Abstract
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.
Keywords
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References
- Observations on an ethnic classification of idiots.London Hosp Clin Lectures Rep. 1866; 3: 259-262
- Mental retardation and developmental disabilities.in: Wortis J. Epilepsy. Bruner/Mazel, New York1975: 70-111
- Mongolism and convulsive seizures.Arch Neurol Psychiatry. 1955; 74: 559-563
- Seizure disorders in Down syndrome.Arch Neurol. 1991; 48: 318-320
- Seizures in children with Down syndrome: etiology, characteristics and outcome.Dev Med Child Neurol. 1991; 33: 191-200
- Epilepsy in childhood Down syndrome.Brain Dev. 1984; 6: 37-44
- Seizures in patients with trisomy 21.Am J Med Genet Suppl. 1990; 7: 298-300
- 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
- Epilepsy in Down syndrome: clinical aspects and possible mechanisms.Am J Ment Retard. 1993; 98: 12-26
- Normal maturation of the neonatal and infant brain. MR imaging at 1.5 T.Radiology. 1988; 166: 173-180
- Bayley N. Bayley scales of infant development. 2nd ed. The Psychological Cooperation, New York1999
- Seizures in persons with Down syndrome: cause and prognosis.Ann Neurol. 1988; 24: 308-309
- Synaptic dysgenesis.Can J Neurol Sci. 1991; 18: 170-180
- Neuropathology of Down syndrome.in: Nadel L. The psychobiology of Down syndrome. MIT Press, Cambridge, MA1988: 269-289
- Down syndrome children often have brain with maturation delay, retardation of growth, and cortical dysgenesis.Am J Med Genet Suppl. 1990; 7: 274-281
- Abnormal electric membrane properties of Down syndrome DRG neurons in cell culture.Dev Brain Res. 1982; 2: 257-270
- Dysregulation of gene expression in mouse trisomy 16, an animal model of Down syndrome.EMBO J. 1992; 11: 619-627
- Infantile spasms in children with Down syndrome.Dev Med Child Neurol. 1994; 36: 576-585
Article info
Publication history
Accepted:
June 13,
2001
Received in revised form:
June 6,
2001
Received:
December 21,
1999
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
