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进行性多发性颅神经病变作为 CAR-T 神经毒性的表现

英文原题:Progressive Multiple Cranial Neuropathies as a Manifestation of CAR-T Neurotoxicity.

查看英文原题

Progressive Multiple Cranial Neuropathies as a Manifestation of CAR-T Neurotoxicity.

PubMed 2025/10/25(内容时间) Neurohospitalist Q4 · IF 0.6(JCR 2025)

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中文摘要

本文报告一例多发性骨髓瘤患者接受CAR-T 细胞治疗后,迟发性多发性颅神经病变作为神经毒性表现。CAR-T 治疗后的免疫效应细胞相关神经毒性综合征(ICANS)虽已有充分报道,但典型表现为脑病、癫痫、失语、震颤、头痛和脑水肿。既往有CAR-T 神经毒性继发单侧孤立性面神经病变的报道,但尚未见进行性多发性颅神经病变。本病例为一名75岁男性,在多发性骨髓瘤CAR-T 治疗开始后19天出现左侧面神经麻痹。增强脑MRI显示对侧右侧面神经强化,左侧面瘫给予类固醇和伐昔洛韦治疗7天。面瘫仍持续,并进展为双侧面神经及左侧第VI脑神经受累;至CAR-T 治疗后第31天,检查发现左眼外展受限,面部运动几乎消失。

复查增强脑MRI显示双侧面神经轻度强化。广泛的血清和脑脊液检查均无异常。最初口服类固醇治疗7天无效。考虑到类固醇可能影响CAR-T 疗效,治疗剂量和疗程受到限制。曾考虑使用阿那白滞素,但最终未给药。随后改用静脉大剂量类固醇,继而长程逐渐减量使用泼尼松,症状改善:发病后2.5个月(完成治疗后2周)第VI脑神经麻痹消退;发病后5.5个月(完成治疗后3.5个月)双侧面瘫中度改善。CAR-T 神经毒性可表现为进行性多发性颅神经病变。目前此类病例的最佳治疗尚不明确;但该患者在较长时间随访期间接受类固醇和面部康复治疗后有所好转。

展开英文摘要原文

To describe a case of delayed onset multiple cranial neuropathies as a manifestation of neurotoxicity after chimeric antigen receptor T-cell (CAR-T) therapy for multiple myeloma. While ICANS following CAR-T is a well-reported complication, it classically presents with encephalopathy, seizures, dysphasia, tremors, headache, and cerebral edema. Isolated unilateral facial neuropathies secondary to CAR-T neurotoxicity have been described, but progressive multiple cranial neuropathies have not.

Herein, a 75-year-old male presented with left facial nerve palsy 19 days after initiating CAR-T therapy for multiple myeloma. Contrasted brain MRI showed contralateral right facial nerve enhancement, and his left facial palsy was treated with steroids and valacyclovir for 7 days. The facial palsy persisted and progressed to involve bilateral facial nerves and left cranial nerve VI by 31 days post-CAR-T. Specifically, his exam showed impaired abduction of left eye and nearly absent facial movement. Repeat contrasted MRI brain showed mild enhancement of bilateral facial nerves. Extensive serum and CSF testing was unremarkable. Initial treatment with oral steroids for 7 days was ineffective.

Concern regarding the impact of steroids on CAR-T efficacy influenced treatment dose and duration. Anakinra was considered but not given. Subsequent treatment with intravenous high dose steroids, followed by a prolonged prednisone taper, led to resolution of CN VI palsy at 2. 5 months from onset (2 weeks after completed therapy), and moderate improvement of bilateral facial palsy 5.

5 months from onset (3. 5 months after completed therapy). CAR-T neurotoxicity can present with progressive multiple cranial neuropathies. The best treatment of these cases is unknown; however, this patient improved in the context of corticosteroids and facial rehabilitation over a prolonged period of follow-up.

论文信息

作者
Lazzari ZT、Gandh AS、Sannananja B、Belagaje SR、Hutto SK
单位
Department of Neurology, School of Medicine, Emory University, Atlanta, GA, USA.United States
文献类型
病例报告
期刊
The Neurohospitalist2025 Oct 25
原文标识
PubMed 41147017 · DOI 10.1177/19418744251393076