Bilateral focal polymicrogyria in Ehlers-Danlos syndrome.
Arch Neurol
; 57(1): 123-7, 2000 Jan.
Article
em En
| MEDLINE
| ID: mdl-10634459
ABSTRACT
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 polymicrogyria 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.CONCLUSIONS:
The association of bilateral focal polymicrogyria and EDS in these 2 patients suggests that extracellular matrix proteins implicated in the pathogenesis of EDS, such as collagen and tenascin, may play an important role in cerebral cortical formation and organization. In a clinical setting, the association of EDS with these cortical structural lesions has implications for diagnosis and management.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Ehlers-Danlos
/
Malformações do Sistema Nervoso
Limite:
Adult
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Arch Neurol
Ano de publicação:
2000
Tipo de documento:
Article
País de afiliação:
França