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Abstract
Background: Benign non-familial neonatal seizures (BNFNS) represent a recognized epileptic syndrome that is heterogeneous in its presentation. The objective of this study was to analyze age of onset, electroclinical characteristics, treatment, and progress of patients diagnosed with BNFNS.
Methods: Retrospective study of 40 cases that met inclusion criteria for BNFNS from July 1996 to February 2016.
Results: 40 patients (21 female, 19 male) were examined, with a follow-up of 1 year and 10 months up to 16 years of age. Age of onset: 1–7 days, 30/40 (75%); 8–20 days, 10/40 (25%); mean: 6 days; median: 3.5 days. All patients presented focal clonic seizures (unifocal or alternating; isolated or clustered), and four of them evolved into status epilepticus. Interictal electroencephalogram (EEG) was normal in 37 cases; three showed sharp focal or multifocal waves. An ictal EEG was performed in a patient and initial spike-wave activity was noted in temporal area, which quickly spread to the homologous hemisphere. Phenobarbital was the initial drug in all infants; 10/40 cases required combination with carbamazepine. In their evolution, two patients with a history of status presented intellectual disability; seven children had afebrile seizures in the first year of life; and two patients evolved into focal epilepsy in childhood. Of all the patients, 95% are seizure-free at the moment and do not receive antiepileptic treatment.
Conclusions: In infants with clonic focal seizures, with no history of relevance and normal complementary tests, BNFNS should be considered. Although the majority of . patients evolved favorably, some presented epilepsy and intellectual disability during their follow-up.
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References
- References:
- Volpe JJ. Neonatal Seizures. In: Volpe JJ (ed.), Neurology of the Newborn. Philadelphia: WB Saunders, 1995; pp.172–207.
- Mizrahi EM, Kellaway P. Characterization and classification of neonatal seizures. Neurology 1987;37:1837–44.
- Patrick J, Elia M, Engel J, Guerrini R, Mizrahi E, Moshe S, Plouin P. Proposal of an algorithm for diagnosis and treatment of neonatal seizures in developing countries. Epilepsia 2007;48(6):1158–64.
- Berg AT, Berkovich SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010;51:676–85.
- Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001;42:796–803.
- Engel J Jr. Report of the ILAE Classification Core Group. Epilepsia 2006;47(9):1558–68.
- Singh NA, Charlier C, Stauffer D, DuPont BR, Leach RJ, Melis R, et al. A novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns. Nat Genet 1998;18: 23–9.
- Charlier C, Singh NA, Ryan SG, Lewis TB, Reus BE, Leach RJ, Leppert M. A pore mutation in a novel KQT-like potassium channel gene in an idiopathic epilepsy family. Nat Genet 1998;18:53–5.
- Kapland RE, Lacey DJ. Benign familial neonatal-infantile seizures. Am J Med Genet 1983;16:595–9.
- Heron SE, Crossland KM, Andermann E, Phillips HA, Hall AJ, Bleasel A, et al. Sodium-channel defects in benign familial neonatal-infantile seizures. The Lancet 2002;360:851–2.
- Berkovic SF, Heron SE, Giordano L, Marini C, Guerrini R, Kaplan RE, el al. Benign familial neonatal-infantile seizures: characterization of a new sodium channelopathy. Ann Neurol 2004;55:550–7.
- Dehan M, Quilleron D, Navelet Y, D´Allest AM, Vial M, Retbi JM, et al. Convulsions in the fifth day of life: a new síndrome? Arch FrPed 1977;34:730–42.
- Plouin P, Neubauer B. Benign familial and non-familial neonatal seizures. In: In: Bureau M, Genton P, Dravet C, Delgado-Escueta A, Tassinari CA, Thomas P, Wolf P, (eds.), Epileptic Syndromes in Infancy, Childhood and Adolescence, 5th edition. Montrouge, France: John Libbey Eurotext 2012; pp. 77–88.
- Hoffmann M, de la Vega M, López R, Travaini V, Stengurt M, del Barco M, Espeche A. Incidencia de convulsiones neonatales en una Unidad Neonatal del Noroeste Argentino. Rev SANI 2014;5(6):21–8.
- North N, Storey GNB, Handerson-Smart DJ. Fith day fits in the newborn. Aust Paediatr J 1989;25:284–7.
- André M, Vert P, Bouchez T. Á propos des convulsions du cinquiéme jour. Arch Fr Ped 1978;35:922–3.
- Plouin P, Sternberg B, Bour F, Lerique A. États de mal néonatauxd´étiologieindéterminée. Rev EEG Neurophysiol 1981;11:385–9.
