Original article| Volume 23, ISSUE 6, P375-378, October 2001

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Seizure frequency and characteristics in children with Down syndrome


      Seizures have not historically been considered a major component of Down syndrome. We examined the prevalence of epileptic seizures in 350 children and adolescents with Down syndrome evaluated at a regional center between 1985 and 1997. Results showed that 28 patients (8%) had epileptic seizures: 13 (47%) partial seizures; 9 (32%) infantile spasms, and 6 (21%) generalized tonic–clonic seizures. In the infantile spasm group, there was no relationship between the initial electroencephalogram (EEG) pattern and response to treatment or long-term seizure control, or between type of pharmacologic treatment (valproic acid, adrenocorticotropic hormone or both) and clinical remission, EEG normalization or long-term seizure control. Neurodevelopmental outcome was poor despite good seizure control in the infantile spasm group. This regional study reinforces the relative association of seizures and Down syndrome. A prospective study including a national/international registry with emphasis on developmental assessment and long-term follow up is warranted.


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        • Down J.L.H.
        Observations on an ethnic classification of idiots.
        London Hosp Clin Lectures Rep. 1866; 3: 259-262
        • Corbett J.A.
        • Harris R.
        • Robinson R.
        Mental retardation and developmental disabilities.
        in: Wortis J. Epilepsy. Bruner/Mazel, New York1975: 70-111
        • Walter R.D.
        • Yeager C.L.
        • Rubin H.K.
        Mongolism and convulsive seizures.
        Arch Neurol Psychiatry. 1955; 74: 559-563
        • Pueschel S.M.
        • Louis S.
        • McKnight P.
        Seizure disorders in Down syndrome.
        Arch Neurol. 1991; 48: 318-320
        • Stafstrom C.E.
        • Patxot D.F.
        • Gilmore H.E.
        • Wisniewski K.E.
        Seizures in children with Down syndrome: etiology, characteristics and outcome.
        Dev Med Child Neurol. 1991; 33: 191-200
        • Tatsuno M.
        • Hayashi M.
        • Iwamoto H.
        • Suzuki H.
        • Kuroki Y.
        Epilepsy in childhood Down syndrome.
        Brain Dev. 1984; 6: 37-44
        • Romano C.
        • Tine A.
        • Fazior G.
        • Rizzo R.
        • Colognola R.M.
        • Sorge G.
        • et al.
        Seizures in patients with trisomy 21.
        Am J Med Genet Suppl. 1990; 7: 298-300
        • Wisniewski K.E.
        • Laure-Kamionowska M.
        • Connel F.
        • Wen G.Y.
        Neuronal density and synaptogenesis in the postnatal stage of brain maturation in Down syndrome.
        in: Epstein C.J. The neurobiology of Down syndrome. Raven, New York1986: 29-44
        • Stafstrom C.E.
        Epilepsy in Down syndrome: clinical aspects and possible mechanisms.
        Am J Ment Retard. 1993; 98: 12-26
        • Barkovich A.J.
        • Kjos B.O.
        • Jackson Jr, D.E.
        • Norman D.
        Normal maturation of the neonatal and infant brain. MR imaging at 1.5 T.
        Radiology. 1988; 166: 173-180
      1. Bayley N. Bayley scales of infant development. 2nd ed. The Psychological Cooperation, New York1999
        • Stafstrom C.E.
        • Gilmore H.E.
        • Ehrenberg B.L.
        Seizures in persons with Down syndrome: cause and prognosis.
        Ann Neurol. 1988; 24: 308-309
        • Becker L.E.
        Synaptic dysgenesis.
        Can J Neurol Sci. 1991; 18: 170-180
        • Kemper T.L.
        Neuropathology of Down syndrome.
        in: Nadel L. The psychobiology of Down syndrome. MIT Press, Cambridge, MA1988: 269-289
        • Wisniewski K.E.
        Down syndrome children often have brain with maturation delay, retardation of growth, and cortical dysgenesis.
        Am J Med Genet Suppl. 1990; 7: 274-281
        • Scott B.S.
        • Petit T.L.
        • Becker L.E.
        • Edwards B.A.V.
        Abnormal electric membrane properties of Down syndrome DRG neurons in cell culture.
        Dev Brain Res. 1982; 2: 257-270
        • Holtzman D.M.
        • Bayney R.M.
        • Li Y.
        • Khosrovi H.
        • Berger C.N.
        • Epstein C.P.
        • et al.
        Dysregulation of gene expression in mouse trisomy 16, an animal model of Down syndrome.
        EMBO J. 1992; 11: 619-627
        • Stafstrom C.E.
        • Konkol R.J.
        Infantile spasms in children with Down syndrome.
        Dev Med Child Neurol. 1994; 36: 576-585