is commonly found in idiopathic generalized epilepsies, GSW in patients with MAE are characterized by BOLD signal changes in brain structures associated with motor function. The results are in line with animal studies demonstrating that somatosensory cortex, putamen, and cerebellum are involved in the generation of myoclonic seizures. The involvement of these structures might predispose to the typical seizure semiology of myoclonic jerks observed in MAE.
From the Department of Neuropediatrics (F.M., K.G., J.M., H.M., U.S.) and Institute of Neuroradiology (S.W., O.J.), Christian-Albrechts-University, Kiel; and University Hospital of Child and Adolescent Psychiatry (M.S.), Johann Wolfgang Goethe-University of Frankfurt, Germany.
Friederike
F
Moeller
Kristina
K
Groening
Jan
J
Moehring
Hiltrud
H
Muhle
Stephan
S
Wolff
Olav
O
Jansen
Ulrich
U
Stephani
Michael
M
Siniatchkin
D000293:Adolescent; D001921:Brain / Q000098:blood supply* / Q000473:pathology; D058256:Brain Waves / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D010100:Oxygen / Q000097:blood
D010100:Oxygen