Zhang Weihe, Cui Lei, Dong Mingrui, Tian Zhaohui, Jiao Yujuan, Jiao Jinsong
Department of Neurology, China-Japan Friendship Hospital, Beijing, China.
Front Neurol. 2020 Aug 18;11:730. doi: 10.3389/fneur.2020.00730. eCollection 2020.
The area postrema syndrome (APS) is a unique diagnostic criterion for neuromyelitis optica spectrum disorders (NMOSD). However, APS has rarely been reported in cases of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). A 36-year-old woman presented with APS and clinical features of diffuse central nervous system involvement during the early stage of the disease. Owing to the absence of serum aquaporin 4 antibodies, she was initially misdiagnosed as a case of seronegative NMOSD. However, the distinct neuroimaging characteristics [symmetrical small punctuate gadolinium enhancing lesions (pepper-like)], typical clinical/radiological relapse, and intense steroid-dependence in this case, prompted us to correct the diagnosis as probable CLIPPERS. To prevent relapse, long-term oral steroids and an immunosuppressive agent were administered. CLIPPERS may present as APS, and should be considered in the differential diagnosis of NMOSD.
最后区综合征（APS）是视神经脊髓炎谱系障碍（NMOSD）的一项独特诊断标准。然而，在对类固醇有反应的桥脑周围血管增强的慢性淋巴细胞性炎症（CLIPPERS）病例中，APS鲜有报道。一名36岁女性在疾病早期出现APS及弥漫性中枢神经系统受累的临床特征。由于血清水通道蛋白4抗体阴性，她最初被误诊为血清阴性NMOSD病例。然而，该病例独特的神经影像学特征[对称的小斑点状钆增强病变（胡椒样）]、典型的临床/放射学复发以及对类固醇的强烈依赖，促使我们将诊断修正为可能的CLIPPERS。为防止复发，给予了长期口服类固醇和一种免疫抑制剂。CLIPPERS可能表现为APS，在NMOSD的鉴别诊断中应予以考虑。
