Straube A, Padovan C S, Seelos K
Neurologie und Abteilung für Neuroradiologie, Universität München.
Nervenarzt. 2001 Aug;72(8):641-6. doi: 10.1007/s001150170066.
The Parry-Romberg syndrome is a rare and poorly understood disease characterized by slowly progressive, localized atrophy of the skin, subcutaneous tissue, muscles, and bones. The atrophy is typically localized in the face and begins in youth. In some patients, imaging can show the lesions and atrophy of the ipsilateral hemisphere of the brain. We report on a patient in whom the disease has lasted 36 years and discuss the possibility that the Parry-Romberg syndrome is related to known autoimmune disorders of the soft tissue (e.g., linear scleroderma) and Rasmussen's syndrome. There are some remarkable clinical similarities between these two syndromes, including age of onset, unilateral manifestation, and occurrence of focal seizures. It is most probable that both diseases have an autoimmunological background.
帕里-罗姆伯格综合征是一种罕见且了解甚少的疾病,其特征为皮肤、皮下组织、肌肉和骨骼的缓慢进行性局部萎缩。萎缩通常局限于面部,且始于青少年时期。在一些患者中,影像学检查可显示同侧大脑半球的病变和萎缩。我们报告了一名患病36年的患者,并讨论了帕里-罗姆伯格综合征与已知的软组织自身免疫性疾病(如线状硬皮病)和拉斯穆森综合征相关的可能性。这两种综合征在临床方面存在一些显著相似之处,包括发病年龄、单侧表现以及局灶性癫痫发作的发生。这两种疾病很可能都具有自身免疫背景。