A Verrotti1, D Laino1, V E Rinaldi1, A Suppiej2, L Giordano3, I Toldo2, L Margari4, P Parisi5, R Rizzo6, S Matricardi7,8, R Cusmai9, S Grosso10, R Gaggero11, N Zamponi12, P Pavone13, G Capovilla14, M Rauchenzauner15, C Cerminara16, G Di Gennaro17, M Esposito18, P Striano19, S Savasta20, G Coppola21, S Siliquini12, F Operto4, V Belcastro22, F Ragona8, G L Marseglia20, A Spalice23. 1. Department of Paediatrics, University of Perugia, Perugia, Italy. 2. Paediatric Neurology Unit, Department of Women's and Children's Health, University of Padova, Padova, Italy. 3. Paediatric Neuropsychiatric Division, Spedali Civili, Brescia, Italy. 4. Unit of Child Neuropsychiatry, Department of Basic Medical Sciences, Neuroscience and Sense Organs, 'Aldo Moro' University of Bari, Bari, Italy. 5. NESMOS Department, Chair of Paediatrics, Paediatric Headache Centre, Paediatric Sleep Centre and Child Neurology, Faculty of Medicine and Psychology, Sant'Andrea Hospital, 'Sapienza University', Roma, Italy. 6. Section of Child Neuropsychiatry, Department of Paediatrics, University of Catania, Catania, Italy. 7. Departement of Paediatrics, University of Chieti, Chieti, Italy. 8. Department of Paediatric Neuroscience, Foundation IRCCS Neurological Institute 'C. Besta', Milano, Italy. 9. Neurology Unit, 'Bambino Gesù' Children's Hospital, IRCCS, Roma, Italy. 10. Paediatric Neurology-Immunology and Endocrinology Unit, University of Siena, Siena, Italy. 11. Paediatric Unit, San Paolo Hospital, Savona, Italy. 12. Child Neuropsychiatry Unit, Polytechnic University of the Marche, Ancona, Italy. 13. Unit of Paediatrics and Paediatric Emergency 'Costanza Gravina', University-Hospital 'Vittorio Emanuele, Policlinic', University of Catania, Catania, Italy. 14. Department of Child Neuropsychiatry, Epilepsy Centre, C. Poma Hospital, Mantova, Italy. 15. Department of Paediatrics, Medical University Innsbruck, Innsbruck, Austria. 16. Paediatric Neurology, Department of Neurosciences, Tor Vergata University of Rome, Roma, Italy. 17. IRCCS 'NEUROMED', Pozzilli, Italy. 18. Department of Mental Health, Physical and Preventive Medicine, Clinic of Child and Adolescent Neuropsychiatry, Centre for Childhood Headache, Second University of Naples, Napoli, Italy. 19. Paediatric Neurology and Muscular Diseases Unit, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, 'G. Gaslini' Institute, Genova, Italy. 20. Department of Paediatrics, Pavia University Foundation, IRCCS Policlinico San Matteo, Pavia, Italy. 21. Child and Adolescent Neuropsychiatry, Medical School, University of Salerno, Salerno, Italy. 22. Neurology Unit, Department of Neuroscience, 'Sant'Anna' Hospital, Como, Italy. 23. Child Neurology, Chair of Paediatrics, II Faculty of Medicine, University of Rome, Roma, Italy.
Abstract
BACKGROUND AND PURPOSE: Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes. METHODS: This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.1-23.0 years. The following clinical characteristics were evaluated: gender, patient age at seizure onset, history of febrile seizures and migraine, family history of epilepsy, duration and seizure manifestations, circadian distribution and frequency of seizures, history of medications including the number of drugs, therapeutic response and final outcome. RESULTS: Visual hallucinations were the first symptom in 62% and the only manifestation in 38.8% of patients. Patients were subdivided into two groups: group A with isolated COE-G; group B with other epileptic syndromes associated with COE-G. The most significant (P < 0.05) difference concerned antiepileptic therapy: in group A, 45 children responded to monotherapy; in group B only 15 children responded to monotherapy. At the end of follow-up, the percentage of seizure-free patients was significantly higher in group A than in group B. CONCLUSIONS: Childhood occipital epilepsy of Gastaut has an overall favourable prognosis and a good response to antiepileptic therapy with resolution of seizures and of electroencephalogram abnormalities. The association of typical COE-G symptoms with other types of seizure could be related to a poor epilepsy outcome.
BACKGROUND AND PURPOSE: Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes. METHODS: This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.1-23.0 years. The following clinical characteristics were evaluated: gender, patient age at seizure onset, history of febrile seizures and migraine, family history of epilepsy, duration and seizure manifestations, circadian distribution and frequency of seizures, history of medications including the number of drugs, therapeutic response and final outcome. RESULTS:Visual hallucinations were the first symptom in 62% and the only manifestation in 38.8% of patients. Patients were subdivided into two groups: group A with isolated COE-G; group B with other epileptic syndromes associated with COE-G. The most significant (P < 0.05) difference concerned antiepileptic therapy: in group A, 45 children responded to monotherapy; in group B only 15 children responded to monotherapy. At the end of follow-up, the percentage of seizure-freepatients was significantly higher in group A than in group B. CONCLUSIONS: Childhood occipital epilepsy of Gastaut has an overall favourable prognosis and a good response to antiepileptic therapy with resolution of seizures and of electroencephalogram abnormalities. The association of typical COE-G symptoms with other types of seizure could be related to a poor epilepsy outcome.