Aggressive immunosuppressive therapies have been proposed to treat primary angiitis of the central nervous system (PACNS). Here, we report the first successfully stabilized case of childhood, small-vessel PACNS with intravenous immunoglobulin (IVIG) therapy. A 12-year-old boy was admitted to our hospital complaining of recurrent headaches and upper-left homonymous quadrantanopia, since the age of 11 years. Brain computed tomography scans revealed fine calcification in the right temporal and occipital lobes. Brain magnetic resonance imaging scans revealed white matter lesions, with gadolinium enhancement, which waxed, waned, and migrated for 1 year, without immunomodulatory therapies. A cerebrospinal fluid study showed pleocytosis (12 cells per µl). No clinical or serological findings suggested systemic inflammation or vasculitis. Brain angiography was unremarkable. Brain biopsy revealed thickened and hyalinized small vessels, with intramural infiltration of inflammatory cells, which confirmed the diagnosis of small-vessel PACNS. Because the patient developed surgical site infection following biopsy, the administration of monthly IVIG (2 g/kg) was prescribed, instead of immunosuppressive agents. After IVIG therapy, the patient remained stable, except for a single episode of mild radiological exacerbation at 16 months, which occurred when the IVIG interval was expanded. Oral prednisone was added and gradually tapered. At 50 months, his intellectual abilities and motor functions were normal, although he showed residual upper-left homonymous quadrantanopia and post-exercise headache. A temporary headache, associated with the immunoglobulin infusion, was resolved by slowing the infusion rate. PACNS should be treated aggressively to improve prognosis. However, when immunosuppressants are contraindicated, IVIG may be an alternative therapeutic option.

中文翻译：

---

IVIG治疗儿童中枢神经系统原发性血管炎一例

已经提出积极的免疫抑制疗法来治疗中枢神经系统的原发性血管炎 (PACNS)。在这里，我们报告了首例通过静脉注射免疫球蛋白 (IVIG) 治疗成功稳定的儿童小血管 PACNS 病例。一名 12 岁男孩自 11 岁起因反复头痛和左上同向象限性视疲劳入院。脑部计算机断层扫描显示右侧颞叶和枕叶有细微钙化。脑磁共振成像扫描显示白质病变，钆增强，在没有免疫调节治疗的情况下，增强、减弱和迁移 1 年。脑脊液研究显示细胞增多症（每微升 12 个细胞）。没有临床或血清学发现提示全身炎症或血管炎。脑血管造影无异常。脑活检显示小血管增厚和透明化，壁内有炎性细胞浸润，证实了小血管 PACNS 的诊断。由于患者在活检后出现手术部位感染，因此规定每月使用 IVIG (2 g/kg)，而不是免疫抑制剂。IVIG 治疗后，患者保持稳定，除了在 16 个月时出现单次轻度放射学恶化，这是在扩大 IVIG 间隔时发生的。添加口服泼尼松并逐渐减量。在 50 个月时，他的智力和运动功能正常，尽管他表现出残留的左上同向象限和运动后头痛。与免疫球蛋白输注有关的暂时性头痛，已通过减慢输液速度解决。PACNS 应积极治疗以改善预后。然而，当禁用免疫抑制剂时，IVIG 可能是另一种治疗选择。