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

Iyer RS - Epilepsy Behav Case Rep (2014)

Bottom Line: Depending on the degree of disturbance and the resultant cortical excitability, this could result in various epilepsy syndromes.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, KG Hospital & Post Graduate Medical Institute, Coimbatore, Tamil Nadu, India.

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.

No MeSH data available.


Related in: MedlinePlus

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).
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f0005: 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).

Mentions: A video-EEG evaluation showed few focal seizures involving the right half of the body (Video 1) and the remaining few involving the left half of the body (Video 2). The corresponding ictal EEG showed seizure origin from the left hemisphere (Fig. 1d) and from the right hemisphere (Fig. 1c). The interictal EEG showed multifocal spikes (Fig. 1a) and burst-attenuation pattern (Fig. 1b). Karyotyping revealed a supermale with 47XYY pattern (Fig. 2).


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

Iyer RS - Epilepsy Behav Case Rep (2014)

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).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4307872&req=5

f0005: 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).
Mentions: A video-EEG evaluation showed few focal seizures involving the right half of the body (Video 1) and the remaining few involving the left half of the body (Video 2). The corresponding ictal EEG showed seizure origin from the left hemisphere (Fig. 1d) and from the right hemisphere (Fig. 1c). The interictal EEG showed multifocal spikes (Fig. 1a) and burst-attenuation pattern (Fig. 1b). Karyotyping revealed a supermale with 47XYY pattern (Fig. 2).

Bottom Line: Depending on the degree of disturbance and the resultant cortical excitability, this could result in various epilepsy syndromes.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, KG Hospital & Post Graduate Medical Institute, Coimbatore, Tamil Nadu, India.

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.

No MeSH data available.


Related in: MedlinePlus