Abstract：
BACKGROUND: Anti-IgLON5 disease is a rare but potentially reversible cause of cognitive impairment, sleep disturbances, dysautonomia, and movement disorders. It is an autoimmune encephalitis which, due to its insidious onset, could mimic neurodegenerative disorders, and multiple symptoms overlap with those seen in dementia with Lewy bodies (DLB). We hypothesized that the symptomatology and findings in patients with anti-IgLON5 disease overlapped with that of DLB.
OBJECTIVE: To assess the commonality of features in anti-IgLON5 disease and DLB and identify potential red flags for anti-IgLON5 disease in patients undergoing diagnostic evaluation for DLB.
METHODS: We searched in MEDLINE, Web of Science, and Embase from inception on December the 8th, 2022 with the search term \"IgLON5\". We performed a systematic review of case reports and case series of anti-IgLON5 disease, and two reviewers independently extracted data on symptoms and findings. Frequencies of symptoms were compared with consensus criteria for DLB.
RESULTS: We included 57 studies with 127 individual case reports of anti-IgLON5 disease (mean age 63 years at diagnosis, median symptom duration 2 years). Cognitive dysfunction was reported in 45% of cases, REM-sleep behavioral disorder in 15%, and 14% had parkinsonism. Respiratory insufficiency was reported in 37%, and bulbar symptoms in 67%.
CONCLUSIONS: We found a significant overlap between anti-IgLON5 disease and DLB. We propose that anti-IgLON5 disease should be considered in young patients with DLB with chorea, gaze palsy, early dysphagia, or prominent respiratory symptoms. Our study contributes to the emerging knowledge on symptoms and biomarkers in anti-IgLON5 disease.
OBJECTIVE: To assess the commonality of features in anti-IgLON5 disease and DLB and identify potential red flags for anti-IgLON5 disease in patients undergoing diagnostic evaluation for DLB.
METHODS: We searched in MEDLINE, Web of Science, and Embase from inception on December the 8th, 2022 with the search term \"IgLON5\". We performed a systematic review of case reports and case series of anti-IgLON5 disease, and two reviewers independently extracted data on symptoms and findings. Frequencies of symptoms were compared with consensus criteria for DLB.
RESULTS: We included 57 studies with 127 individual case reports of anti-IgLON5 disease (mean age 63 years at diagnosis, median symptom duration 2 years). Cognitive dysfunction was reported in 45% of cases, REM-sleep behavioral disorder in 15%, and 14% had parkinsonism. Respiratory insufficiency was reported in 37%, and bulbar symptoms in 67%.
CONCLUSIONS: We found a significant overlap between anti-IgLON5 disease and DLB. We propose that anti-IgLON5 disease should be considered in young patients with DLB with chorea, gaze palsy, early dysphagia, or prominent respiratory symptoms. Our study contributes to the emerging knowledge on symptoms and biomarkers in anti-IgLON5 disease.
摘要：
背景：抗IgLON5病是一种罕见但可能可逆的认知障碍原因，睡眠障碍,自主神经失调,和运动障碍。这是一种自身免疫性脑炎，由于其阴险的发作，可以模仿神经退行性疾病，多种症状与路易体痴呆（DLB）的症状重叠。我们假设抗IgLON5病患者的症状和发现与DLB重叠。
目的：评估抗IgLON5疾病和DLB的共同特征，并在接受DLB诊断评估的患者中确定抗IgLON5疾病的潜在危险信号。
方法：我们在MEDLINE中搜索，WebofScience,和Embase从12月8日开始，2022年，搜索词为“IgLON5”。我们对抗IgLON5疾病的病例报告和病例系列进行了系统评价，两名审稿人独立提取症状和发现的数据。将症状的频率与DLB的共识标准进行比较。
结果：我们纳入了57项研究，其中127例抗IgLON5疾病的个例报告（诊断时平均年龄63岁，中位症状持续时间2年)。45％的病例报告了认知功能障碍，15%的REM-睡眠行为障碍,14%患有帕金森病。有37%的人报告呼吸功能不全，球症状占67%。
结论：我们发现抗IgLON5疾病和DLB之间存在显著重叠。我们建议在患有舞蹈症的年轻DLB患者中应考虑抗IgLON5疾病，凝视麻痹,早期吞咽困难,或突出的呼吸道症状。我们的研究有助于对抗IgLON5疾病的症状和生物标志物的新知识。
目的：评估抗IgLON5疾病和DLB的共同特征，并在接受DLB诊断评估的患者中确定抗IgLON5疾病的潜在危险信号。
方法：我们在MEDLINE中搜索，WebofScience,和Embase从12月8日开始，2022年，搜索词为“IgLON5”。我们对抗IgLON5疾病的病例报告和病例系列进行了系统评价，两名审稿人独立提取症状和发现的数据。将症状的频率与DLB的共识标准进行比较。
结果：我们纳入了57项研究，其中127例抗IgLON5疾病的个例报告（诊断时平均年龄63岁，中位症状持续时间2年)。45％的病例报告了认知功能障碍，15%的REM-睡眠行为障碍,14%患有帕金森病。有37%的人报告呼吸功能不全，球症状占67%。
结论：我们发现抗IgLON5疾病和DLB之间存在显著重叠。我们建议在患有舞蹈症的年轻DLB患者中应考虑抗IgLON5疾病，凝视麻痹,早期吞咽困难,或突出的呼吸道症状。我们的研究有助于对抗IgLON5疾病的症状和生物标志物的新知识。
