A Clinical and Electroencephalographic Study of Myoclonic Epilepsies in Infancy and Early Childhood Part 1. On the Classification Key Words: myoclonic

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1 A Clinical and Electroencephalographic Study of Myoclonic Epilepsies in Infancy and Early Childhood Part 1. On the Classification Key Words: myoclonic epilepsy, classification, infancy and early childhood, prognosis, syndrome. Hirokazu Oguni, Yukio Fukuyama Department of Pediatrics, Tokyo Women's Medical College

2 Fig.1 Typical examples of generalized bilateral synchronous epileptic discharges recorded during sleep in patients with myoclonic seizures Type 1=Generalized bilateral synchrc nous multiple spikes and waves complexes Type 2=Generalized bilateral synchronous multiple sharp and slow waves complexes Type 3=Generalized burst of irregular multiple spikes associated with wave

3 Table 1 Subclassification of 149 patients with myoclonic seizures according to the clinial and electroencephalographic features The remaining 12 patients could not be classified. Abbreviations are as follows; Genetics=% of patients with positive family history of febrile convulsion or epilepsy BME=Benign myoclonic epilepsy in infants (Dravet, C) CMC=Cryptogenic myoclonic epilepsy of childhood (Aicardi, J) MEC=Myoclonic epilepsy of childhood (Jeavons, PM) SME=Severe myoclonic epilepsy in infants (Dravet, C) MAE=Myoclonic astatic epilepsy of early childhood (Doose, H)

4 lepsy of childhood (Aicardi)1 `3), benign myoclonic epilepsy in infants (Dravet)9), myoclonic astatic epilepsy of early childhood (Doose)6,7) Ì

5 severe myoclonic epilepsy in infants (Dravet10)) epilepsy with non-progressive encephalopathy jerks, myoclonic petit-mal, cryptogenicc myoclonic epilepsy of childhood, generalized epilepsy

6 mary generalized myoclonic astatic petit mal, myoclonic astatic epilepsy of early childhood

1) Aicardi, J. (1986) Epilepsy in children. International Review of Child Neurology Series, 39-65, Raven Press, Nw York. 2) Aicardi, J. (1986) Myoclonic epilepsies of in- and childhood.

9 1) Aicardi, J. (1986) Epilepsy in children. International Review of Child Neurology Series, 39-65, Raven Press, Nw York. 2) Aicardi, J. (1986) Myoclonic epilepsies of in- and childhood. In: Advance fancy in Neurol- ogy (Fahn, S., Marsden, D., Van Woert, M. eds.) Vol 43, 11-31, Raven Press, New York. 3) Aicardi, J., Chevrie, J.J. (1971) Myoclonic epilepsies of childhood. Neuropediatrie 3, ) Aicardi, J. (1979) Course and prognosis of certain childhood epilepsy with predominantly myoclonic seizures. In: Advance in epileptology: The 10 th Epilepsy International Symposium (Wada, J.A., Penry, J.K. eds.) , Raven Press, New York. 5) Dalla Bernardina, B., Colomaria, V., Capcvilla, G., Bondavalli, S. (1982) Nosological Classification of epilepsies in the first three years of life. In: Epilepsy: An update on research and therapy (Nistico, G., Di Perri R., Meinardi, H. eds.) , Alan Liss, New York. 6) Doose, H., Gerken, H., Leonhardt, R., Volzke, E., Volz, C. (1970) Centrencephalic myoclonic- petit mal. Neuropediatrie astatic 2, ) Doose, H. (1985) Myoclonic astatic epilepsy of early childhood. In: Epileptic syndromes in infancy, childhood and adolescence (Roger, J., Dravet, C., Bureau, M., Dreifuss, F.E., Wolf, P. eds.) 58-67, John Libbey, London. 8) Doose, H. (1983) Nonconvulsive status epilept-

10 icus in childhood: clinical aspect and classification. In: Advance in Neurology, Status epilepticus (Delgado-Escueta, A.V., Wasterlain, C.G., Treiman, D.M., Porter, R.J. eds.) Vol 34, 83-92, Raven Press, New York. 9) Dravet, C., Bureau, M., Roger, J. (1985) Benign myoclonic epilepsy in infants. In: Epileptic syndromes in infancy, childhood and adolescence (Roger, J., Dravet, C., Bureau, M., Dreifuss, F.E., Wolf, P. eds.) 51-57, John Libbey, London. 10) Dravet, C., Bureau, M., Roger, J. (1985) Severe myoclonic epilepsy in infants. In: Epileptic syndromes in infancy, childhood and adolescence (Roger, J., Dravet, C., Bureau, M., Dreifuss, F.E., Wolf, P. eds.) 58-67, John Libbey, London. 11) Dravet, C., Roger, J., Bureau, M., Dalla Bernardina, B. (1982) Myoclonic epilepsies in childhood. In: Advance in epileptology: The 13th Epilepsy International Symposium (Akimoto, H., Kazamatsuri, H., Seino, M., Ward, A.A. eds.) , Raven Press, New York. 12) Harper, J.R. (1968) True myoclonic epilepsy in childhood. Arch Dis Childh 43, ) Jeavons, P.M. (1977) Nosological problem of myoclonic epilepsies in childhood and adolescence. Dev Med Child Neurol 19, ) Jeavons, P.M. (1982) Myoclonic epilepsies. Therapy and prognosis. In: Advance in epileptology: The 13th Epilepsy International Symposium (Akimoto, H., Kazamatsuri, H., Seino, M., Ward, A.A. eds.) , Raven New York. Press, 15) Seki, T., Yamawaki, H., Suzuki, N. (1980) Prognosis of myoclonic seizures in childhood epilepsy: A follcw-up study of more than three years. Folia Psychiatr Neurol Jpn 34, Summary A Clinical and Electroencephalographic Study of Myoclonic Epilepsies in Infancy and Early Childhood Part 1. On the Classification Hirokazu Oguni, Yukio Fukuyama Clinical and electroencephalographic features of myoclonic seizures (MS) were studied in 149 patients. On the basis of early development history, age at onset of epilepsy, combination of tonic spasm (TS), and an existence of generalized bilateral synchronous slow spike and wave burst (GSSW) or multifocal spikes (MFS) in interictal EEG, the patients could be placed into 5 groups: Group A (60 cases): Normal prior development to onset of epilepsy, absence of TS, GSSW and MFS. This group showed a high incidence of either febrile convulsions or epilepsy in the family history(fh), and MS being predominant seizure type. Prognosis for seizure control and mental outcome ranged from fair to good. Group B (18 cases): Normal prior development, presence of TS, GSSW. Seizure and mental outcome was poor. Group C (13 cases): Normal prior development, absence of TS, GSSW, and the combination of MFS and the age of onset less than 1 year. This group also exhibited a high incidence of FH and multiple seizure types, easily precipitated by fever. Prognosis was very poor. Group D (17 cases): Delayed development with gross neurological abnormalities often associated cerebral palsy. Group E (29 cases): Delayed development before onset of seizures and history of West syndrome without gross neurological abnormalities in all patients. The remaining 12 patients could not be classified. J. Jpn. Epil. Soc. 1989; 7: 67-76

Pure Petit Mal (Absence) Clinical and Electroencephalographic - Studies- Tohru SEKI, M. D., Itoko MORI, M. D., Setuko SUGIURA, M. D., Nobuko INADA, M.

Pure Petit Mal (Absence) Clinical and Electroencephalographic - Studies- Tohru SEKI, M. D., Itoko MORI, M. D., Setuko SUGIURA, M. D., Nobuko INADA, M. D., Mototugu KYO, M. D., Yo shihumi YAMAMOTO, M. D.