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可治疗性快速进展性痴呆:台湾首例抗二肽基肽酶样蛋白 6 脑炎病例报告。

Treatable Rapid Progressive Dementia: A First Case Report of Anti-dipeptidyl-peptidase-like Protein 6 Encephalitis in Taiwan.

机构信息

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.

出版信息

Acta Neurol Taiwan. 2021 Sep 30;30(3):118-122.

Abstract

PURPOSE

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.

CASE REPORT

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.

CONCLUSION

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.

摘要

目的

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。

病例报告

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。

结论

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。

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