From the Neurology Department (M.A.A.), Mount Auburn Hospital, Harvard Medical School, Cambridge, MA; Neurology Department (J.M.H., M.M.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (Y.G.), Beijing Tongren Hospital, Capital Medical University, China; Department of Pathology (M.M.-L.), Massachusetts General Hospital, Harvard Medical School, Boston; Neurology Department (T.G.), The George Washington University, DC; Neurology Department (J.S.), Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA; and Neurology Department (T.C.), University of Iowa.
Neurol Neuroimmunol Neuroinflamm. 2021 Jan 5;8(2). doi: 10.1212/NXI.0000000000000936. Print 2021 Mar 4.
To determine whether studying patients with strictly unilateral relapsing primary angiitis of the CNS (UR-PACNS) can support hemispheric differences in immune response mechanisms, we reviewed characteristics of a group of such patients.
We surveiled our institution for patients with UR-PACNS, after characterizing one such case. We defined UR-PACNS as PACNS with clinical and radiographic relapses strictly recurring in 1 brain hemisphere, with or without hemiatrophy. PACNS must have been biopsy proven. Three total cases were identified at our institution. A literature search for similar reports yielded 4 additional cases. The combined 7 cases were reviewed for demographic, clinical, imaging, and pathologic trends.
The median age at time of clinical onset among the 7 cases was 26 years (range 10-49 years); 5 were male (71%). All 7 patients presented with seizures. The mean follow-up duration was 7.5 years (4-14.1 years). The annualized relapse rate ranged between 0.2 and 1. UR-PACNS involved the left cerebral hemisphere in 5 of the 7 patients. There was no consistent relationship between the patient's dominant hand and the diseased side. When performed (5 cases), conventional angiogram was nondiagnostic. CSF examination showed nucleated cells and protein levels in normal range in 3 cases and ranged from 6 to 11 cells/μL and 49 to 110 mg/dL in 4 cases, respectively. All cases were diagnosed with lesional biopsy, showing lymphocytic type of vasculitis of the small- and medium-sized vessels. Patients treated with steroids alone showed progression. Induction therapy with cyclophosphamide or rituximab followed by a steroid sparing agent resulted in the most consistent disease remission.
Combining our 3 cases with others reported in the literature allows better clinical understanding about this rare and extremely puzzling disease entity. We hypothesize that a functional difference in immune responses, caused by such discrepancies as basal levels of cytokines, asymmetric distribution of microglia, and differences in modulation of the systemic immune functions, rather than a structural antigenic difference, between the right and left brain may explain this phenomenon, but this is speculative.
为了确定研究单侧复发原发性中枢神经系统血管炎（UR-PACNS）患者是否能够支持半球间免疫反应机制的差异，我们回顾了一组此类患者的特征。
在描述了 1 例此类病例后，我们对我院的 UR-PACNS 患者进行了调查。我们将 UR-PACNS 定义为临床和影像学复发严格局限于 1 个脑半球的 PACNS，伴有或不伴有偏瘫。PACNS 必须经过活检证实。在我院共发现 3 例病例。通过文献检索，又发现了 4 例类似的病例报告。对这 7 例病例的人口统计学、临床、影像学和病理学趋势进行了综合回顾。
7 例患者的临床发病中位年龄为 26 岁（范围 10-49 岁）；5 例为男性（71%）。所有 7 例患者均有癫痫发作。平均随访时间为 7.5 年（4-14.1 年）。年复发率在 0.2 至 1 之间。UR-PACNS 累及 7 例患者中的 5 例左侧大脑半球。患者的利手与患病侧之间没有一致的关系。当进行常规血管造影时（5 例），结果无诊断意义。CSF 检查显示 3 例患者有核细胞和正常蛋白水平，4 例患者分别为 6-11 个细胞/μL 和 49-110mg/dL。所有病例均经病变活检诊断为小-中血管淋巴细胞性血管炎。单独使用类固醇治疗的患者病情进展。环磷酰胺或利妥昔单抗诱导治疗后，再使用类固醇维持药物治疗，可使疾病缓解最持久。
将我们的 3 例病例与文献报道的其他病例相结合，可以更好地了解这种罕见且极具挑战性的疾病实体。我们假设，右左大脑半球之间免疫反应的功能差异，而不是结构抗原差异，可能导致这种现象，这种差异可能是由细胞因子基础水平、小胶质细胞不对称分布和全身免疫功能调节的差异等引起的，但这只是推测。
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