Department of Neurology, Mayo Clinic, 4500 San Pablo Rd., Jacksonville, FL, 32224, USA.
Clin Auton Res. 2018 Apr;28(2):215-221. doi: 10.1007/s10286-017-0500-0. Epub 2018 Jan 8.
The initial symptoms of multiple system atrophy (MSA) and, in particular, early autonomic symptoms, have received less attention than motor symptoms. Whereas pathognomonic motor signs are essential to diagnostic specificity, early symptoms important to recognition of a neurodegenerative disorder may be less apparent or diagnostically ambiguous. This observational study sought to identify the very earliest symptoms in the natural history of MSA.
Detailed clinical histories focusing on early symptoms were obtained from 30 subjects recently diagnosed with MSA. Historical data were correlated with neurological examinations and laboratory autonomic testing.
Subjects' mean age was 63.9 years. Ten were classified as having MSA-P and 20 MSA-C. The evaluations occurred 2.9 ± 0.4 months after diagnosis. The first symptom of MSA was autonomic in 22 (73%) and motor in 3 (10%) subjects (p < 0.0001). The most frequent first symptom was erectile failure, which occurred in all men beginning 4.2 ± 2.6 years prior to diagnosis. After erectile failure, postural lightheadness or fatigue following exercise, urinary urgency or hesitancy, and violent dream enactment behavior consistent with REM behavioral sleep disorder were the most frequent initial symptoms. Neither the order of symptom progression, which was highly variable, nor autonomic severity scores differentiated between MSA-P and MSA-C.
The first symptoms of MSA are frequently autonomic and may predate recognition of motor manifestations. Orthostatic hypotension and, in men, erectile failure are among the first symptoms that, when evaluated in the context of associated clinical findings, may facilitate accurate and earlier diagnosis.
多系统萎缩（MSA）的初始症状，尤其是早期自主症状，受到的关注不如运动症状多。虽然特征性运动体征对诊断特异性至关重要，但对识别神经退行性疾病很重要的早期症状可能不太明显或诊断上存在歧义。本观察性研究旨在确定 MSA 自然史中最早的症状。
从最近被诊断为 MSA 的 30 名患者中获得了详细的以早期症状为重点的临床病史。将病史与神经系统检查和实验室自主神经测试相关联。
患者的平均年龄为 63.9 岁。10 人被归类为 MSA-P，20 人被归类为 MSA-C。评估发生在诊断后 2.9±0.4 个月。MSA 的第一个症状在 22 名（73%）患者中为自主神经症状，在 3 名（10%）患者中为运动症状（p<0.0001）。最常见的首发症状是勃起功能障碍，所有男性在诊断前 4.2±2.6 年就出现了这种症状。勃起功能障碍后，体位性头晕或运动后疲劳、尿急或犹豫、以及与 REM 行为睡眠障碍一致的强烈梦境发作行为是最常见的初始症状。症状进展的顺序既不具有高度可变性，自主神经严重程度评分也不能区分 MSA-P 和 MSA-C。
MSA 的首发症状通常为自主神经症状，可能早于运动表现的识别。直立性低血压和男性的勃起功能障碍是最早出现的症状之一，如果结合相关临床发现进行评估，可能有助于更准确和更早地诊断。
