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Spasms in clusters in epilepsies other than typical West syndrome

      Abstract

      Although spasms in clusters are one of the major characteristics of West syndrome (WS), there are a significant number of patients who show spasms in clusters but do not fit the standard pattern of WS. It is possible to divide these atypical cases into the following three groups. Group 1: refractory epilepsies beginning in early infancy, associated with atypical electroencephalographic (EEG) features; Group 2: generalized epilepsies with spasms in clusters at ages of 2–3 years or above; and Group 3: localization-related epilepsies with spasms in clusters. Ictal clinical and EEG findings of spasms in clusters in these atypical patients and also those in WS are similar. Patients in Group 1 often suffer from Aicardi syndrome, cortical malformations, early myoclonic encephalopathy and Ohtahara syndrome. Most patients in Group 2 suffer from Lennox–Gastaut syndrome and other generalized epilepsies such as severe epilepsy with multiple independent spike foci. A significant number of them had a history of WS. Small number of patients in Group 2 can be diagnosed as having late-onset WS or long-lasting WS. In Groups 1 and 3 patients, cortical mechanisms play a critical role in their pathophysiology. The presence of older patients with spasms in clusters might indicate not only developing process of the brain but also some selective dysfunction of the brain plays an important role in the occurrence of spasms in clusters. Investigations on these atypical patients can help the understanding of pathophysiological mechanisms of WS and its related epileptic syndromes.