- Pryor Ds, Don N, Macourt DC. Fifth day fits: a syndrome of neonatal convulsions. Arch Dis Child 1981;56:753–8.
- Navelet Y, D´Allest AM, Dehan M, Gabilan JC. Á propos du syndrome des convulsions néonatales du cinquiéme jour. Rev EEG Neurphysiol 1981; 11: 390–96.
- Panayotopulos CP. Neonatal seizures and neonatal syndromes. In: Panayiotopoulos CP (ed.), The Epilepsies: seizures, syndromes and management. Oxford: Bladon Medical Publshing. 2005; pp.87–115.
- Dehan M, Navelet Y, D´Allest AM, Vial M, Ropert JC, Boulley AM, Gabilan JC. Quelquesprecisions sur le syndrome des convulsionsdu cinquiémejour de vie. Arch FrPed 1982;39:405–7.
- Ronen G, Rosales TO, Connolly M, Anderson VE, Leppert M. Seizure characteristics in chromosome 20 benign familial neonatal convulsions. Neurology 1993;43:1355–60.
- Vidaurre JA, Ballaban-Gil KR, Pouin P, Moshe SL. Benign Neonatal Familial Seizures. In: Gilman S (ed.), Medlink Neurology. San Diego SA: Arbor Publishing Corp, 2004, 81.
- Rett AR, Teubel R. Neugeborenenkrampfe im Rahmen einer epilepisch belasten Familie. Wien Lin Wschr 1964;76:609–13
- Grinton BE, Heron SE, Pelekanos JT, Zuberi SM, Kivity S, Afawi Z, et al. Familial neonatal seizures in 36 families: Clinical and genetic features correlate with outcome. Epilepsia 2015;56(7):1071–80.
- Specchio N, Terracciano A, Trivisano M, Cappelletti S, Claps D, Travaglini L, Bertini E. PRRT2 is mutated in familial and non-familial benign infantile seizures. Eur J Paediatr Neurol 2013;17(1):77–81.
- Goldberg HJ, Sheehy EM. Fifth day fits: an acute zinc deficiency syndrome? Arch Dis Child 1983;57:633–5.
- Hermrmann B, Lawrenz-Wolf B, Seewald C, Selb B, Wehinger H. 5-tages-krämpfe des neugeborenenbei Rota Virus infektionen. Monatsschr Kinderheilkd 1993;141:120–3.
- Rao S, Lewis B, Ghosh S, Nagarajan L. Clinical approach to a neonate with seizures. In: Nagarajan L (ed.), Neonatal seizures: Current management and future challenges. London: Mac Keith Press 2016; pp,114–127.
- Caraballo R. Convulsiones familiares y no familiares benignas del lactante. In: Ruggieri V, Caraballo R, Arroyo H (eds.), Temas de Neuropediatría. Buenos Aires, Argentina: Editorial Panamericana 2004, pp.53–58.
- Nagarajan L (ed.). Neonatal seizures: Current management and future challenges. London: Mac Keith Press, 2016.
References
References:
Volpe JJ. Neonatal Seizures. In: Volpe JJ (ed.), Neurology of the Newborn. Philadelphia: WB Saunders, 1995; pp.172–207.
Mizrahi EM, Kellaway P. Characterization and classification of neonatal seizures. Neurology 1987;37:1837–44.
Patrick J, Elia M, Engel J, Guerrini R, Mizrahi E, Moshe S, Plouin P. Proposal of an algorithm for diagnosis and treatment of neonatal seizures in developing countries. Epilepsia 2007;48(6):1158–64.
Berg AT, Berkovich SF, Brodie MJ, Buchhalter J, Cross JH, van Emde Boas W, et al. Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia 2010;51:676–85.
Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001;42:796–803.
Engel J Jr. Report of the ILAE Classification Core Group. Epilepsia 2006;47(9):1558–68.
Singh NA, Charlier C, Stauffer D, DuPont BR, Leach RJ, Melis R, et al. A novel potassium channel gene, KCNQ2, is mutated in an inherited epilepsy of newborns. Nat Genet 1998;18: 23–9.
Charlier C, Singh NA, Ryan SG, Lewis TB, Reus BE, Leach RJ, Leppert M. A pore mutation in a novel KQT-like potassium channel gene in an idiopathic epilepsy family. Nat Genet 1998;18:53–5.
Kapland RE, Lacey DJ. Benign familial neonatal-infantile seizures. Am J Med Genet 1983;16:595–9.
Heron SE, Crossland KM, Andermann E, Phillips HA, Hall AJ, Bleasel A, et al. Sodium-channel defects in benign familial neonatal-infantile seizures. The Lancet 2002;360:851–2.
