Abstract
The purpose of the study was to identify the clinical symptomatology of children with focal sharp waves of genetic nature. Genetic determination was assumed if the EEG of at least one sib revealed typical focal sharp waves. Forty-one probands (27 boys, 14 girls) and their 44 sibs showed a broad spectrum of different seizure types of focal origin including so-called atypical benign partial epilepsy (pseudo-Lennox-syndrome) and febrile convulsions. Numerous cases showed psychomental retardation or selective deficits of development. The classical syndrome of rolandic seizures with normal psychomental development and normal neurological findings was present in only a few cases. Similarly, EEG findings were quite variable. Rolandic sharp wave foci alone were found in only 22% of the probands. The remainder had foci in other regions (17%), or multiple foci (61%). Depending on age at first seizure, 42%–79% of the seizure-affected probands and 50% of the affected sibs showed generalized heritable EEG traits such as 4–7/s rhythms, generalized irregular spikes and waves, and/ or photoparoxysmal response. Three pairs of monozygotic twins were concordant for the special type of EEG findings, two of them also for clinical symptoms.
Similar content being viewed by others
Abbreviations
- ESES:
-
electrical status epilepticus during sleep
References
Aicardi J (1986) Epilepsy in children. Raven Press, New York, pp 39–66
Aicardi J, Chevrie JJ (1982) Atypical benign partial epilepsy of childhood. Dev Med Child Neurol 24:281–292
Aicardi J, Newton R (1987) Clinical findings in children with occipital spike wave complexes suppressed by eye opening. In: Andermann F, Lugaresi F (eds) Migraine and epilepsy. Butterworths, Boston London Durban, pp 111–124
Annegers JF, Hauser WA, Elveback LR, Kurland LT (1979) The risk of epilepsy following febrile convulsions. Neurology 29:297–303
Beaumanoir A, Ballis T, Varfis G, Ansari K (1974) Benign epilepsy of childhood with rolandic spikes. Epilepsia 15:301–315
Beaussart M (1972) Benign epilepsy of children with rolandic (centro-temporal) paroxysmal foci. Epilepsia 13:785–811
Bray PF, Wiser WC (1965) The relation of focal to diffuse epileptiform EEG discharges in genetic epilepsy. Arch Neurol 13:223–237
Dalla Bernardina B, Tassinari CA, Dravet C, Bureau M, Beghini G, Roger J (1978) Epilepsie partielle bénigne et état de mal électroencéphalographique pendant le sommeil. Rev Electroencephalogr Neurophysiol Clin 8:350–353
Dalla Bernardina B, Chiamenti C, Capovilla G, Colamaria V (1985) Benign partial epilepsies in childhood. In: Roger J, Dravet C, Bureau M, Dreyfuss FE, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence, Libbey Eurotext, London Paris, pp 137–149
Doose H, Baier WK (1989) Benign partial epilepsy and related conditions: multifactorial pathogenesis with hereditary impairment of brain maturation. Eur J Pediatr 149:152–158
Doose H, Gerken H, Hien-Voelpel KF, Voelzke E (1969) Genetics of photosensitive epilepsy. Neuropaediatrie 1:56–73
Eeg-Olofsson O, Petersèn I, Sellden U (1971) The development of the electroencephalogram in normal children from the age of 1 through 15 years. Paroxysmal activity. Neuropaediatrie 2:375–404
Engle M, Lüders H, Chutorian AM (1977) The significance of focal spikes in epileptic and nonepileptic children who are otherwise normal. Ann Neurol 2:257–258
Gastaut H (1982) A new type of epilepsy: benign partial epilepsy of childhood with occipital spike-waves. Clin Electroencephalogr 13:13–22
Gastaut H (1985) Benign epilepsy of childhood with occipital paroxysms. In: Roger J, Dravet C, Bureau M, Dreyfuss FE, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence. Libbey Eurotext, London Paris, pp 159–170
Gastaut H, Zifkin BG (1987) Benign epilepsy of childhood with occipital spike and wave complexes. In: Andermann F, Lugaresi E (eds) Migraine and epilepsy. Butterworth, Boston London Durban, pp 47–81
Gerken H (1971) Ueber konstitutionelle EEG-Anomalien bei kindlichen Epilepsien. Habilitation Thesis, Kiel University, Kiel
Gibbs EL, Gillen HW, Gibbs FA (1954) Disappearence and migration of epileptic foci in childhood. Am J Dis Child 88:596–603
Kellaway P (1982) Maturational and biorhythmic changes in the electroencephalogram. In: Anderson VE, Hauser WA, Penry JK, Sing CF (eds) Genetic basis of the epilepsies. Raven Press, New York, pp 21–33
Kuzniecki R, Rosenblatt B (1987) Benign occipital epilepsy: a family study. Epilepsia 28:346–350
Lerman P, Kivity S (1975) Benign focal epilepsy of childhood. Arch Neurol 32:261–264
Lombroso C (1967) Sylvian seizures and midtemporal spike foci in children. Arch Neurol 17:51–59
Lüders H, Lesser RP, Dinner DS, Morris III HH (1987) Benign focal epilepsy of childhood. In: Lüders H, Lesser RP (eds) Epilepsy: electroclinical syndromes. Springer, Berlin Heidelberg New York, pp 303–346
Petersen J, Nielsen CJ, Gulmann NC (1983) Atypical EEG abnormalities in children with benign partial (Rolandic) epilepsy. Acta Neurol Scand [Suppl] 94:57–62
Stollhoff K, Albani M, Landau-Kleffner-Syndrome — a family study (in preparation)
Tassinari CA, Bureau M, Dravet C, Dalla Bernardina B, Roger J (1985) Epilepsy with continuous spikes and waves during slow sleep — otherwise described as ESES (epilepsy with electrical status epilepticus during slow sleep). In: Roger J, Dravet C, Bureau M, Dreyfuss FE, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence. Libbey Eurotext, London Paris, pp 194–204
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Doose, H. Symptomatology in children with focal sharp waves of genetic origin. Eur J Pediatr 149, 210–215 (1989). https://doi.org/10.1007/BF01958285
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01958285