Sturge-Weber Syndrome. Study of 55 Patients

作者: Ignacio Pascual-Castroviejo , Samuel-Ignacio Pascual-Pascual , Ramón Velazquez-Fragua , Juán Viaño , None

DOI: 10.1017/S0317167100008878

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摘要: . Objectif : Il s'agit d'une revue des manifestations cliniques et de neuroimagerie observees chez une grande serie patients atteints du syndrome Sturge-Weber (SSW) examines au cours periode 40 ans. Methodes Cinquante-cinq SSW (30 hommes 25 femmes), ont ete entre 1965 2004. Nous revoyons les resultats examens neurologiques ophtalmologiques, etudes electroencephalographiques neuroimagerie. Tous par un auteurs (I.P - C). Resultats Les plus frequentes severes dans cette cas etaient l'epilepsie, l'hemiparesie, le retard mental problemes oculaires. Le naevus flammeus facial etait unilateral 35 (63,5%) d'angiomes leptomeninges, bilateral 17 (31%) absent 3 (5,5%). Sept (41%) parmi avaient angiomes leptomeninges unilateraux. Quarante-sept (85,5%) presentaient crises convulsives. Un controle absolu a obtenu 20 (42,5%). Cependant, 2 ces controlees seulement apres lobectomie. porteurs ou la paupiere superieure choroido-retiniens ipsilateraux, unilateraux bilateraux. Seulement 55 (36%) intelligence normale basse limite (QI < 70). Aucune relation n'a observee taille naevi faciaux severite lesions cerebrales. Conclusions: L'epilepsie, oculaires serie. cerebrales progressaient tot l'enfance, mais elle ne pas suite. traitement chirurgical precoce permis controler convulsives mitiges en ce qui concerne autres comme l'hemiparesie deficits intellectuels. debut convulsives, reponse mitigee traitement, bilaterales unilaterales associees pronostic defavorable. Une habiletes sociales limitees, apparence peu esthetique compromettaient l'integration SSW. Ces doivent etre abordes afin d'ameliorer interactions qualite vie favoriser leur acces emploi remunerateur.

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