Idiopathic rolandic epilepsy with "interictal" facial myoclonia and oromotor deficit: a longitudinal EEG and PET study.
OBJECTIVE
The prognosis of benign epilepsy with centrotemporal spikes (BECTS) is always favorable as far as the epilepsy is concerned. However, some data suggest that affected children may be at risk for minor cognitive impairment. We report here the longitudinal study of a young girl demonstrating that BECTS also may be associated with severe motor disturbances.


METHODS
BECTS (rare left oromotor seizures, right rolandic spike-waves activated during sleep) started at the age of 3 years 6 months in a girl with normal initial psychomotor development. Her clinical, neuropsychological, and EEG status was assessed every 3-6 months. Regional cerebral glucose metabolism was measured by using the [18F]fluorodeoxyglucose-positron emission tomography (FDG-PET) method.


RESULTS
Between the age of 5 and 6 years, the girl had (a) increased seizure frequency; (b) brief perioral and palpebral myoclonic jerks, concomitant with the spike component of interictal spike-waves, and (c) persistent but fluctuating oromotor deficits (drooling, dysarthria, dysphagia). The EEG showed a marked increase in abundance and amplitude of wake and sleep interictal abnormalities, which became bilateral. Awake FDG-PET revealed a bilateral increase of glucose metabolism in opercular regions. A complete and definitive EEG and clinical remission occurred at age 5 years 11 months and has persisted since (present age, 7 years 9 months).


CONCLUSIONS
This case confirms that during BECTS, epileptiform dysfunctions within rolandic areas may induce "interictal" positive or negative oromotor symptoms, independent of classic seizures.
de Saint-Martin
A
A
Service de Pédiatrie I, INSERM U 398, Faculté de Médecine, Strasbourg, France.
Petiau
C
C
Massa
R
R
Maquet
P
P
Marescaux
C
C
Hirsch
E
E
Metz-Lutz
M N
MN
D001921:Brain / Q000000981:diagnostic imaging* / Q000378:metabolism; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology; D015897:Comorbidity; D004569:Electroencephalography; D019305:Epilepsy, Rolandic / Q000175:diagnosis* / Q000000981:diagnostic imaging* / Q000453:epidemiology; D005152:Facial Muscles / Q000503:physiopathology; D005260:Female; D019788:Fluorodeoxyglucose F18; D007839:Functional Laterality / Q000502:physiology; D005947:Glucose / Q000378:metabolism; D006801:Humans; D008137:Longitudinal Studies; D009207:Myoclonus / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology; D009483:Neuropsychological Tests; D012890:Sleep / Q000502:physiology; D014055:Tomography, Emission-Computed
D019788:Fluorodeoxyglucose F18; D005947:Glucose