PDF(Pubmed)
Abstract：
UNASSIGNED: Anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis is a rare autoimmune encephalitis, and clinical and experimental information regarding this disease is limited. We conducted this study to comprehensively describe the clinical characteristics, ancillary test results, neuroimaging results, and treatment response in a group of Chinese patients with anti-DPPX encephalitis for better understanding this disease.
UNASSIGNED: We recruited 14 patients who tested positive for anti-DPPX antibodies in the serum and/or cerebrospinal fluid from 11 medical centers between March 2021 and June 2023. This retrospective study evaluated data on symptoms, autoantibody test, auxiliary examinations, treatments, and outcomes.
UNASSIGNED: The average age at diagnosis was 45.93 ± 4.62 years (range: 11-72 years), and 9 of the 14 patients were males. The main symptoms included cognitive impairment (50.0%, 7/14), central nervous system hyperexcitability (42.9%, 6/14), gastrointestinal dysfunction (35.7%, 5/14), and psychiatric disorders (35.7%, 5/14). Notably, we discovered specific findings on 18F-fluorodeoxyglucose positron-emission tomography (PET)/magnetic resonance imaging in two patients. Co-existing autoantibodies were identified in two patients. Parainfection was identified in four patients. One patient had other autoimmune diseases, and one had tumor. Eleven patients received immunotherapy and most patients improved at discharge. Surprisingly, three male patients but no female patients relapsed during the 6 months of follow-up.
UNASSIGNED: The development and outcome of anti-DPPX encephalitis are variable. Male patients were predominant in our cohort. The most common symptoms were the classical triad of prodromal gastrointestinal dysfunction, cognitive and mental disorders, and central nervous system hyperexcitability. Infections, immune dysregulation, and tumors may be important etiologies. Long-term monitoring of disease development should be done in male patients. Overall, our results highlight novel clinical characteristics of anti-DPPX encephalitis.
UNASSIGNED: We recruited 14 patients who tested positive for anti-DPPX antibodies in the serum and/or cerebrospinal fluid from 11 medical centers between March 2021 and June 2023. This retrospective study evaluated data on symptoms, autoantibody test, auxiliary examinations, treatments, and outcomes.
UNASSIGNED: The average age at diagnosis was 45.93 ± 4.62 years (range: 11-72 years), and 9 of the 14 patients were males. The main symptoms included cognitive impairment (50.0%, 7/14), central nervous system hyperexcitability (42.9%, 6/14), gastrointestinal dysfunction (35.7%, 5/14), and psychiatric disorders (35.7%, 5/14). Notably, we discovered specific findings on 18F-fluorodeoxyglucose positron-emission tomography (PET)/magnetic resonance imaging in two patients. Co-existing autoantibodies were identified in two patients. Parainfection was identified in four patients. One patient had other autoimmune diseases, and one had tumor. Eleven patients received immunotherapy and most patients improved at discharge. Surprisingly, three male patients but no female patients relapsed during the 6 months of follow-up.
UNASSIGNED: The development and outcome of anti-DPPX encephalitis are variable. Male patients were predominant in our cohort. The most common symptoms were the classical triad of prodromal gastrointestinal dysfunction, cognitive and mental disorders, and central nervous system hyperexcitability. Infections, immune dysregulation, and tumors may be important etiologies. Long-term monitoring of disease development should be done in male patients. Overall, our results highlight novel clinical characteristics of anti-DPPX encephalitis.
摘要：
■抗二肽基肽酶样蛋白-6（DPPX）脑炎是一种罕见的自身免疫性脑炎，关于这种疾病的临床和实验信息有限。我们进行了这项研究，以全面描述临床特征，辅助测试结果，神经影像学检查结果，以及一组中国抗DPPX脑炎患者的治疗反应，以更好地了解这种疾病。
■我们在2021年3月至2023年6月之间从11个医疗中心招募了14名血清和/或脑脊液中抗DPPX抗体检测呈阳性的患者。这项回顾性研究评估了症状数据，自身抗体测试,辅助考试,治疗,和结果。
■诊断时的平均年龄为45.93±4.62岁（范围：11-72岁），14例患者中有9例为男性。主要症状包括认知障碍(50.0%,7/14),中枢神经系统兴奋过度(42.9%，6/14),胃肠功能障碍（35.7%，5/14),和精神疾病(35.7%，5/14）。值得注意的是,我们在两名患者中发现了18F-氟代脱氧葡萄糖正电子发射断层扫描(PET)/磁共振成像的具体发现.在两名患者中鉴定出共存的自身抗体。在四名患者中发现了共感染。一名患者患有其他自身免疫性疾病，还有一个有肿瘤.11名患者接受了免疫治疗，大多数患者在出院时有所改善。令人惊讶的是,在6个月的随访期间,3例男性患者复发,无女性患者复发.
■抗DPPX脑炎的发展和结果是可变的。男性患者在我们的队列中占主导地位。最常见的症状是前驱胃肠功能障碍的经典三联征，认知和精神障碍，和中枢神经系统兴奋过度。感染,免疫失调，肿瘤可能是重要的病因。对男性患者应进行疾病发展的长期监测。总的来说,我们的研究结果突出了抗DPPX脑炎的新临床特征.
■我们在2021年3月至2023年6月之间从11个医疗中心招募了14名血清和/或脑脊液中抗DPPX抗体检测呈阳性的患者。这项回顾性研究评估了症状数据，自身抗体测试,辅助考试,治疗,和结果。
■诊断时的平均年龄为45.93±4.62岁（范围：11-72岁），14例患者中有9例为男性。主要症状包括认知障碍(50.0%,7/14),中枢神经系统兴奋过度(42.9%，6/14),胃肠功能障碍（35.7%，5/14),和精神疾病(35.7%，5/14）。值得注意的是,我们在两名患者中发现了18F-氟代脱氧葡萄糖正电子发射断层扫描(PET)/磁共振成像的具体发现.在两名患者中鉴定出共存的自身抗体。在四名患者中发现了共感染。一名患者患有其他自身免疫性疾病，还有一个有肿瘤.11名患者接受了免疫治疗，大多数患者在出院时有所改善。令人惊讶的是,在6个月的随访期间,3例男性患者复发,无女性患者复发.
■抗DPPX脑炎的发展和结果是可变的。男性患者在我们的队列中占主导地位。最常见的症状是前驱胃肠功能障碍的经典三联征，认知和精神障碍，和中枢神经系统兴奋过度。感染,免疫失调，肿瘤可能是重要的病因。对男性患者应进行疾病发展的长期监测。总的来说,我们的研究结果突出了抗DPPX脑炎的新临床特征.
