Dai Yijie, Zheng Yang, Zhu Jiahui, Ding Jiao, Qiu Kefan, Tang Bo
Zhejiang Chinese Medical University, China.
The First Affiliated Hospital of Zhejiang Chinese Medical University, China.
Heliyon. 2024 Feb 29;10(5):e27413. doi: 10.1016/j.heliyon.2024.e27413. eCollection 2024 Mar 15.
Dipeptidyl-peptidase-like protein 6 (DPPX) antibody-mediated encephalitis is a rare type of autoimmune encephalitis (AE), which mainly manifests as diarrhea accompanied by weight loss, cognitive decline, epileptic seizures, and even psychiatric symptoms. Remarkably, it is also reported to be associated with tumors, predominantly B-cell lymphoma. Overall, the AE remains uncharacterized clinically and its long-term prognosis remains elusive. Herein, we report the first case of DPPX antibody-mediated AE secondary to breast cancer. Importantly, it substantially improves after aggressive immunotherapy. Our case highlights DPPX antibody-mediated AE as a paraneoplastic syndrome and discusses the pearls in its diagnosis and management.
二肽基肽酶样蛋白6(DPPX)抗体介导的脑炎是一种罕见的自身免疫性脑炎(AE),主要表现为腹泻伴体重减轻、认知功能下降、癫痫发作,甚至精神症状。值得注意的是,也有报道称其与肿瘤有关,主要是B细胞淋巴瘤。总体而言,这种AE在临床上仍未得到充分认识,其长期预后也尚不明确。在此,我们报告首例继发于乳腺癌的DPPX抗体介导的AE。重要的是,积极免疫治疗后病情显著改善。我们的病例突出了DPPX抗体介导的AE作为一种副肿瘤综合征,并讨论了其诊断和管理要点。