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Journal of Child Neurology
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0883073808327841v1
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Article

Bilateral Operculum Syndrome in Childhood

Nóra Szabó, MD1, Ágnes Hegyi, AM, PhD1, Márta Boda, MD, PhD1, Margit Páncsics, MD2, Csenge Pap1, Kristóf Zágonyi1, Éva Romhányi, MD1, Sándor Túri, MD, PhD, DSc1, and László Sztriha, MD, PhD, DSc1*

1 Department of Paediatrics, University of Szeged, Szeged, Hungary
2 Department of Paediatrics, Municipal Hospital, Baja, Hungary

* To whom correspondence should be addressed. E-mail: sztriha{at}pedia.szote.u-szeged.hu.


  Abstract
We describe 3 patients with bilateral operculum syndrome. They presented with various degrees of suprabulbar (pseudobulbar) signs in addition to delay in cognitive, motor, and speech development in 2 children and developmental language disorder in the third one. A patient with schizencephaly in the left perisylvian area and contralateral polymicrogyria had spastic hemiparesis on the right side, whereas another patient showed bilateral underdevelopment of the opercula in association with axial hypotonia and spastic diplegia. Both of them had epileptiform discharges on the electroencephalogram without clinical manifestations of seizures. The magnetic resonance imaging of the third child with developmental language disorder was normal; however, his electroencephalogram showed frequent bilateral subclinical centrotemporal epileptiform discharges, probably responsible for the speech delay. Structural or functional involvement of the opercula bilaterally was a common finding in all the 3 patients and they had symptoms similar to those described in the developmental type of Foix-Chavany-Marie and Worster-Drought syndromes.

First published on February 5, 2009, doi:10.1177/0883073808327841

Journal of Child Neurology 2009;24:544.

A more recent version of this article appeared on May 1, 2009

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