Albanese A, Colosimo C, Bentivoglio A R, Fenici R, Melillo G, Colosimo C, Tonali P
Istituto di Neurologia, Università Cattolica del Sacro Cuore, Roma, Italy.
J Neurol Neurosurg Psychiatry. 1995 Aug;59(2):144-51. doi: 10.1136/jnnp.59.2.144.
To evaluate the possibility that parkinsonian signs may be the only presenting feature of multiple system atrophy (MSA), parkinsonian patients were studied who had no atypical clinical signs and had no symptoms of autonomic dysfunction, but who reported that they had not experienced the anticipated good response to dopaminergic treatment. These stringent criteria identified 20 patients from a series of 298 consecutive parkinsonian outpatients. The following clinical pointers were analysed: (a) rate of disease progression; (b) symmetry of parkinsonian symptoms and signs; (c) occurrence of resting tremor during the first three years from onset. In addition, all patients underwent (d) acute and chronic challenge with dopaminergic drugs; (e) cardiovascular reflex autonomic function tests; (f) high field MRI. Rapid progression of disease was seen in 45% of patients, onset was symmetric in 25%, tremor was absent at onset in 70%, response to dopaminergic drug challenges was inadequate in 40%, abnormal cardiovascular reflexes occurred in 50%, and some abnormal MRI finding occurred in 35% of cases. Each of these features was equally weighted by giving to each patient a 0 to 6 point score corresponding to the number of abnormal findings. Fifteen patients scoring higher than 1 were considered at risk for having MSA: five of them were classified as clinically possible (score 2), six as clinically probable (score 3-4), and four patients were classified as clinically definite multiple system atrophy (score 5). The six pointers considered were variably combined in each patient, none of them being universally abnormal in patients with high scores. The patients were followed up for a mean 2.1 (SEM 0.65) years. All but one of the 10 patients prospectively classified as probable or definite MSA developed unequivocal clinical signs of fully symptomatic MSA. A receiver operator characteristic cure was plotted for the prospective score based on follow up diagnosis. The best compromise for trade off between sensitivity and specificity was a cut off value at a score of 3. The sensitivity and specificity of the individual pointers considered to predict fully symptomatic MSA varied considerably, and no single item could predict whether patients presenting with just parkinsonian signs went on during the two year follow up period to develop fully symptomatic MSA. Instead, the number of abnormalities offered a predictive value for the clinical prognosis of these parkinsonian patients.
为评估帕金森氏症体征可能是多系统萎缩（MSA）唯一表现特征的可能性，我们对帕金森氏症患者进行了研究，这些患者没有非典型临床体征且没有自主神经功能障碍症状，但他们表示未经历预期的对多巴胺能治疗的良好反应。这些严格标准从298例连续的帕金森氏症门诊患者中识别出20例患者。分析了以下临床指标：（a）疾病进展速度；（b）帕金森氏症症状和体征的对称性；（c）起病后头三年静止性震颤的发生情况。此外，所有患者均接受了（d）多巴胺能药物的急性和慢性激发试验；（e）心血管反射自主神经功能测试；（f）高场强磁共振成像（MRI）。45%的患者疾病进展迅速，25%起病对称，70%起病时无震颤，40%对多巴胺能药物激发试验反应不佳，50%心血管反射异常，35%的病例有一些MRI异常发现。通过给每位患者一个0至6分的评分（对应异常发现的数量），对这些特征进行同等加权。15例评分高于1分的患者被认为有患MSA的风险：其中5例被分类为临床可能（评分2分），6例为临床很可能（评分3 - 4分），4例患者被分类为临床确诊的多系统萎缩（评分5分）。所考虑的六个指标在每位患者中以不同方式组合，高分患者中没有一个指标普遍异常。对患者进行了平均2.1（标准误0.65）年的随访。前瞻性分类为很可能或确诊MSA的10例患者中，除1例之外，其余均出现了明确的完全有症状MSA的临床体征。根据随访诊断绘制了预测评分的受试者工作特征曲线。在敏感性和特异性之间进行权衡的最佳折衷点是评分3分的截断值。所考虑的用于预测完全有症状MSA的各个指标的敏感性和特异性差异很大，没有单个指标能够预测仅表现为帕金森氏症体征的患者在两年随访期间是否会发展为完全有症状的MSA。相反，异常的数量为这些帕金森氏症患者的临床预后提供了预测价值。
