Montague Turlough, Han Jenny, Cheung Emily, Drummond James, Soh Hwei Choo, Lechner-Scott Jeannette, Parratt John
Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia.
Department of Neurology, John Hunter Hospital, Newcastle, NSW, Australia.
Front Immunol. 2025 Jun 2;16:1502022. doi: 10.3389/fimmu.2025.1502022. eCollection 2025.
Unilateral relapsing primary central nervous system vasculitis (UR-PCNSV) is a scarcely reported subtype of PCNSV. It is characterised by frequent relapses with lesions confined to a single hemisphere. Herein, we expand the phenotype of UR-PCNSV, adding three cases to the existing 13 in the literature.
A retrospective review of clinic databases at two adult tertiary referral centres in New South Wales, Australia, was undertaken to identify cases of UR-PCNSV. Predefined inclusion criteria were (1) biopsy-proven PCNSV, (2) lesions confined to a single hemisphere, and (3) two or more relapses as evidenced by new enhancing lesions on MRI.
Three cases of biopsy-proven UR-PCNSV were identified. All demonstrated three or more relapses with new lesions confined to the same hemisphere. The mean age was 34.5 (± 8.6) years, and the median delay to diagnosis was 12 months (IQR 7.5-21). Headache was the first symptom in all patients, and they developed unilateral motor and sensory deficits. Cognitive impairment was a prominent feature in one and none developed seizures. CT and/or MR angiography showed normal results. MRI head showed both subcortical and cortical lesions with parenchymal and leptomeningeal enhancement. The protein level was normal in all patients, and one had a mildly raised white cell count (9 × 10/L). Biopsy in all three demonstrated a T-cell predominant perivascular lymphocytic infiltrate with areas of transmural inflammation and infarct-like necrosis. Despite treatment with anti-CD20 monoclonal antibodies, relapses occurred after steroid withdrawal in all. Prolonged steroid with additional immunosuppression was required to maintain remission. All patients demonstrated hemiatrophy within 12 months of presentation.
Compared with typical PCNSV, this rare unilateral, relapsing subtype has a younger age of onset, lower prevalence of angiographic abnormalities, and frequent relapses. Our patients had persisting lesion enhancement despite anti-CD20 mAb monotherapy and demonstrated hemiatrophy within the first year, indicating high inflammatory activity and a requirement for additional immunosuppression. This case series additionally highlights the overlapping clinical and radiological features of PCNSV and CNS demyelination, which may contribute to diagnostic delay.
单侧复发性原发性中枢神经系统血管炎（UR-PCNSV）是原发性中枢神经系统血管炎（PCNSV）中一种鲜有报道的亚型。其特征为频繁复发且病变局限于单个半球。在此，我们扩展了UR-PCNSV的表型，在文献中已有的13例基础上又增加了3例。
对澳大利亚新南威尔士州两家成人三级转诊中心的临床数据库进行回顾性研究，以确定UR-PCNSV病例。预定义的纳入标准为：（1）经活检证实的PCNSV；（2）病变局限于单个半球；（3）磁共振成像（MRI）显示有新的强化病变，证实有两次或更多次复发。
确定了3例经活检证实的UR-PCNSV病例。所有病例均表现出三次或更多次复发，新病变局限于同一半球。平均年龄为34.5（±8.6）岁，诊断延迟的中位数为12个月（四分位间距7.5 - 21）。头痛是所有患者的首发症状，且均出现单侧运动和感觉障碍。认知障碍在1例患者中较为突出，无患者发生癫痫。CT和/或磁共振血管造影结果正常。头颅MRI显示皮质下和皮质病变，伴有实质和软脑膜强化。所有患者的蛋白水平正常，1例患者白细胞计数轻度升高（9×10⁹/L）。所有3例患者的活检均显示以T细胞为主的血管周围淋巴细胞浸润，伴有透壁炎症和梗死样坏死区域。尽管使用了抗CD20单克隆抗体进行治疗，但所有患者在停用类固醇后均复发。需要延长类固醇治疗并加用免疫抑制剂以维持缓解。所有患者在就诊后12个月内均出现半侧萎缩。
与典型的PCNSV相比，这种罕见的单侧复发性亚型发病年龄较轻，血管造影异常的发生率较低，且频繁复发。我们的患者尽管接受了抗CD20单克隆抗体单药治疗，但病变仍持续强化，并在第一年出现半侧萎缩，表明炎症活动度高且需要额外的免疫抑制治疗。该病例系列还突出了PCNSV和中枢神经系统脱髓鞘在临床和影像学上的重叠特征，这可能导致诊断延迟。
