ICNC2018 Abstracts & Symposia Proposals, ICNC 2014

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Down Syndrome: Clinical and EEG Correlates during Development
Ahmed Khamis Bamaga, Miguel A. Cortez

Last modified: 2014-04-03

Abstract


Background:Down syndrome is the most common genetic cause of mental retardation and it is estimated that 5-13 % of persons affected with Down syndrome have seizures. In one series of 350 children with Down syndrome, seizures occurred in almost 8 % and one-third was infantile spasm.  Seizures evolution and EEG findings in Down syndrome haven’t been well investigated and studied.

Objectives:

  • To review the experience of Hospital for Sick Children on Down syndrome patients with seizure disorder over the last 20 years.
  • To follow and study the seizures’ evolution (clinically and EEG) and responding to pharmacological treatment.

Methods:

  • A retrospective analysis of HSC’s data including clinical information and EEGs result collected from 1990 to 2012 for Down syndrome patients with seizure.
  • All patients with Down's syndrome whose have MRI, EEG or presenting to ED or neurology clinic with seizures.

Hypothesis:

Down syndrome patients with hypsarrhythmia would present with worse cognitive outcomes

Result:

  • Total number of Down Syndrome patients with seizures seen at HSC between 1990 until 2012 was 71.
  • 34 out of the 71 DS patients had infantile spasm (47 % ) and 37 had other seizures type ( 53 % ).
  • Out of the 34 DS with infantile spasms; 23 patients had Hypsarrhythmia and 11 had no Hypsarrythmia.
  • 7 patients out of the 23 patients had sever developmental delay.

Conclusion:

Severe developmental outcomes tend to be more prevalent in Down syndrome patients with hypsarrhythmia than those Down syndrome patients without hypsarrhythmia.


Keywords


Infantile spasm, Down Syndrome

References


1. Arya, R., M. Kabra, and S. Gulati, Epilepsy in children with Down syndrome. Epileptic Disord, 2011. 13(1): p. 1-7.2. Cowan, L.D. and L.S. Hudson, The epidemiology and natural history of infantile spasms. J Child Neurol, 1991. 6(4): p. 355-64.3. Eisermann, M.M., et al., Infantile spasms in Down syndrome--effects of delayed anticonvulsive treatment. Epilepsy Res, 2003. 55(1-2): p. 21-7.4. Escofet, C., et al., [Infantile spasms in children with Down's syndrome]. Rev Neurol, 1995. 23(120): p. 315-7.5. Goldberg-Stern, H., et al., Seizure frequency and characteristics in children with Down syndrome. Brain Dev, 2001. 23(6): p. 375-8.6. Guerrini, R., et al., [The evolution of epilepsy in the most common genetic forms with mental retardation (Down's syndrome and the fragile X syndrome)]. Pediatr Med Chir, 1993. 15 Suppl 1: p. 19-22.7. Kajimoto, M., et al., West syndrome associated with mosaic Down syndrome. Brain Dev, 2007. 29(7): p. 447-9.8. Kajimoto, M., et al., West syndrome associated with mosaic Down syndrome. Brain Dev, 2007. 29(7): p. 447-9.9. Kwong, K.L. and V. Wong, Neurodevelopmental profile of Down syndrome in Chinese children. J Paediatr Child Health, 1996. 32(2): p. 153-7.10. Lott, I.T., Neurological phenotypes for Down syndrome across the life span. Prog Brain Res, 2012. 197: p. 101-21.11. Nabbout, R., et al., Infantile spasms in Down syndrome: good response to a short course of vigabatrin. Epilepsia, 2001. 42(12): p. 1580-3.12. Pueschel, S.M., S. Louis, and P. McKnight, Seizure disorders in Down syndrome. Arch Neurol, 1991. 48(3): p. 318-20.13. Romano, C., et al., Seizures in patients with trisomy 21. Am J Med Genet Suppl, 1990. 7: p. 298-300.14. Silva, M.L., et al., Early clinical and EEG features of infantile spasms in Down syndrome. Epilepsia, 1996. 37(10): p. 977-82.15. Stafstrom, C.E., Infantile spasms: a critical review of emerging animal models. Epilepsy Curr, 2009. 9(3): p. 75-81.16. Stafstrom, C.E. and R.J. Konkol, Infantile spasms in children with Down syndrome. Dev Med Child Neurol, 1994. 36(7): p. 576-85.17. Stafstrom, C.E., et al., Seizures in children with Down syndrome: etiology, characteristics and outcome. Dev Med Child Neurol, 1991. 33(3): p. 191-200.18. Tatsuno, M., et al., Epilepsy in childhood Down syndrome. Brain Dev, 1984. 6(1): p. 37-44.19. Pollack, M.A., et al., Infantile spasms in Down syndrome: a report of 5 cases and review of the literature. Ann Neurol, 1978. 3(5): p. 406-8.


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