Department of Pediatrics, National Taiwan University Hospital Yunlin Branch, Yunlin, Taiwan.
Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan; and.
Pediatrics. 2019 Nov;144(5). doi: 10.1542/peds.2019-0505.
Schilder disease, also termed diffuse myelinoclastic sclerosis, is characterized by a large demyelinating lesion involving 1 or both sides of the centrum semiovale of the cerebral hemispheres. It often presents with tumorlike features and poses a diagnostic challenge. Schilder disease can be monophasic or relapsing, and disease-modifying therapy for the latter scenario is largely empirical. Here, we report a 14-year-old girl with relapsing Schilder disease within 1 year after disease onset. She has been followed-up for nearly 10 years and remains in sustained remission ever since interferon-β therapy was prescribed after the second attack. In this case study, it is suggested that interferon-β may induce long-term remission in relapsing Schilder disease and is therefore worth considering in this regard.
希尔德病，又称弥漫性髓鞘崩解性硬化症，其特征是大的脱髓鞘病变累及大脑半球半卵圆中心的 1 侧或双侧。它常表现为肿瘤样特征，诊断具有挑战性。希尔德病可为单相或复发型，后者的疾病修饰治疗在很大程度上是经验性的。本研究报告了 1 例发病 1 年后 1 年内复发的希尔德病患儿。自第二次发作后接受干扰素-β治疗以来，她已随访近 10 年，此后一直处于持续缓解状态。在本病例研究中，提示干扰素-β可能诱导复发性希尔德病的长期缓解，因此值得考虑。
