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Bilateral Focal Polymicrogyria in Ehlers-Danlos Syndrome

2000; American Medical Association; Volume: 57; Issue: 1 Linguagem: Inglês

10.1001/archneur.57.1.123

1538-3687

Andoni Echaniz-Laguna, Anne de Saint Martin, Anne Lafontaine, E. Tasch, Pierre Thomas, Edouard Hirsh, Christian Marescaux, F. Andermann,

Caveolin-1 and cellular processes

Background: Ehlers-Danlos syndrome (EDS) is a heterogeneous group of generalized connective tissue disorders that has been described in association with epilepsy and cerebral cortical dysplasia, mostly gray matter heterotopias, in 3 reports.However, to our knowledge, association of EDS with another type of cortical cerebral dysplasia, bilateral focal polymicrogyria, has never previously been described.Setting: Two research-oriented hospitals.Patients: We describe 2 patients with EDS and bilateral polymicrogyria.The first, a 29-year-old black man, presented with EDS of unspecified type, seizures, and bilateral frontocentral and frontoposterior polymicrogy-ria with hypoplasia of the inferior part of the cerebellar vermis.The second, a 20-year-old woman, had type III EDS, seizures and congenital bilateral perisylvian syndrome with polymicrogyria.