Berkovic SF, Heron SE, Giordano L, Marini C, Guerrini R, Kaplan RE, el al. Benign familial neonatal-infantile seizures: characterization of a new sodium channelopathy. Ann Neurol 2004;55:550–7.
Dehan M, Quilleron D, Navelet Y, D´Allest AM, Vial M, Retbi JM, et al. Convulsions in the fifth day of life: a new síndrome? Arch FrPed 1977;34:730–42.
Plouin P, Neubauer B. Benign familial and non-familial neonatal seizures. In: In: Bureau M, Genton P, Dravet C, Delgado-Escueta A, Tassinari CA, Thomas P, Wolf P, (eds.), Epileptic Syndromes in Infancy, Childhood and Adolescence, 5th edition. Montrouge, France: John Libbey Eurotext 2012; pp. 77–88.
Hoffmann M, de la Vega M, López R, Travaini V, Stengurt M, del Barco M, Espeche A. Incidencia de convulsiones neonatales en una Unidad Neonatal del Noroeste Argentino. Rev SANI 2014;5(6):21–8.
North N, Storey GNB, Handerson-Smart DJ. Fith day fits in the newborn. Aust Paediatr J 1989;25:284–7.
André M, Vert P, Bouchez T. Á propos des convulsions du cinquiéme jour. Arch Fr Ped 1978;35:922–3.
Plouin P, Sternberg B, Bour F, Lerique A. États de mal néonatauxd´étiologieindéterminée. Rev EEG Neurophysiol 1981;11:385–9.
Pryor Ds, Don N, Macourt DC. Fifth day fits: a syndrome of neonatal convulsions. Arch Dis Child 1981;56:753–8.
Navelet Y, D´Allest AM, Dehan M, Gabilan JC. Á propos du syndrome des convulsions néonatales du cinquiéme jour. Rev EEG Neurphysiol 1981; 11: 390–96.
Panayotopulos CP. Neonatal seizures and neonatal syndromes. In: Panayiotopoulos CP (ed.), The Epilepsies: seizures, syndromes and management. Oxford: Bladon Medical Publshing. 2005; pp.87–115.
Dehan M, Navelet Y, D´Allest AM, Vial M, Ropert JC, Boulley AM, Gabilan JC. Quelquesprecisions sur le syndrome des convulsionsdu cinquiémejour de vie. Arch FrPed 1982;39:405–7.
Ronen G, Rosales TO, Connolly M, Anderson VE, Leppert M. Seizure characteristics in chromosome 20 benign familial neonatal convulsions. Neurology 1993;43:1355–60.
Vidaurre JA, Ballaban-Gil KR, Pouin P, Moshe SL. Benign Neonatal Familial Seizures. In: Gilman S (ed.), Medlink Neurology. San Diego SA: Arbor Publishing Corp, 2004, 81.
Rett AR, Teubel R. Neugeborenenkrampfe im Rahmen einer epilepisch belasten Familie. Wien Lin Wschr 1964;76:609–13
Grinton BE, Heron SE, Pelekanos JT, Zuberi SM, Kivity S, Afawi Z, et al. Familial neonatal seizures in 36 families: Clinical and genetic features correlate with outcome. Epilepsia 2015;56(7):1071–80.
Specchio N, Terracciano A, Trivisano M, Cappelletti S, Claps D, Travaglini L, Bertini E. PRRT2 is mutated in familial and non-familial benign infantile seizures. Eur J Paediatr Neurol 2013;17(1):77–81.
Goldberg HJ, Sheehy EM. Fifth day fits: an acute zinc deficiency syndrome? Arch Dis Child 1983;57:633–5.
Hermrmann B, Lawrenz-Wolf B, Seewald C, Selb B, Wehinger H. 5-tages-krämpfe des neugeborenenbei Rota Virus infektionen. Monatsschr Kinderheilkd 1993;141:120–3.
Rao S, Lewis B, Ghosh S, Nagarajan L. Clinical approach to a neonate with seizures. In: Nagarajan L (ed.), Neonatal seizures: Current management and future challenges. London: Mac Keith Press 2016; pp,114–127.
Caraballo R. Convulsiones familiares y no familiares benignas del lactante. In: Ruggieri V, Caraballo R, Arroyo H (eds.), Temas de Neuropediatría. Buenos Aires, Argentina: Editorial Panamericana 2004, pp.53–58.
Nagarajan L (ed.). Neonatal seizures: Current management and future challenges. London: Mac Keith Press, 2016.