Supplementary Material for: Neurosarcoidosis Presenting with Prominent Periventricular White-Matter Lesions during Steroid Treatment for Autoimmune Hepatitis

A 63-year-old woman under treatment of autoimmune hepatitis presented with headache, memory loss, and somnolence. Three months before admission, the patient experienced liver inflammation relapse after prednisolone (PSL) cessation. Consequently, PSL was resumed and then tapered. Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with remarkably reduced glucose and elevated angiotensin-converting enzyme and soluble interleukin-2 receptor levels. Magnetic resonance imaging (MRI) revealed prominent bilateral periventricular white-matter lesions, hydrocephalus, ischemic stroke with gadolinium enhancement of frontoparietal and basilar meninges on contrast-enhanced fluid-attenuated inversion recovery. Magnetic resonance angiography (MRA) showed narrowing of the bilateral middle cerebral arteries. Based on these findings, we diagnosed the patient with neurosarcoidosis. Re-increment of PSL improved the neurological symptoms, CSF findings, and abnormalities found on MRI and MRA. This case suggests that neurosarcoidosis may occur as a complication of some autoimmune diseases during immunotherapy administration.

一名63岁正在接受自身免疫性肝炎（autoimmune hepatitis）治疗的女性患者，出现头痛、记忆力减退及嗜睡症状。入院前3个月，该患者在停用泼尼松龙（prednisolone，PSL）后出现肝炎复发，遂重新予以泼尼松龙治疗并逐步减量。脑脊液（cerebrospinal fluid，CSF）检查显示淋巴细胞增多，葡萄糖水平显著降低，血管紧张素转换酶（angiotensin-converting enzyme）及可溶性白细胞介素-2受体（soluble interleukin-2 receptor）水平升高。磁共振成像（magnetic resonance imaging，MRI）结果显示双侧脑室周围白质病变显著，伴脑积水；对比增强液体衰减反转恢复序列成像可见额顶叶及基底脑膜钆增强（gadolinium enhancement）的缺血性脑卒中表现。磁共振血管造影（magnetic resonance angiography，MRA）显示双侧大脑中动脉狭窄。基于上述检查结果，我们诊断该患者为神经结节病（neurosarcoidosis）。再次增加泼尼松龙剂量后，患者的神经系统症状、脑脊液检查结果及MRI、MRA发现的异常均得到改善。本病例提示，神经结节病可能作为部分自身免疫性疾病的并发症，在免疫治疗（immunotherapy）期间发生。