GABA-A Receptor Encephalitis After Autologous Hematopoietic Stem Cell Transplant forMultiple Myeloma: Three Cases and Literature Review.

Yoji Hoshina, Jonathan Galli, Ka-Ho Wong, Tibor Kovacsovics, Mary Steinbach, Karen L Salzman, Joseph Scott McNally, Eric Lancaster, M Mateo Paz Soldán, Stacey L Clardy
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Abstract

Background and objectives: The relationship between autologous hematopoietic stem cell transplant (aHSCT) for multiple myeloma (MM) and anti-GABAA receptor (GABAAR) encephalitis is unknown. We aimed to describe the clinical features, diagnostic process, and outcome of 3 cases of anti-GABAAR encephalitis in patients with a history of prior aHSCT for MM.

Methods: A case series of 3 patients. Anti-GABAAR antibody was tested at the University of Pennsylvania Laboratory.

Results: 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-GABAAR 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-GABAAR antibodies in all 3 cases. Cases 1 and 3 also tested positive for anti-GABAAR 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.

Discussion: We highlight the importance of considering anti-GABAAR 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.

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自体造血干细胞移植致多发性骨髓瘤后GABA-A受体脑炎3例及文献复习
背景与目的:自体造血干细胞移植(aHSCT)治疗多发性骨髓瘤(MM)与抗gabaa受体(GABAAR)脑炎之间的关系尚不清楚。我们的目的是描述3例抗gabaar脑炎患者的临床特征、诊断过程和预后,这些患者既往有aHSCT病史。抗gabaar抗体在宾夕法尼亚大学实验室进行了测试。结果:患者均为男性,脑炎发病年龄分别为52岁(例1)、61岁(例2)、62岁(例3)。从aHSCT完成到脑炎发作的时间分别为43个月、18个月和9个月。所有3例患者均出现新的癫痫发作和认知功能改变。其他症状包括病例1和2的头痛和视力模糊,病例3的难治性眩晕和躁狂。脑MRI显示所有3例患者均出现非增强的多灶t2加权/液体衰减反转恢复皮层和皮层下高信号。病例2和3在开始免疫调节治疗前进行了脑活检,结果显示非特异性脑炎伴白质星形胶质细胞增生;这2例患者分别在首次出现22天和3个月后开始接受抗gabaar脑炎的免疫治疗。病例1在发病后8天内开始经验性免疫治疗,不需要脑活检,给予特征性MRI成像。脑脊液分析显示,所有3例患者均存在抗gabaar抗体。病例1和病例3血清抗gabaar抗体阳性(病例2未进行血清检测)。病例1和病例2在1年内恢复全职工作。病例3报告偶有肌阵挛样运动。讨论:我们强调在癫痫发作、多灶性非增强性脑病变和有MM aHSCT病史的患者中考虑抗gabaar脑炎的重要性。在aHSCT后恢复的MM患者中意识到这一点可能是至关重要的,因为及时识别可以避免脑活检和治疗延误。在等待自身抗体结果的同时快速启动免疫治疗可能会改善功能结果。
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