【家族性帕金森病的临床与流行病学特点】。

Archivos de neurobiologia Pub Date : 1990-09-01
B Morales, R Astarloa, R González Maldonado, N Peñafiel, P J Garcia Ruiz, A Vázquez, D G Urra, J G de Yébenes
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引用次数: 0

摘要

我们研究了44例被诊断为特发性帕金森病的患者,这些患者包括在我们的刚性动力学综合征数据库中。我们将他们的人口学、环境和临床特征与22名被诊断为特发性帕金森病并有一些一级亲属患有同样疾病的患者进行了比较。家族性帕金森病患者与散发性帕金森病患者的区别在于发病早、亲缘关系高、父母患类似疾病的频率高。此外,我们发现家族性帕金森病患者一生中喝的井水比散发性帕金森病患者多,出现宽运动性障碍、肌张力障碍、夜间运动障碍、左旋多巴波动的频率更高,头发过早变白的频率更高。我们既没有发现流行病学资料可以解释家族性病例的出现,也没有发现环境因素可以产生家族性帕金森病。两组之间的临床差异可能是由于家族性帕金森病病例的早期症状。根据我们的数据,我们不能得出家族性和散发性帕金森病之间有显著差异的结论,以证明两种明确定义的疾病。甚至,家族性特发性帕金森病可能是帕金森病的正常形式,如果长期生存是一个有利因素的疾病发病前症状的人。
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[Clinical and epidemiologic characteristics of familial Parkinson disease].

We have studied 44 patients diagnosed of idiopathic Parkinson disease included in our database of rigid-akinetic syndromes. We have compared their demographic, environmental and clinical features with the ones that presented a group on 22 patients diagnosed of idiopathic Parkinson disease and had some first degree relatives with the same disease. Patients with familial Parkinson disease are distinguished from the ones that suffer from sporadic Parkinson disease because of an early start, greater consanguinity rate and greater frequency of a similar disease in their parents. Moreover, we have seen that familial Parkinson disease patients have drunk more water from wells during their lives than the ones that suffer sporadic Parkinson disease, present greater frequency of wide motoricity disorders, dystonia, night hypokinesia, fluctuations in relation to L-DOPA and greater frequency of early going grey. We have not found either epidemiologic data which could explain the appearance of familial cases or environmental causes which could produce familial Parkinson disease. Clinical differences between the two groups are likely due to an early start of symptoms in familial Parkinson disease cases. According to our data we could not conclude that between familial and sporadic Parkinson disease are significant differences in to justify two well-defined diseases. Even, the familial presentation of idiopathic Parkinson disease could be the normal form of Parkinson disease if long survival was a favourable factor of disease onset in pre-symptomatic persons.

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