Early-Onset Form of Benign Childhood Epilepsy with Centro-Temporal EEG Foci - A Different Nosological Perspective from Panayiotopoulos Syndrome

Abstract

Purpose: We have studied the clinical differences between early-onset benign epilepsy with centro-temporal spikes (early-onset BECT) and Panayiotopoulos syndrome (PS) to investigate the hypothesis that BECT and PS nosologically constitute age-dependent benign childhood seizure susceptibility syndromes based on a common etiopathogenesis.

Subjects and Methods: The subjects were 24 patients with BECT and 62 patients with PS, who satisfied the following definitions: 1) onset of epilepsy before 5 years of age; 2) the BECT and PS seizures started mainly with orofacial focal motor attacks and emetic symptoms followed by focal seizures, respectively; 3) follow-up examinations for longer than 2 years. We compared the various clinical features between these two groups.

Results: In children with early-onset BECT, the seizures at times manifested with hypersalivation, vomiting, and focal motor seizures, but the vomiting that developed in the middle of seizures was different from the initial vomiting observed in patients with PS. Although the seizures recurred more frequently in patients with early-onset BECT, the incidence of status epilepticus as well as prolonged seizures was higher in those with PS. The patients demonstrating below borderline IQ scores and mild developmental behavioral disorders were more frequently seen in early-onset BECT than PS, accounting for 37.5 and 14.6% (P<0.05), and for 8% and 21%, respectively (P<0.05).

Discussion: Early-onset BECT and PS have heterogeneous clinical characteristics, except for the same onset age, and appear to be nosologically different epileptic syndromes. The former seems to develop in combination with other acquired disturbances based on a BECT predisposition, while the latter develops based on a PS predisposition and involves a better prognosis.

Key words

early-onset BECT - Rolandic EEG foci - Panayiotopoulos syndrome - orofacial motor seizures - autonomic seizures - idiopathic focal epilepsy

