Aseptic meningoencephalitis during nusinersen therapy in a patient with type III spinal muscular atrophy: a case report

IF 2.7 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-05-01 DOI:10.1016/j.nmd.2025.105365

Lukasz Kolakowski , Olivia Zschau , Jérôme Bonzon , Anthony de Vere-Tyndall , Marina Herwerth , Christoph Neuwirth , Michael Weller , Patrick Roth , Bettina Schreiner , Veronika Kana

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Abstract

Nusinersen, an intrathecally-administered antisense oligonucleotide for the treatment of spinal muscular atrophy (SMA), may rarely cause mild aseptic meningitis early in treatment. We report a severe late-onset aseptic brain stem meningoencephalitis in a 42-year-old man with type III SMA, occurring 38 months after starting nusinersen. The patient showed clinical and radiological signs of brain stem meningoencephalitis with significant CSF neutrophilic pleocytosis, despite negative infectious disease tests. After high-dose steroids and tapering, symptoms and imaging findings resolved. This case suggests a potential link between nusinersen and aseptic meningoencephalitis in adult SMA, extending previous reports of rare drug-induced aseptic meningitis. Physicians should consider this as a possible adverse event in patients undergoing intrathecal nusinersen therapy.

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无菌性脑膜脑炎在nusinsen治疗的患者与III型脊髓性肌萎缩：1例报告

Nusinersen是一种用于治疗脊髓性肌萎缩症（SMA）的鞘内注射反义寡核苷酸，在治疗早期很少引起轻度无菌性脑膜炎。我们报告一个严重的晚发性无菌性脑干脑膜脑炎在一个42岁的男人与III型SMA，发生38个月后开始使用nusinsen。患者表现为脑干脑膜脑炎的临床和影像学征象，伴明显的脑脊液嗜中性粒细胞增多症，尽管传染病检查呈阴性。在大剂量类固醇和减量治疗后，症状和影像学表现得到缓解。这一病例提示nusinersen和成人SMA无菌性脑膜脑炎之间的潜在联系，扩展了以前罕见的药物引起的无菌性脑膜炎的报道。在接受鞘内麻醉治疗的患者中，医生应该考虑到这是一个可能的不良事件。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.