Department of Neurology, Bagcilar Research and Training Hospital, Istanbul, Turkey.
Department of Neurology, Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
Ideggyogy Sz. 2021 Nov 30;74(11-12):409-412. doi: 10.18071/isz.74.0409.
Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare, slowly progressive disorder characterized by unilateral, painless atrophy of the skin and subcutaneous tissue of the face. Neurological manifestations such as epilepsy, migraine and trigeminal neuralgia are relatively common and accompany in 15-20% of cases. Various etiologies such as infection, trauma, embryonic developmental dysfunction, sympathetic dysfunction and autoimmune disorders have been suggested as possible causes. Here we describe a 37-year-old woman whose disease manifested with dynamic contrast enhanced white matter changes over a period of two years, suggesting a "relapsing-remitting" course. Besides the inflammatory activity, positive serum-autoantibodies, inflammatory findings in cerebrospinal fluid, and an overlapping systemic autoimmune disorder may further support the hypothesis of autoimmune-inflammatory mediated pathogenesis.
进行性半侧颜面萎缩症，又称 Parry-Romberg 综合征，是一种罕见的、缓慢进展性疾病，其特征为单侧、无痛性的面部皮肤及皮下组织萎缩。癫痫、偏头痛和三叉神经痛等神经学表现较为常见，在 15-20%的病例中伴发。感染、创伤、胚胎发育功能障碍、交感神经功能障碍和自身免疫紊乱等多种病因被认为可能与此病相关。本文报道了一位 37 岁女性患者，其疾病在两年内表现为动态对比增强脑白质病变，提示“复发-缓解”病程。除了炎症活动外，血清自身抗体阳性、脑脊液炎症表现以及重叠的系统性自身免疫性疾病可能进一步支持自身免疫-炎症介导发病假说。
