This Article Full Text (PDF) All Versions of this Article: 0883073808327841v1 24/5/544    most recent References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Szabó, N. Articles by Sztriha, L. Search for Related Content PubMed PubMed Citation Articles by Szabó, N. Articles by Sztriha, L. Pubmed/NCBI databases Medline Plus Health Information Developmental Disabilities Social Bookmarking                         What's this?

Bilateral Operculum Syndrome in Childhood

Department of Paediatrics, University of Szeged, Szeged

Ágnes Hegyi, AM, PhD

Department of Paediatrics, University of Szeged, Szeged

Márta Boda, MD, PhD

Department of Paediatrics, University of Szeged, Szeged

Margit Páncsics, MD

Department of Paediatrics, Municipal Hospital, Baja Hungary

Csenge Pap

Department of Paediatrics, University of Szeged, Szeged

Kristóf Zágonyi

Department of Paediatrics, University of Szeged, Szeged

Éva Romhányi, MD

Department of Paediatrics, University of Szeged, Szeged

Sándor Túri, MD, PhD, DSc

Department of Paediatrics, University of Szeged, Szeged

László Sztriha, MD, PhD, DSc

Department of Paediatrics, University of Szeged, Szeged, sztriha{at}pedia.szote.u-szeged.hu

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.

Key Words: operculum • schizencephaly • perisylvian polymicrogyria • opercular underdevelopment • benign childhood epilepsy with centrotemporal (Rolandic) spikes • developmental language disorder • suprabulbar (pseudobulbar) paresis

This version was published on May 1, 2009

Journal of Child Neurology, Vol. 24, No. 5, 544-550 (2009)
DOI: 10.1177/0883073808327841


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?