Absence epilepsy with onset before age three years: a heterogeneous and often severe condition.
OBJECTIVE
The classification of epilepsies and epileptic syndromes recognizes three syndromes with typical absences [TA, i.e., childhood and juvenile absence epilepsies (CAE and JAE), and epilepsy with myoclonic absences (EMA), none of which is characterized by onset in early childhood]. Although several other forms of absence epilepsies have been described recently, none concerns infants and very young children, and little is known about the nosology and prognosis of early-onset absences.


METHODS
We retrospectively selected all cases with onset of absences as the only or major seizure type before age 3 years and >/=2 years of follow-up among cases newly referred between 1986 and 2002. Neuropsychological assessments (generally IQ measure), behavior patterns, and schooling situations were reviewed for each child.


RESULTS
We found 10 patients (7 F, 3 M). No child had sensory or motor deficits: neuroimaging was performed in nine and was normal in eight, with aspecific findings in one. Only two could be characterized as CAE and EMA, respectively, both with seizure control and a good cognitive outcome. Among the remaining eight cases, four had a fairly homogeneous presentation with predominantly brief absences and clearly asymmetric interictal EEGs. All eight had neuropsychological and/or behavioral difficulties. Three had full seizure control, and five, persisting absences, with a follow-up ranging between 2 years 8 months to 9 years 4 months; only one child was older than 12 years.


CONCLUSIONS
Great heterogeneity exists among absence epilepsies of early onset, which are rare conditions. Only a few patients can be categorized into well-known syndromes. The overall prognosis is poor. Early onset of absences is uncommon, and multicenter studies should help clarify the nosology and prognosis.
Chaix
Yves
Y
Centre Saint-Paul, Hôpital Henri Gastaut, Marseille, France.
Daquin
Géraldine
G
Monteiro
Franklin
F
Villeneuve
Nathalie
N
Laguitton
Virginie
V
Genton
Pierre
P
D000293:Adolescent; D000367:Age Factors; D000927:Anticonvulsants / Q000627:therapeutic use; D001925:Brain Damage, Chronic / Q000175:diagnosis / Q000503:physiopathology / Q000523:psychology; D002540:Cerebral Cortex / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004292:Dominance, Cerebral / Q000502:physiology; D004359:Drug Therapy, Combination; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
D000927:Anticonvulsants