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From Northern Illinois University, Department of Biological Sciences (Dr. Berg), DeKalb, IL; Montefiore Medical Center, Albert Einstein College of Medicine, Departments of Neurology, and Pediatrics, and the Comprehensive Epilepsy Management Center (Dr. Shinnar), Bronx, NY; and Yale University, Departments of Pediatrics (Drs. Levy, Testa, Smith-Rapaport, and Beckerman) and Neurology (Drs. Levy and Testa), New Haven, CT.
Address correspondence and reprint requests to Dr. Anne T. Berg, Department of Biological Sciences, Northern Illinois University, DeKalb, IL 60115; e-mail: atberg{at}niu.edu
Background: Little is known about early prediction of intractable epilepsy (IE) in children. Such information could help guide the early use of new therapies in selected patients. Methods: Children with newly diagnosed epilepsy (n = 613) were prospectively identified from child neurology practices in Connecticut (1993–1997) and followed-up for the occurrence of IE (failure of >2 drugs, >1 seizure/month, over 18 months). Etiology and epilepsy syndromes were classified per International League Against Epilepsy guidelines. Results: The median follow-up is 4.8 years, and 599 (97.7%) have been followed for more than 18 months. Sixty children (10.0%) have met the criteria for IE, including 34.6% with cryptogenic/symptomatic generalized, 2.7% with idiopathic, 10.7% with other localization–related, and 8.2% with unclassified epilepsy (p < 0.0001). After multivariable adjustment for epilepsy syndrome, initial seizure frequency (p < 0.0001), focal EEG slowing (p = 0.02), and acute symptomatic or neonatal status epilepticus (p = 0.001) were associated with an increased risk of IE, and age at onset between 5 and 9 years was associated with a lowered risk (p = 0.03). The absolute number of seizures and unprovoked or febrile status epilepticus were not associated substantially with IE. Conclusions: Approximately 10% of children meet criteria for IE early in the course of their epilepsy. Cryptogenic/symptomatic generalized syndromes carry the highest risk and idiopathic syndromes the lowest. Half of IE occurs in children with nonidiopathic localization-related syndromes. Initial seizure frequency is highly predictive of IE. By contrast, absolute number of seizures and unprovoked or febrile status epilepticus are not.
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