From the Department of Neurology (Y.H., J.G., K.-H.W., M.M.P.S., S.L.C.), University of Utah Health; Department of Neurology (J.G., M.M.P.S., S.L.C.), Veterans Affairs Medical Center, UT; Division of Hematology and Hematologic Malignancies (T.K., M.S.), Huntsman Cancer Institute, University of Utah Health; Department of Radiology and Imaging Sciences (K.L.S., J.S.M.), University of Utah Health; and Department of Neurology (E.L.), University of Pennsylvania, Philadelphia.
Neurol Neuroimmunol Neuroinflamm. 2022 Aug 26;9(6). doi: 10.1212/NXI.0000000000200024. Print 2022 Nov.
The relationship between autologous hematopoietic stem cell transplant (aHSCT) for multiple myeloma (MM) and anti-GABA receptor (GABAR) encephalitis is unknown. We aimed to describe the clinical features, diagnostic process, and outcome of 3 cases of anti-GABAR encephalitis in patients with a history of prior aHSCT for MM.
A case series of 3 patients. Anti-GABAR antibody was tested at the University of Pennsylvania Laboratory.
The patients were all male, aged 52 (case 1), 61 (case 2), and 62 (case 3) years at encephalitis symptom onset. The duration between completion of aHSCT and the onset of encephalitis was 43, 18, and 9 months, respectively. All 3 patients presented with new seizures and altered cognitive function. Other symptoms included headache and visual obscurations in cases 1 and 2 and intractable vertigo and mania in case 3. Brain MRI demonstrated nonenhancing multifocal T2-weighted/fluid-attenuated inversion recovery cortical and subcortical hyperintensities in all 3 patients. Cases 2 and 3 underwent brain biopsy before initiating immunomodulatory therapy, which demonstrated nonspecific encephalitis with astrogliosis in the white matter; these 2 patients were started on immunotherapy for the treatment of anti-GABAR encephalitis after 22 days and 3 months, respectively, from the first presentation. Case 1 was started on empiric immunotherapy within 8 days of presentation without requiring brain biopsy, given characteristic MRI imaging. CSF analysis demonstrated the presence of anti-GABAR antibodies in all 3 cases. Cases 1 and 3 also tested positive for anti-GABAR antibodies in the serum (serum test was not performed in case 2). Cases 1 and 2 recovered to work full-time within 1 year. Case 3 reported occasional myoclonic-like movement.
We highlight the importance of considering anti-GABAR encephalitis in patients with seizures, multifocal nonenhancing brain lesions, and a history of aHSCT for MM. Awareness in recovered post-aHSCT patients with MM may be crucial because prompt recognition can avoid brain biopsy and delays in treatment. The rapid initiation of immunotherapy while awaiting autoantibody results will likely improve functional outcomes.
自体造血干细胞移植（aHSCT）治疗多发性骨髓瘤（MM）与抗 GABA 受体（GABAR）脑炎之间的关系尚不清楚。我们旨在描述 3 例既往有 MM 病史行 aHSCT 后发生抗 GABAR 脑炎患者的临床特征、诊断过程和结局。
我们对 3 例患者进行了病例系列研究。抗 GABAR 抗体检测在宾夕法尼亚大学实验室进行。
3 例患者均为男性，脑炎症状发作时年龄分别为 52 岁（病例 1）、61 岁（病例 2）和 62 岁（病例 3）。aHSCT 完成后至脑炎发作的时间分别为 43、18 和 9 个月。所有 3 例患者均表现为新发癫痫发作和认知功能改变。其他症状包括病例 1 和 2 的头痛和视力障碍，以及病例 3 的难治性眩晕和躁狂。3 例患者的脑 MRI 均显示非增强性多灶性 T2 加权/液体衰减反转恢复皮质和皮质下高信号。病例 2 和 3 在开始免疫调节治疗前进行了脑活检，结果显示白质内存在非特异性脑炎和星形胶质增生；这 2 例患者分别在首发后 22 天和 3 个月开始接受抗 GABAR 脑炎的免疫治疗。病例 1 在出现症状后 8 天内开始经验性免疫治疗，而无需进行脑活检，因为其 MRI 影像学具有特征性。CSF 分析显示所有 3 例患者均存在抗 GABAR 抗体。病例 1 和 3 的血清也检测到抗 GABAR 抗体（病例 2 未进行血清检测）。病例 1 和 2 在 1 年内恢复全职工作。病例 3 报告偶尔出现肌阵挛样运动。
我们强调了在多发性骨髓瘤患者出现癫痫发作、多灶性非增强性脑病变和既往接受 aHSCT 治疗时，应考虑抗 GABAR 脑炎。在多发性骨髓瘤患者接受 aHSCT 后康复期间，对这种疾病的认识可能非常重要，因为及时识别可以避免脑活检和治疗延迟。在等待自身抗体结果的同时，快速开始免疫治疗可能会改善功能结局。
