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Case Reports
. 2014 Mar 20:2:43-5.
doi: 10.1016/j.ebcr.2014.02.003. eCollection 2014.

Migrating partial seizures in infancy and 47XYY syndrome: Cause or coincidence?

Rajesh Shankar Iyer  1 , Thanikasalam  1 , Mugundhan Krishnan  2
Affiliations
Case Reports

Migrating partial seizures in infancy and 47XYY syndrome: Cause or coincidence?

Rajesh Shankar Iyer et al. Epilepsy Behav Case Rep. .

Abstract

Migrating partial seizures in infancy (MPSI) is a rare epilepsy syndrome with poor prognosis. The exact etiology of MPSI is still not known. We report a 14-month-old baby with 47XYY karyotype who presented with developmental delay and drug-refractory seizures satisfying the diagnostic criteria for MPSI and discuss the possible association between the 47XYY karyotype and this syndrome. The excess of genes due to an additional Y chromosome could cause disturbance in various stages of formation, migration, or differentiation of neurons. Depending on the degree of disturbance and the resultant cortical excitability, this could result in various epilepsy syndromes. We feel that this association is more likely causal than coincidental. Chromosome studies need to be performed in more individuals with atypical and uncommon epilepsies. Multicenter studies are required to establish the association between epilepsy syndrome and these rare chromosome disorders.

Keywords: 47XYY; Karyotyping; Migrating partial seizures; Supermale; X chromosome; Y chromosome.

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Figures

Fig. 1
Fig. 1
Interictal EEG showing multifocal spikes (a) and burst-attenuation pattern (b). Ictal EEG showing right hemispheric seizure onset (arrows) with fast activity noted over the right hemisphere best expressed over the posterior leads (c) and left hemispheric seizure onset (arrows) with fast activity noted over the left hemisphere best expressed over the posterior leads (d).
Fig. 2
Fig. 2
Chromosome preparation showing an additional Y chromosome.
See this image and copyright information in PMC

References

    1. Coppola G., Plouin P., Chiron C., Robain O., Dulac O. Migrating partial seizures in infancy: a malignant disorder with developmental arrest. Epilepsia. 1995;36:1017–1024. - PubMed
    1. Coppola G., Veggiotti P., Del Giudice E.M., Bellini G., Longaretti F., Taglialatela M. Mutational scanning of potassium, sodium and chloride ion channels in malignant migrating partial seizures in infancy. Brain Dev. 2006;28:76–79. - PubMed
    1. Dulac O. Malignant migrating partial seizures in infancy. In: Roger J., Bureau M., Dravet C., Genton P., Tassinari C.A., Wolf P., editors. Epileptic syndromes in infancy, childhood and adolescence. 3rd ed. John Libbey & Company Ltd.; London: 2002. pp. 65–68.
    1. Wilmshurst J.M., Appleton D.B., Grattan-Smith P.J. Migrating partial seizures in infancy: two new cases. J Child Neurol. 2000;15:717–722. - PubMed
    1. Caraballo R.H., Fontana E., Darra F., Cassar L., Negrini F., Fiorini E. Migrating focal seizures in infancy: analysis of the electroclinical patterns in 17 patients. J Child Neurol. 2008;23:497–506. - PubMed

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