The pimple sign of progressive supranuclear palsy syndrome
The pimple sign of progressive supranuclear palsy syndrome
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DOI：
10.1016/j.parkreldis.2013.10.023
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发表时间：
2014-02-01
影响因子：
4.1
通讯作者：
Josephs, Keith A.
中科院分区：
文献类型：
--
作者：
Botha, Hugo;Whitwell, Jennifer L.;Josephs, Keith A.
背景：一些进行性核上性麻痹综合征 (PSPS) 患者在 FDG-PET 扫描中显示出中脑代谢低下的局灶性区域，我们称之为“丘疹征”。我们评估了它与中脑萎缩的关联、其可靠性以及区分 PSPS 与皮质基底节综合征 (CBS) 和多系统萎缩 (MSA) 的能力。方法：我们确定了 67 名患有 PSPS、CBS 或 MSA 的患者，他们接受了体积 MRI 以及 FOG-PET 成像。测量中脑体积并表示为颅内总体积的百分比。两位独立、盲目的专家将 FDG-PET 上的“丘疹征兆”评为“不存在”、“可能”或“确定”。对这些组的中脑体积进行比较，并使用 kappa 统计数据评估可靠性。使用 CBS 和 MSA 患者作为对照来计算敏感性和特异性。结果：与“不存在”和“可能”组相比，“确定”组的中脑体积减少 (p = 0.0036)。丘疹征兆的评估者间可靠性很高 (k = 0.90)。 “明确的丘疹征象”对 PSPS 具有高特异性 (100%)，但敏感性较低 (29%)，而可能存在或明确的征象的敏感性为 79%。结论：PSPS 的“丘疹征象”与中脑萎缩相关，可能有助于区分 PSPS 与 CBS 和 MSA。 (C) 2013 Elsevier Ltd. 保留所有权利。
Background: Some patients with progressive supranuclear palsy syndrome (PSPS) demonstrate a focal area of midbrain hypometabolism on FDG-PET scans which we call the 'pimple sign'. We assessed its association with midbrain atrophy, its reliability and its ability to differentiate PSPS from corticobasal syndrome (CBS) and multiple system atrophy (MSA).Methods: We identified 67 patients with PSPS, CBS or MSA who had volumetric MRI as well as FOG-PET imaging. Midbrain volume was measured and expressed as a percentage of total intracranial volume. Two independent, blinded specialists rated the 'pimple sign' on FDG-PET as 'absent', 'possible' or 'definite'. Midbrain volumes were compared across these groups and reliability assessed with the kappa statistic. Sensitivity and specificity were calculated using CBS and MSA patients as controls.Results: Midbrain volume was decreased in the 'definite' group compared to the 'absent' and 'possible' groups (p = 0.0036). Inter-rater reliability for the pimple sign was high (k = 0.90). A 'definite pimple sign' had a high specificity (100%) but low sensitivity (29%) for PSPS, whilst the presence of a possible or definite sign had a sensitivity of 79%.Conclusion: The 'pimple sign' of PSPS is associated with midbrain atrophy, and may be helpful in differentiating PSPS from CBS and MSA. (C) 2013 Elsevier Ltd. All rights reserved.