Rafai M A, Boulaajaj F Z, El Moutawakil B, Bourezgui M, Sibai M, Mahtar M, Chekkouri A, Slassi I
Service de neurologie - explorations fonctionnelles, CHU Ibn Rochd, Casablanca, Maroc.
Rev Neurol (Paris). 2007 Dec;163(12):1246-8. doi: 10.1016/S0035-3787(07)78412-6.
Parry-Romberg syndrome is a clinical entity consisting of progressive hemifacial atrophy appearing at a young age. This syndrome has features of linear scleroderma 'en coup de sabre'. The aetiology of this affection is poorly understood. A number of neurologic disorders associations have been reported particularly seizures.
We report case of Parry Romberg syndrome in 29 years old men associated with dysphonia. Neurological examination and Brain CT scan were normal.
Different mechanisms have been discussed, immunological disorders, sympathic dysfunction or infectious origin. Management consists on reconstructive surgery, symptomatic treatment, with psychological issues.
帕里-龙贝格综合征是一种临床病症，表现为在年轻时出现进行性半侧面部萎缩。该综合征具有“剑伤状”线状硬皮病的特征。这种病症的病因尚不清楚。已经报道了一些与神经系统疾病的关联，特别是癫痫发作。
我们报告一例29岁男性的帕里-龙贝格综合征，伴有发音障碍。神经检查和脑部CT扫描均正常。
已经讨论了不同的机制，包括免疫紊乱、交感神经功能障碍或感染源。治疗包括重建手术、对症治疗以及心理问题处理。
