Background:Scans without evidence of dopaminergic deficit (SWEDDS) on 123I-FP-CIT SPECT (DAT) can occur in patients with clinical evidence of Parkinsonism. In this diverse patient population, autonomic function testing may elucidate the underlying clinical disorder. Objective:To evaluate SWEDD patients undergoing autonomic testing and determine the severity and pattern of autonomic dysfunction. Methods:All patients with a diagnosis of SWEDD and formal autonomic function testing at Mayo Clinic, MN were retrospectively reviewed. Autonomic failure was quantified using composite autonomic severity score (CASS). The Modified Hoehn and Yahr score (HYS) determined Parkinsonism severity. Results:Of 1,874 patients with DAT imaging at Mayo Clinic, 13 met criteria. The median age of symptom onset was 56.0 (IQR 40.5–75.5). Autonomic dysfunction was present in 12/13 on ARS and/or TST. The median CASS was 2.50 (IQR 1.00–3.00). Distal anhidrosis was most common (7/13) while 3/13 had widespread anhidrosis on TST and/or QSART testing. Patients with a distal pattern of anhidrosis had a median score of 3.0 (IQR 2.38–4.25) on the HYS versus 2.0 (IQR 1.00–2.00) for those with a diffuse pattern (p = 0.048). Patients with more advanced Parkinsonism were more likely to respond to L-Dopa, with higher HYS in the dopa-responsive versus non-Dopa-responsive (p = 0.026). No correlation existed between severity of Parkinsonism, and CASS (p = 0.39). Conclusion:Autonomic function testing may detect autonomic dysfunction in most patients with SWEDD. The pattern of dysfunction is suggestive of the degree of clinical Parkinsonism, and autonomic testing may predict whether patients with SWEDD respond to L-Dopa.

中文翻译：

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无多巴胺能缺陷 (SWEDD) 证据的扫描中的自主测试概况。

背景：在具有帕金森症临床证据的患者中，可能会出现在 123I-FP-CIT SPECT (DAT) 上没有多巴胺能缺陷 (SWEDDS) 证据的扫描。在这个多样化的患者群体中，自主神经功能测试可能会阐明潜在的临床疾病。目的：评估接受自主神经测试的 SWEDD 患者并确定自主神经功能障碍的严重程度和模式。方法：对所有在明尼苏达州梅奥诊所诊断为 SWEDD 并进行正式自主神经功能测试的患者进行回顾性分析。使用综合自主神经严重程度评分 (CASS) 量化自主神经衰竭。改良 Hoehn 和 Yahr 评分 (HYS) 确定帕金森病的严重程度。结果：在 Mayo Clinic 的 1,874 名 DAT 成像患者中，13 名符合标准。出现症状的中位年龄为 56.0 (IQR 40.5–75.5)。12/13 的 ARS 和/或 TST 出现自主神经功能障碍。中位数 CASS 为 2.50（IQR 1.00–3.00）。远端无汗是最常见的 (7/13)，而 3/13 在 TST 和/或 QSART 测试中有广泛的无汗。远端无汗症患者 HYS 的中位评分为 3.0（IQR 2.38-4.25），而弥漫型患者为 2.0（IQR 1.00-2.00）（p = 0.048）。帕金森病晚期患者更可能对左旋多巴有反应，多巴反应性与非多巴反应性的 HYS 更高（p = 0.026）。帕金森病的严重程度与 CASS 之间不存在相关性（p = 0.39）。结论：自主神经功能检测可检测出大部分 SWEDD 患者的自主神经功能障碍。功能障碍的模式提示临床帕金森病的程度，