Department of Neurology, Hospital of Lithuanian University of Health Sciences Kauno Klinikos, Kaunas, Lithuania.
Department of Neurology, Lithuanian University of Health Sciences Clinical, Kaunas, Lithuania.
BMC Neurol. 2022 Aug 19;22(1):306. doi: 10.1186/s12883-022-02839-3.
Fingolimod is associated with an increased risk of developing progressive multifocal leukoencephalopathy (PML); however, its discontinuation may cause severe immune reconstitution inflammatory syndrome (IRIS). As both of these conditions (especially fingolimod induced PML) are rarely described in medical case reports distinguishing between PML-IRIS and MS-IRIS may be diagnostically challenging.
We report a patient with severe clinical decline (Expanded Disability Status Scale (EDSS) increasing from 3.5 to 7.5) and multiple, large, contrast-enhancing lesions on brain magnetic resonance imaging (MRI) a few months after fingolimod withdrawal. The diagnostic possibilities included IRIS due to fingolimod withdrawal versus PML-IRIS. The JC virus (JCV) antibody index was positive (2.56); however, cerebrospinal fluid (CSF) JCV real-time polymerase chain reaction (JCV-PCR) was negative and brain biopsy was not performed. After a long course of aggressive treatment (several pulsed methylprednisolone infusions, plasmapheresis, intravenous dexamethasone, oral mirtazapine) the patient gradually recovered (EDSS 2.5) and MRI lesions decreased.
This case report demonstrates the importance of monitoring patients carefully after the discontinuation of fingolimod for PML-IRIS and rebound MS with IRIS as these conditions may manifest similarly.
芬戈莫德会增加发生进行性多灶性白质脑病（PML）的风险；然而，停用芬戈莫德可能会导致严重的免疫重建炎症综合征（IRIS）。由于这两种情况（特别是由芬戈莫德引起的 PML）在医学病例报告中很少描述，因此区分 PML-IRIS 和 MS-IRIS 可能具有诊断挑战性。
我们报告了一例患者，在停用芬戈莫德几个月后出现严重的临床恶化（扩展残疾状态量表（EDSS）从 3.5 增加到 7.5）和大脑磁共振成像（MRI）上多个大的对比增强病变。诊断的可能性包括因停用芬戈莫德而导致的 IRIS 与 PML-IRIS。JC 病毒（JCV）抗体指数阳性（2.56）；然而，脑脊液（CSF）JCV 实时聚合酶链反应（JCV-PCR）为阴性，未进行脑活检。经过长时间的积极治疗（多次脉冲甲基强的松龙输注、血浆置换、静脉注射地塞米松、口服米氮平），患者逐渐恢复（EDSS 2.5），MRI 病变减少。
本病例报告表明，在停用芬戈莫德后，密切监测患者的 PML-IRIS 和反弹 MS 与 IRIS 非常重要，因为这些情况可能表现相似。