References

• 1 Ambrosetto G, Rossi PG, Tassinari CA. Predictive factors of seizure frequency and duration of antiepileptic treatment in Rolandic epilepsy: a retrospective study.  Brain Dev. 1987;  9 300-304
• 2 Bali B, Kull LL, Strug LJ, Clarke T, Murphy PL, Akman CI, Greenberg DA, Pal DK. Autosomal dominant inheritance of centrotemporal sharp waves in rolandic epilepsy families.  Epilepsia. 2007;  48 2266-2272
• 3 Beassart M. Benign epilepsy of children with rolandic (centro-temporal) paroxysmal foci: A clinical entity. Study of 221 cases.  Epilepsia. 1972;  13 795-811
• 4 Bouma PA, Bovenkerk AC, Westendorp RG, Brouwer OF. The course of benign partial epilepsy with centrotemporal spikes: a meta-analysis.  Neurology. 1997;  48 430-437
• 5 Caraballo R, Cersosimo R, Medina C, Fejerman N. Panayiotopoulos-type benign childhood occipital epilepsy. A prospective study.  Neurology. 2000;  55 1096-1100
• 6 Cavazzuti JB. Epidemiology of different types of epilepsy in school age children of Modena (Italy).  Epilepsia. 1980;  21 57-63
• 7 Covanis A, Lada C, Skiadas K. Children with Rolandic spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome?.  Epileptic Disord. 2003;  5 139-143
• 8 Covanis A, Ferrie CD, Koutroumanidis M, Oguni H, Panayotopoulos CP. Panayiotopoulos syndrome and Gastaut type idiopathic childhood occipital epilepsy. In: Roger J, Bureau M, Dravet C, Genton P, Tassinary CA, Wolf P (Eds). Epileptic syndromes in infancy, childhood and adolescence. 4th edn. London: John Libbey 2005: 227-253
• 9 Datta A, Sinclair DB. Benign epilepsy of childhood with rolan-dic spikes: typical and atypical variants.  Pediatr Neurol. 2007;  36 141-145
• 10 Doose H, Baier WK. Benign partial epilepsy and related conditions: multifactorial pathogenesis with hereditary impairment of brain maturation.  Eur J Pediatr. 1989;  149 152-158
• 11 Doose H, Brigger-Heuer B, Neubauer B. Children with focal sharp waves: clinical and genetic aspects.  Epilepsia. 1997;  38 788-796
• 12 Ferrie CD, Beaumanoir A, Guerrini R, Kivity S, Vigevano F, Takaishi Y. et al . Early-onset benign occipital seizures susceptibility syndrome.  Epilepsia. 1997;  38 285-293
• 13 Gunduz E, Demirblek V, Korkmaz B. Benign rolandic epilepsy: neuropsychological findings.  Seizure. 1997;  8 246-249
• 14 Holtmann M, Becker K, Kentner-Figura B, Schmidt MH. Increased frequency of rolandic spikes in ADHD children.  Epilepsia. 2003;  44 1241-1244
• 15 Kanazawa O, Tohyama J, Akasawa N, Kamimura T. A magnetoencephalographic study of patients with Panayiotopoulos syndrome.  Epilepsia. 2005;  46 1106-1113
• 16 Lada C, Skidas K, Theodorou V, Covanis A. A study of 43 patients with Panayiotopoulos syndrome: A common and benign childhood seizure susceptibility.  Epilepsia. 2003;  44 81-88
• 17 Loisseau P, Beaussart M. The seizures of benign childhood epilepsy with rolandic paroxysmal discharges.  Epilepsia. 1973;  14 381-389
• 18 Loiseau P, Duche B, Cordova S, Dartigues JF, Cohadon S. Prognosis of benign childhood epilepsy with centrotemporal spikes: a follow-up study of 168 patients.  Epilepsia. 1988;  29 229-235
• 19 Mets-Luts MN, Kleitz C, Saint Martin A de, Massa R, Hirsch E, Marescaux C. Cognitive development in benign focal epilepsies of childhood.  Dev Neurosci. 1999;  21 182-190
• 20 Oguni H, Hayashi K, Imai K, Hirano Y, Mutoh A, Osawa M. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome.  Epilepsia. 1999;  40 1020-1030
• 21 Oguni H. Panayotopoulos syndrome.  Lancet. 2001;  358 69
• 22 Oguni H, Hayashi K, Funatsuka M, Osawa M. Study on early-onset occipital seizure susceptibility syndrome.  Pediatr Neurol. 2001;  25 312-318
• 23 Ohtsu M, Oguni H, Hayashi K, Funatsuka M, Imai K, Osawa M. EEG in children with Early-onset Benign Occipital Seizure Susceptibility Syndrome: Panayiotopoulos Syndrome.  Epilepsia. 2003;  44 435-442
• 24 Panayiotopoulos CP. Vomiting as an ictal manifestation of epileptic seizures and syndromes.  J Neurol, Neurosurg Psychiatry. 1998;  51 1448-1451
• 25 Panayiotopoulos CP. The unified concept for the benign childhood partial seizures: benign childhood seizure susceptibility syndromes. In: Panayiotopoulos CP (Ed). Panayiotopoulos syndrome: A common and benign childhood epileptic syndrome. London: John Libbey & Co Ltd 2002: 104-105
• 26 Panayiotopoulos CP. Panayiotopoulos syndrome. In: A clinical guide to epileptic syndromes and their treatment. 2nd edn., Springer-Verlag, London 2007: 293-302
• 27 Roger J. Prognostic features of petit mal absences.  Epilepsia. 1974;  15 433
• 28 Sanders S, Rowlinson S, Manidakis I, Ferrie CD, Koutroumanidis M. The contribution of the EEG technologists in the diagnosis of Panayiotopoulos syndrome (susceptibility to early onset benign childhood autonomic seizures).  Seizure. 2004;  13 565-573
• 29 Sato S, Dreifuss FE, Penry JK, Kirby DD, Palesch Y. Long-term follow-up of absence seizures.  Neurology. 1983;  33 1590-1595
• 30 Su JY, Kim D-S, Ko T-S. Benign childhood epilepsy with centro-temporal spikes (BCECTS): early onset of seizures is associated with poorer response to initial treatment.  Epileptic Disord. 2006;  8 285-288
• 31 Takahashi K, Saito M, Kyo K, Gomibuchi K, Tada H, Honda T. et al . The effects of clonazepam on Rolandic discharges of benign epilepsy of children with centro-temporal EEG foci.  Jpn J Psychiatry Neurol. 2006;  45 468-470

Correspondence