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      References

        • Jeavons P.M.
        • Livet M.O.
        West syndrome: infantile spasms.
        in: Roger J. Bureau M. Dravet Ch. Dreifuss F.E. Perret A. Wolf P. Epileptic syndromes in infancy, childhood and adolescence. 2nd ed. John Libbey, London1992: 53-65
        • Ohtsuka Y.
        West syndrome and its related epileptic syndromes.
        Epilepsia. 1998; 39: 30-37
        • Chevrie J.J.
        • Aicardi J.
        The Aicardi syndrome.
        in: Pedley T.A. Meldrum B.S. Recent advances in epilepsy. Churchill Livingstone, Edinburgh1986: 189-210
        • Ohtsuka Y.
        • Oka E.
        • Terasaki T.
        • Ohtahara S.
        Aicardi syndrome: a longitudinal clinical and electroencephalographic study.
        Epilepsia. 1993; 34: 627-634
        • Aicardi J.
        The agyria–pachygyria complex: a spectrum of cortical malformations.
        Brain Dev. 1991; 13: 1-8
        • Vigevano F.
        • Fusco L.
        • Granata T.
        • Fariello G.
        • Di Rocco C.
        • Cusmai R.
        Hemimegalencephaly: clinical and EEG characteristics.
        in: Guerrini R. Andermann F. Canapicchi R. Roger J. Zifkin B.G. Pfanner P. Dysplasias of cerebral cortex and epilepsy. Lippincott-Raven, Philadelphia, PA1996: 285-294
        • Ohtsuka Y.
        • Ohno S.
        • Oka E.
        Electroclinical characteristics of hemimegalencephaly.
        Pediatr Neurol. 1999; 20: 390-393
        • Aicardi J.
        Early myoclonic encephalopathy (neonatal myoclonic encephalopathy).
        in: Roger J. Bureau M. Dravet Ch. Dreifuss F.E. Perret A. Wolf P. Epileptic syndromes in infancy, childhood and adolescence. 2nd ed. John Libbey, London1992: 13-23
        • Ohtahara S.
        • Ohtsuka Y.
        • Yamatogi Y.
        • Oka E.
        • Inoue H.
        Early-infantile epileptic encephalopathy with suppression-bursts.
        in: Roger J. Bureau M. Dravet Ch. Dreifuss F.E. Perret A. Wolf P. Epileptic syndromes in infancy, childhood and adolescence. 2nd ed. John Libbey, London1992: 25-34
        • Kobayashi K.
        • Ohtsuka Y.
        • Ohno S.
        • Ogino T.
        • Ohmori I.
        • Yoshinaga H.
        • et al.
        Clinical spectrum of epileptic spasms associated with cortical malformation.
        Neuropediatrics. 2001; (in press)
        • Ohtsuka Y.
        • Murashima I.
        • Asano T.
        • Oka E.
        • Ohtahara S.
        Partial seizures in West syndrome.
        Epilepsia. 1996; 37: 1060-1067
        • Donat J.F.
        • Wright F.S.
        Simultaneous infantile spasms and partial seizures.
        J Child Neurol. 1991; 6: 246-250
        • Murakami N.
        • Ohtsuka Y.
        • Ohtahara S.
        Early infantile epileptic syndromes with suppression-bursts: early myoclonic encephalopathy vs. Ohtahara syndrome.
        Jpn J Psychiatr Neurol. 1993; 47: 197-200
        • Boniver C.
        • Dravet C.
        • Bureau M.
        • Roger J.
        Idiopathic Lennox–Gastaut syndrome.
        in: Wolf P. Dam M. Janz D. Dreifuss F.E. Advances in epileptology. Raven Press, New York, NY1987: 195-200
        • Shigematsu H.
        • Yagi K.
        • Seino M.
        A peculiar minor seizures characterized by flexor spasms in Lennox–Gastaut syndrome evolved from West syndrome.
        Jpn J Psychiatr Neurol. 1987; 41: 457-458
        • Donat J.F.
        • Wright F.S.
        Seizures in series: similarities between seizures of the West and Lennox–Gastaut syndromes.
        Epilepsia. 1991; 32: 504-509
        • Mizukawa M.
        • Ohtsuka Y.
        • Kobayashi K.
        • Ohno S.
        • Murakami N.
        • Oka E.
        • et al.
        Seizures-in-series observed in symptomatic generalized epilepsy other than West syndrome.
        Psychiatry Clin Neurosci. 1995; 49: S296-S298
        • Ohtsuka Y.
        • Amano R.
        • Mizukawa M.
        • Maniwa S.
        • Ohtahara S.
        Long-term prognosis of the Lennox–Gastaut syndrome: consideration in its evolutional change.
        in: Fukuyama Y. Kamoshita S. Ohtsuka C. Suzuki Y. Modern perspectives of child neurology. The Japanese Society of Child Neurology, Tokyo1991: 215-222
        • Mizukawa M.
        Severe epilepsy with multiple independent spike foci.
        Tenkankenkyu (J Jpn Epil Soc). 1992; 10 (in Japanese): 78-87
        • Bednarek N.
        • Motte J.
        • Soufflet C.
        • Plouin P.
        • Dulac O.
        Evidence of late-onset infantile spasms.
        Epilepsia. 1998; 39: 55-60
        • Hrachovy R.A.
        • Frost J.D.
        • Gospe S.M.
        • Glaze D.G.
        Infantile spasms following near-drowning: a report of two cases.
        Epilepsia. 1987; 28: 45-48
        • Ohtsuka Y.
        • Asano T.
        Analysis of ictal EEGs of spasms-in-series in West syndrome and other generalized epilepsies.
        Ann Rep Jpn Epil Res Found (Osaka). 1998; 10 (in Japanese): 115-124
        • Kellaway P.
        • Hrachovy R.A.
        • Frost J.D.
        • Zion T.
        Precise characterization and quantification of infantile spasms.
        Ann Neurol. 1979; 6: 214-218
        • Fusco L.
        • Vigevano F.
        Ictal clinical electroencephalographic findings of spasms in West syndrome.
        Epilepsia. 1993; 34: 671-678
        • Ohtahara S.
        Clinico-electrical delineation of epileptic encephalopathies in childhood.
        Asian Med J. 1978; 21: 499-509
        • Gobbi G.
        • Bruno L.
        • Pini A.
        • Giovanardi Rossi P.
        • Tassinari C.A.
        Periodic spasms: an unclassified type of epileptic seizure in childhood.
        Dev Med Child Neurol. 1987; 29: 766-775
        • Gobbi G.
        • Pini A.
        • Parmeggiani A.
        • Santucci M.
        • Giovanardi Rossi P.
        • Guerrini R.
        Periodic spasms in cortical dysplasia.
        in: Guerrini R. Andermann F. Canapicchi R. Roger J. Zifkin B.G. Pfanner P. Dysplasias of cerebral cortex and epilepsy. Lippincott-Raven, Philadelphia, PA1996: 311-321
        • Chugani H.T.
        • Shields W.D.
        • Shewmon D.A.
        • Olson D.M.
        • Phelps M.E.
        • Peacock W.J.
        Infantile spasms. I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment.
        Ann Neurol. 1990; 27: 406-413
        • Kubota T.
        • Aso K.
        • Negoro T.
        • Okumura A.
        • Natsume J.
        • Takada H.
        • et al.
        Epileptic spasms preceded by partial seizures with a close temporal association.
        Epilepsia. 1999; 40: 1572-1579
        • Dulac O.
        • Chiron C.
        • Robain O.
        • Plouin P.
        • Jambaque I.
        • Pinard J.
        Infantile spasms: a pathophysiological hypothesis.
        in: Nehlig A. Motte J. Moshé S.L. Plouin P. Childhood epilepsies and brain development. John Libbey, London1999: 93-102
        • Commission on Pediatric Epilepsy of the International League Against Epilepsy
        Workshop on infantile spasms.
        Epilepsia. 1992; 33: 195
        • Hrachovy R.A.
        • Frost J.D.
        • Kellaway P.
        Hypsarrhythmia: variations on the theme.
        Epilepsia. 1984; 25: 317-325
        • Watanabe K.
        • Negoro T.
        • Aso K.
        • Matsumoto A.
        Reappraisal of interictal electroencephalogram in infantile spasms.
        Epilepsia. 1993; 34: 679-685
        • Chugani H.T.
        • Shewmon D.A.
        • Sankar R.
        • Chen B.C.
        • Phelps M.E.
        Infantile spasms: II. Lenticular nuclei and brain stem activation on positron emission tomography.
        Ann Neurol. 1992; 31: 212-219