Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Department of General Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Acta Neurol Taiwan. 2021 Sep 30;30(3):118-122.
Anti-dipeptidyl-peptidase-like protein 6 (DPPX) encephalitis is a rare but treatable autoimmune disorder, characterized by gastrointestinal symptoms, cognitive dysfunction, and central nervous system hyperexcitability.
Herein, we report a case of an 80-year-old male patient who presented with unexplained diarrhea, weight loss, rapidly progressive dementia, tremors, and myoclonus. His serum tested positive for anti-DPPX antibodies. He was treated with plasma exchange, oral prednisolone, and azathioprine. All his symptoms improved substantially after treatment.
Early recognition of anti-DPPX encephalitis is important because it can be treated with immunotherapy. To the best of our knowledge, this is the first reported case of anti-DPPX encephalitis in Taiwan.
抗二肽基肽酶样蛋白 6（DPPX）脑炎是一种罕见但可治疗的自身免疫性疾病，其特征为胃肠道症状、认知功能障碍和中枢神经系统过度兴奋。
在此，我们报告一例 80 岁男性患者，其表现为不明原因腹泻、体重减轻、进行性痴呆、震颤和肌阵挛。其血清抗 DPPX 抗体检测阳性。患者接受了血浆置换、口服泼尼松龙和硫唑嘌呤治疗。治疗后，他的所有症状均明显改善。
早期识别抗 DPPX 脑炎很重要，因为它可以通过免疫疗法治疗。据我们所知，这是首例在台湾报道的抗 DPPX 脑炎病例。
