Autonomic Unit, Department of Neurology, General Hospital of the City of Linz (AKH Linz), Linz, Austria; Autonomic Unit, National Hospital for Neurology and Neurosurgery, Queen Square & Institute of Neurology, University College London, London, UK.
Auton Neurosci. 2013 Dec;179(1-2):159-62. doi: 10.1016/j.autneu.2013.04.006. Epub 2013 May 21.
In pure autonomic failure (PAF) - a rare form of primary dysautonomia - some patients show cerebrovascular lesions usually found in hypertensive subjects. In an autonomic laboratory records of patients with a definitive diagnosis of PAF having had cerebral imaging (cMRI, cCT) were analysed retrospectively. Tilt table data (supine/tilted), 24 hour blood pressure recordings (day/night) and serum catecholamine levels were correlated with cerebrovascular lesions and also compared to published normal values. 50 PAF patients (23 female, 27 male) were identified, mean age 67 years (sd 9.5). Out of these 35 (70%) had pathologic cerebral scans showing white matter lesions (WML) in 30, lacunar strokes in 5 and hemispheric stroke and microbleeds each in 1. Age and supine systolic blood pressure were significantly elevated in patients with pathologic scans (70 compared to 61 years [p=0.007], and 170 compared to 154 mmHg [p=0.045]). Out of 28 patients with WML and ambulatory blood pressure recordings available 24 were non-dippers. The data show that the frequency of WML is lower in PAF patients aged 60 to 80 years compared to age matched community based samples. Although PAF usually results in hypotension, a frequent complication is supine hypertension. Although the overall frequency of WML seems to be reduced in PAF, a number of patients with elevated supine systolic blood pressure (>160 mmHg) develop WML and some of these suffer stroke.
在纯粹自主神经衰竭（PAF）-一种罕见的原发性自主神经功能障碍形式-中，一些患者表现出通常在高血压患者中发现的脑血管病变。在自主神经实验室中，回顾性分析了具有明确 PAF 诊断且已进行脑成像（cMRI、cCT）的患者记录。倾斜台数据（仰卧/倾斜）、24 小时血压记录（白天/夜间）和血清儿茶酚胺水平与脑血管病变相关，并与已发表的正常值进行了比较。确定了 50 名 PAF 患者（23 名女性，27 名男性），平均年龄 67 岁（标准差 9.5）。其中 35 名（70%）患者的脑部扫描异常，显示有 30 名患者有白质病变（WML）、5 名患者有腔隙性脑梗死、1 名患者有半球性脑梗死和微出血。在有病理扫描的患者中，年龄和仰卧位收缩压显著升高（70 岁对比 61 岁，p=0.007；170 毫米汞柱对比 154 毫米汞柱，p=0.045）。在有 WML 和动态血压记录的 28 名患者中，24 名是非杓型血压。这些数据表明，在 60 至 80 岁的 PAF 患者中，WML 的频率低于年龄匹配的社区样本。尽管 PAF 通常导致低血压，但常见的并发症是仰卧位高血压。尽管 PAF 患者的 WML 总体频率似乎降低，但许多仰卧位收缩压升高（>160 毫米汞柱）的患者会发生 WML，其中一些患者会发生中风。
