Zurko Joanna, Mehta Amitkumar
Division of Hematology and Oncology, Department of Medicine, University of Alabama at Birmingham, Birmingham, AL.
Mayo Clin Proc Innov Qual Outcomes. 2018 Feb 1;2(1):74-77. doi: 10.1016/j.mayocpiqo.2017.12.001. eCollection 2018 Mar.
Immune-mediated encephalitis related to immune checkpoint inhibitor therapy is a rare but increasingly described condition that can cause significant morbidity. There are several reported cases in the literature but no previously described cases of immune-mediated cerebellitis. We describe a case of acute cerebellitis that developed in a 20-year-old man with primary refractory Hodgkin lymphoma being treated with the immune checkpoint inhibitor nivolumab. After exposure to 3 cycles of nivolumab, the patient had acute onset of headache, ataxia, nausea, and vomiting, with imaging findings of cerebellar edema, early tonsillar herniation, and early hydrocephalus. Immune-mediated cerebellar encephalitis was suspected and high-dose dexamethasone therapy (8 mg every 6 hours) was initiated. Within 4 days of dexamethasone therapy, his symptoms greatly improved with near-complete resolution of symptoms after a 4-week taper. Differential diagnosis of his condition included viral cerebellitis and paraneoplastic cerebellar degeneration. In cerebellar encephalitis suspected to be due to immune checkpoint inhibitor therapy, prompt recognition and early initiation of high-dose corticosteroids is essential for symptom resolution and treatment success, including the prevention of hydrocephalus and tonsillar herniation. Currently, there are no evidence-based guidelines to guide the initial dose, type, or duration of corticosteroids. Further investigation is needed in the pathogenesis and treatment of cerebellar encephalitis related to immune checkpoint inhibitor therapy to effectively treat this rare, disabling condition.
与免疫检查点抑制剂治疗相关的免疫介导性脑炎是一种罕见但报道日益增多的疾病，可导致严重的发病情况。文献中有几例报道，但此前尚无免疫介导性小脑炎的病例描述。我们报告一例急性小脑炎病例，该病例发生在一名20岁患有原发性难治性霍奇金淋巴瘤的男性患者身上，他正在接受免疫检查点抑制剂纳武单抗治疗。在接受3个周期的纳武单抗治疗后，患者急性起病，出现头痛、共济失调、恶心和呕吐，影像学检查发现小脑水肿、早期扁桃体疝和早期脑积水。怀疑为免疫介导性小脑脑炎，遂开始高剂量地塞米松治疗（每6小时8毫克）。在地塞米松治疗的4天内，他的症状有了很大改善，在4周的减量期后症状几乎完全缓解。对其病情的鉴别诊断包括病毒性小脑炎和副肿瘤性小脑变性。在怀疑由免疫检查点抑制剂治疗引起的小脑脑炎中，迅速识别并早期开始高剂量皮质类固醇治疗对于症状缓解和治疗成功至关重要，包括预防脑积水和扁桃体疝。目前，尚无基于证据的指南来指导皮质类固醇的初始剂量、类型或疗程。需要对与免疫检查点抑制剂治疗相关的小脑脑炎的发病机制和治疗进行进一步研究，以有效治疗这种罕见的致残性疾病。
