Kamimura Naoya, Ueda Naohisa, Kimura Katsuo, Nishikori Asami, Sato Yasuharu, Kishida Hitaru, Tanaka Fumiaki
Department of Neurology, Yokohama City University Medical Center, Yokohama, Kanagawa, Japan.
Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama, Okayama, Japan.
BMJ Neurol Open. 2025 Apr 27;7(1):e000923. doi: 10.1136/bmjno-2024-000923. eCollection 2025.
Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disorder characterised by systemic inflammation resulting from overproduction of interleukin 6 (IL-6). While iMCD primarily affects the lymph nodes and related tissues, it can also rarely involve the central nervous system.
We report the case of a 58-year-old female patient with at least a 3-year history of iMCD, who experienced acute thunderclap headaches due to reversible cerebral vasoconstriction syndrome (RCVS). RCVS occurred 3 months after initiating treatment with tocilizumab, a humanised anti-IL-6 receptor monoclonal antibody, and was accompanied by focal cortical subarachnoid haemorrhage (SAH). Elevated IL-6 levels were found in both serum and cerebrospinal fluid. MR angiography revealed multiple diffuse stenotic lesions in the bilateral middle and posterior cerebral arteries, which, along with bilateral cerebral oedema, resolved within 3 months. The diffuse nature of the cerebral vasospasm and the presence of bilateral brain oedema suggested that cerebral vasospasm was due to RCVS rather than SAH.
In patients with Castleman disease, RCVS may occur due to IL-6-dependent chronic cerebral vascular inflammation, either as a primary condition or as a complication of tocilizumab treatment.
特发性多中心Castleman病（iMCD）是一种罕见的多克隆淋巴细胞增生性疾病，其特征为因白细胞介素6（IL-6）过度产生导致的全身炎症。虽然iMCD主要影响淋巴结及相关组织，但也很少累及中枢神经系统。
我们报告了一例58岁女性患者，患有iMCD至少3年，因可逆性脑血管收缩综合征（RCVS）出现急性霹雳样头痛。RCVS在开始使用托珠单抗（一种人源化抗IL-6受体单克隆抗体）治疗3个月后发生，并伴有局灶性皮质下蛛网膜下腔出血（SAH）。血清和脑脊液中均发现IL-6水平升高。磁共振血管造影显示双侧大脑中动脉和后动脉有多个弥漫性狭窄病变，这些病变连同双侧脑水肿在3个月内消退。脑血管痉挛的弥漫性以及双侧脑水肿的存在提示脑血管痉挛是由RCVS而非SAH引起的。
在Castleman病患者中，RCVS可能由于IL-6依赖性慢性脑血管炎症而发生，可为原发性疾病或托珠单抗治疗的并发症。
