Shiraishi Wataru, Umemura Takeru, Nakayama Yuuki, Yamada Yui, Shijo Masahiro, Hashimoto Tetsuya
Department of Neurology, Kokura Memorial Hospital, Kitakyushu, Japan.
Department of Internal Medicine, Shiraishi Internal Medicine Clinic, Nogata, Japan.
Front Neurol. 2022 Jul 11;13:946180. doi: 10.3389/fneur.2022.946180. eCollection 2022.
Paraneoplastic tumefactive demyelination (TD) is a rare disorder of the central nervous system that can be challenging to diagnose. Here, we describe a 32-year-old Japanese man with a TD associated with testicular seminoma. He presented with symptoms of right-sided motor and sensory impairment 2 days after vaccination for coronavirus disease 2019 (COVID-19). Brain magnetic resonance imaging (MRI) showed a high-intensity lesion in the left internal capsule. He had a 3-year history of enlargement of the left testicle. Blood examination showed tumor marker elevation and the presence of anti-amphiphysin antibodies. Whole-body computed tomography (CT) revealed mass lesions in the left testicle and enlargement of the retroperitoneal lymph nodes. Radical orchiectomy was performed. As the pathology showed testicular seminoma, chemotherapy was administered. After surgery, his neurological symptoms deteriorated. MRI revealed that the brain lesion had enlarged and progressed to a tumefactive lesion without gadolinium enhancement. The cerebrospinal fluid (CSF) examination was normal without pleocytosis or protein elevation. Steroid pulse therapy was added; however, his symptoms did not improve. A brain stereotactic biopsy was performed and the sample showed demyelinating lesions without malignant cells. As the initial corticosteroid therapy was ineffective, gamma globulin therapy was administered in parallel with chemotherapy, and the clinical symptoms and imaging findings were partially ameliorated. TD seldom appears as a paraneoplastic neurological syndrome. In addition, there are few reports of COVID-19 vaccination-associated demyelinating disease. Clinicians should recognize paraneoplastic TD, and the further accumulation of similar cases is needed.
副肿瘤性瘤样脱髓鞘病变（TD）是一种罕见的中枢神经系统疾病，诊断具有挑战性。在此，我们描述一名32岁的日本男性，其患有与睾丸精原细胞瘤相关的TD。他在接种2019冠状病毒病（COVID-19）疫苗2天后出现右侧运动和感觉障碍症状。脑部磁共振成像（MRI）显示左侧内囊有高强度病变。他有左侧睾丸肿大3年的病史。血液检查显示肿瘤标志物升高且存在抗 amphiphysin 抗体。全身计算机断层扫描（CT）显示左侧睾丸有肿块病变以及腹膜后淋巴结肿大。进行了根治性睾丸切除术。病理显示为睾丸精原细胞瘤，于是给予化疗。手术后，他的神经症状恶化。MRI显示脑部病变扩大并进展为无钆增强的瘤样病变。脑脊液（CSF）检查正常，无细胞增多或蛋白质升高。加用了类固醇冲击疗法；然而，他的症状并未改善。进行了脑立体定向活检，样本显示为脱髓鞘病变，无恶性细胞。由于初始的皮质类固醇治疗无效，在化疗的同时给予了丙种球蛋白治疗，临床症状和影像学表现部分得到改善。TD很少表现为副肿瘤性神经综合征。此外，关于COVID-19疫苗接种相关脱髓鞘疾病的报道很少。临床医生应认识到副肿瘤性TD，并且需要进一步积累类似病例。
