Magnetoencephalography reveals a unique neurophysiological profile of focal-onset epileptic spasms.
Epilepsy is defined as a disorder of the brain characterized by an enduring predisposition to experience epileptic seizures and the neurobiological, cognitive, psychological, and social difficulties relating to the condition. An epileptic spasm (ES) is a type of seizure characterized by clusters of short contractions involving axial muscles and proximal segments. However, the precise mechanism of ESs remains unknown. Despite the potential of magnetoencephalography (MEG) as a tool for investigating the neurophysiological mechanism of ESs, it has been difficult to use this methodology due to magnetic artifacts attributable to patient movement. We report on an 8-year-old girl suffering from intractable epileptic spasms from the age of 7 months. She was diagnosed with possible Aicardi syndrome [corrected] (AGS), characterized by the triad of callosal agenesis, infantile spasms, and chorioretinal lacunae. She is now intellectually delayed and suffers from intractable ES. We used both MEG and electroencephalography to investigate her epilepsy. The recording captured two series of spasm clusters. Spikes were clearly identified with MEG in about four-fifths of all spasms but were identified poorly or not at all in the remainder. MEG findings support previous studies that used intracranial electrodes to analyze patients with ESs and that showed variability in ES-associated spikes in terms of manner of cortical involvement and magnitude. Given the limitations of intracranial electrodes, such as sampling restrictions and invasiveness, MEG may be a helpful tool for non-invasively investigating the unique pathophysiological profile of focal-onset ESs.
Epilepsy Center, Neurological Institute Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA. kakisuke@mui.biglobe.ne.jp
Yosuke
Y
Kakisaka
Ajay
A
Gupta
Rei
R
Enatsu
Zhong I
ZI
Wang
Andreas V
AV
Alexopoulos
John C
JC
Mosher
Anne-Sophie
AS
Dubarry
Naomi
N
Hino-Fukuyo
Richard C
RC
Burgess
D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis / Q000503:physiopathology; D002648:Child; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography / Q000379:methods*; D009421:Nervous System Malformations / Q000175:diagnosis / Q000503:physiopathology; D009434:Neural Pathways / Q000503:physiopathology