Newly Diagnosed Epilepsy in Children: Presentation at Diagnosis

Abstract

Summary:Purpose: The current understanding of epilepsy has changed significantly in the past 2 decades. This report presents a description of newly diagnosed childhood-onset epilepsy, with a special emphasis on epilepsy syndromes, in a large, prospectively ascertained community-based cohort evaluated and diagnosed in the mid-1990s.Methods: Children, aged 0 through 15 years at the time of the first seizure, were prospectively identified at the time of diagnosis of epilepsy through the practices of 16 of the 17 child neurologists in Connecticut as well as five adult neurologists and seven pediatricians from January 1993 through December 1997. Parents were interviewed, and all relevant medical records were reviewed. classification of seizures and of epilepsy syndromes was done for each child by each of three pediatric neurologists, Discrepancies were resolved in conference.Results: A total of 613 children was recruited into the study. The median age at time of the first seizure was 5.3 years. Half the cohort was boys. Eighteen percent had a remote symptomatic etiology. Epilepsy syndromes were classifiable in all but four children, although some syndromes are, by definition, relatively nonspecific. In this childhood-onset cohort, 58.6% of the syndromes were localization related, 29.0% generalized, and 12.4% undetermined as to whether focal or generalized. Benign rolandic epilepsy occurred in 10% of the cohort. Primarily generalized syndromes accounted for 20.6%, with childhood absence being the single most common syndrome in this subgroup (12.1 % of the cohort). Secondarily generalized syndromes accounted for 8.5% of the total, with infantile spasms being the most common in this grouping (3.9% of the cohort).Conclusions: This study presents a description of childhoodand adolescent-onset epilepsy as it is diagnosed and evaluated in the 1990s in one state in the United State and based on current classification guidelines. The results should be generalizable to the rest of the country. The prognostic value of early identification of epilepsy syndromes will be determined through subsequent follow-up of this cohort.

Newly Diagnosed Unprovoked Epileptic Seizures: Presentation at Diagnosis in CAROLE Study

“…The relative underrepresentation of elderly might also contribute to the lower proportion with remote symptomatic etiology, 15 vs. 21% (24% with the cases of neurologic deficit at birth) in the NGPSE (10,18). Remote symptomatic etiology increases with age, as was documented by several studies: 18% in a cohort of children with newly diagnosed epilepsy (19), 26% in a cohort of children with two or more seizures prospectively monitored from the time of their first unprovoked seizure (23), 31% in an Icelandic incidence survey (24), 35% in the Rochester study between 1935 and 1984 (25) and between 1980 and 1984 (16), 39% of the patients with unprovoked seizures in the Geneva study (26), 39% in a Finnish incidence survey (27), and 49% in a Swedish incidence survey (13), both in adults.…”

“…Of course, our results (or those of most other studies for that matter) cannot be extended to patients not coming to medical attention, and to patients not included because of a dubious follow-up. However, in France as in the United States (19), specialists' visits are not limited to tertiary centers in which an underestimation of mild cases is likely (17,20). Although a population-based approach could have been used, we note that a chief purpose of this study was to examine in detail the seizure types, syndromes, and etiology in this cohort.…”

“…It would be almost impossible to classify specific syndromes without at least an EEG. In the CAROLE study, as in recent U.S. cohorts (19,23) and in the prospective Dutch Study of Epilepsy in Childhood (33), the very high rate of ancillary tests (EEG in >99% of cases, neuroimaging in 78.2% of the first-seizure group, and 68.3% of the newly-diagnosedepilepsy group) permitted classification of seizures in 78.1% of the first-seizure group and 88.0% of the newlydiagnosed-epilepsy group; classification of syndromes in all the first seizures and 98.6% of those with newly diagnosed epilepsy; and classification of etiology in all the first seizures and 98.8% of those with newly diagnosed epilepsy, with a reasonably high degree of certainty at the time of initial diagnosis.…”

Multi-site voxel-based morphometry: Methods and a feasibility demonstration with childhood absence epilepsy

“…It was applied by at least two experienced epileptologists at all sites as described in Chan et al (2006) (Site A) and Berg et al (1999) (Site B and Site C).…”

Novel Animal Models of Pediatric Epilepsy

“…They also have become the focus of treatment trials and genetic, neuropsychological, and neuroimaging investigations, and have implications for treatment and prognosis. Two-thirds of children can be assigned to specific syndromes early after clinical evaluation and appropriate investigations (EEG and/or neuroimaging) [6,7]. Of the remaining percentage, approximately 30 % will be assigned to a more specific category within 2 years [8].…”