Abstract:
Here, we describe two patients with juvenile xanthogranuloma (JXG) manifesting with Langerhans cell histiocytosis (LCH)-associated neurodegenerative disease (ND)-like radiological findings. One patient showed typical radiological abnormalities at onset, which worsened with progressing central nervous system symptoms 7 years after LCH-oriented chemotherapy. Another showed spontaneous regression of clinical symptoms, with a transient radiological change 1 year after salvage chemotherapy for recurrence of JXG. These data regarding JXG-associated ND will facilitate future investigation of the disease, as well as development of therapeutic interventions.
摘要:
这里,我们描述了两名表现为朗格汉斯细胞组织细胞增生症(LCH)相关神经退行性疾病(ND)样影像学表现的青少年黄色肉芽肿(JXG)患者.一名患者在发病时表现出典型的放射学异常,以LCH为导向的化疗后7年,中枢神经系统症状恶化。另一个显示临床症状自发消退,因JXG复发而挽救化疗1年后出现一过性放射学改变。这些关于JXG相关ND的数据将有助于未来对该疾病的调查,以及治疗干预措施的发展。