Anhidrosis in multiple system atrophy involves pre- and postganglionic sudomotor dysfunction.
Anhidrosis in multiple system atrophy involves pre- and postganglionic sudomotor dysfunction.
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DOI：
10.1002/mds.26864
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发表时间：
2017-03
期刊：
影响因子：
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通讯作者：
Singer W
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文献类型：
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作者：
Coon EA;Fealey RD;Sletten DM;Mandrekar JN;Benarroch EE;Sandroni P;Low PA;Singer W
本研究的目的是描述多系统萎缩（MSA）中泌汗功能障碍的程度、模式、病变部位和时间演变，并评估帕金森病（MSA-帕金森病）和小脑（MSA-小脑）亚型的差异。回顾了2005年至2010年在马约诊所罗切斯特进行的所有MSA病例，包括节后泌汗试验和体温调节汗液试验。模式和病变部位（节前，节后，或混合）的基础上确定体温调节出汗试验和节后催汗试验。232例患者中的大多数为MSA-帕金森综合征（145例，63%）。初始节后泌汗试验异常59%，而体温调节汗液试验异常95%的所有患者。与小脑型相比，帕金森综合征型患者更容易出现体温调节汗液试验异常（98%比90%，P = 0.006），并且与小脑型相比，无汗症的平均百分比（57%）更高（48%; P = 0.033）。常见的无汗症模式为区域性（38%）和全球性（35%）。病变部位为节前的占47%，混合型（节前和节后）占41%。根据对29名患者进行的70次重复体温调节汗液测试，每年无汗症的增加率为6.2%。随着时间的推移，节后催汗异常的频率增加。我们的研究结果表明：（1）泌汗功能障碍几乎总是存在于MSA中，并且在MSA-帕金森综合征中比在MSA-小脑中更常见和更严重;（2）节前模式的汗液损失在MSA中是常见的;然而，节前和节后异常可能共存;和（3）随着时间的推移，节后泌汗功能障碍的频率增加表明在疾病过程后期涉及节后纤维或汗腺。
The objective of this study was to characterize the degree, pattern, lesion site, and temporal evolution of sudomotor dysfunction in multiple system atrophy (MSA) and to evaluate differences by parkinsonian (MSA-parkinsonism) and cerebellar (MSA-cerebellar) subtypes. All cases of MSA evaluated at Mayo Clinic Rochester between 2005 and 2010 with postganglionic sudomotor testing and thermoregulatory sweat test were reviewed. Pattern and lesion site (preganglionic, postganglionic, or mixed) were determined based on thermoregulatory sweat test and postganglionic sudomotor testing. The majority of the 232 patients were MSA-parkinsonism (145, 63%). Initial postganglionic sudomotor testing was abnormal in 59%, whereas thermoregulatory sweat test was abnormal in 95% of all patients. MSA-parkinsonism patients were more likely to have an abnormal thermoregulatory sweat test compared with MSA-cerebellar (98% versus 90%, P = 0.006) and had a higher mean percentage of anhidrosis (57%) compared with MSA-cerebellar (48%; P = 0.033). Common anhidrosis patterns were regional (38%) and global (35%). The site of the lesion was preganglionic in 47% and mixed (preganglionic and postganglionic) in 41%. The increase in anhidrosis per year was 6.2% based on 70 repeat thermoregulatory sweat tests performed on 29 patients. The frequency of postganglionic sudomotor abnormalities increased over time. Our findings suggest: (1) sudomotor dysfunction is almost invariably present in MSA and even more common and severe in MSA-parkinsonism than MSA-cerebellar; (2) a preganglionic pattern of sweat loss is common in MSA; however, pre- and post-ganglionic abnormalities may coexist; and (3) the increasing frequency of postganglionic sudomotor dysfunction over time suggests involvement of postganglionic fibers or sweat glands later in the disease course.