Infratentorial onset of progressive multifocal leukoencephalopathy in a patient with systematic lupus erythematosus complicated with lymphoma: a case report

Infratentorial onset of progressive multifocal leukoencephalopathy in a patient with systematic lupus erythematosus complicated with lymphoma: a case report
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系统性红斑狼疮合并淋巴瘤患者幕下起病的进行性多灶性白质脑病:一例病例报告

DOI:
10.1080/24725625.2021.1899763
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发表时间:
2021
影响因子:
0.8
通讯作者:
Kuwana Masataka
Kuwana Masataka
中科院分区:
--
文献类型:
--
作者:
Sakuraba Mita;Watanabe Shinji;Nishiyama Yasuhiro;Takahashi Kenta;Nakamichi Kazuo;Suzuki Mikito;Nawata Takashi;Komai Kota;Gono Takahisa;Takeno Mitsuhiro;Suzuki Tadaki;Kimura Kazumi;Kuwana Masataka

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进行性多灶性白质脑病(PML)是一种罕见的中枢神经系统机会性感染,由JC病毒(JCV)重新激活引起。典型的PML在磁共振成像(MRI)上显示幕上白质内融合的、双侧但不对称的皮质下病变。我们报告一位50岁女性系统性红斑狼疮合并淋巴瘤,发展为PML,其脑部MRI表现不典型,仅限于幕下区域。在完成包括利妥昔单抗在内的淋巴瘤缓解诱导治疗后,她出现右侧面部麻木,包括舌头、右手笨拙和步态障碍。脑MRI显示仅局限于小脑和脑干的孤立性病变,但脑脊液检查或脑活检标本的暂时性组织学评估不能做出明确诊断。尽管接受了甲基强的松龙冲击治疗,但她的神经功能缺陷逐渐恶化。一个月后,对她的脑脊液和脑活检标本的深入分析证实了JCV的存在。最终,MRI上局限性的单侧新月形小脑病变扩展到对侧小脑、小脑中脑、桥脑和中脑,最终发展为多灶性侵犯大脑半球的白质。我们的病例表明,PML可能首先在免疫功能低下的患者中表现为孤立性幕下病变。
Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system caused by reactivation of JC virus (JCV). Typical PML shows confluent, bilateral but asymmetric, subcortical lesions in the supratentorial white matter on magnetic resonance imaging (MRI). We report here a 50-year-old woman with systemic lupus erythematosus complicated with lymphoma who developed PML with atypical brain MRI findings limited to the infratentorial area at presentation. She presented with numbness on the right side of the face, including her tongue, clumsiness of the right hand, and gait disturbance, after completion of remission induction therapy for lymphoma, including rituximab. Brain MRI demonstrated a solitary lesion limited to the cerebellum and brainstem, but a definitive diagnosis could not be made from cerebrospinal fluid study or tentative histologic evaluation of brain biopsy specimens. Despite methylprednisolone pulse therapy, her neurological deficits progressively worsened. One month later, in-depth analysis of her cerebrospinal fluid and brain biopsy specimens confirmed the presence of JCV. Eventually, the localised unilateral crescent-shaped cerebellar lesions on MRI expanded to the contralateral cerebellum, middle cerebellar hemisphere, pons, and midbrain and finally developed multifocal invasion into the white matter of the cerebral hemispheres. Our case suggests that PML could first present with a solitary infratentorial lesion in immunocompromised patients.
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