The Parkinson's complex: Parkinsonism is just the tip of the iceberg
The Parkinson's complex: Parkinsonism is just the tip of the iceberg
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DOI：
10.1002/ana.20834
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发表时间：
2006-04-01
影响因子：
11.2
通讯作者：
Langston, JW
中科院分区：
文献类型：
--
作者：
Langston, JW
一位78岁男性，有15年的慢性便秘病史。在68岁时，他经历了几乎完全丧失嗅觉。最近，他患上了一种不寻常的睡眠障碍，其特征是夜间突然的，有时是好斗的行为，导致他的配偶在两个不同的场合受伤;他被转介到睡眠障碍诊所。对于大多数医生来说，这种病史不会立即引起帕金森病（PD）。然而，越来越多的临床和病理学证据表明，它应该。PD的传统“教科书”描述将该疾病定义为一种进行性疾病，其特征为强直、运动迟缓和静止性震颤三重征。大量证据表明，这些特征是由于多巴胺能黑质纹状体系统的变性造成的。虽然这些临床特征通常会导致患者去看神经科医生，但现在越来越清楚的是，帕金森症只是一个多方面的复杂疾病的一个方面。此外，越来越清楚的是，这些非帕金森病的特征中至少有一部分可以在帕金森病发作前数年甚至数十年出现。这篇文章认为，帕金森症代表了通过神经科医生有点近视的透镜看到的疾病，而在现实中，帕金森症只是构成这种多样性疾病的冰山一角（图1）。如果人们接受这一观点，那么是时候从根本上重新思考构成这种疾病的复杂特征了，这可能更好地被视为“中心交感肌间神经元病”。这篇论文的意义不仅仅是学术上的。在我们对这种疾病的临床和病理特征的了解迅速扩大的时候，如果不重新评估我们如何看待这种疾病，不仅会对我们所照顾的患者的诊断和管理产生不利影响，而且可能会阻碍研究。
A 78-year-old man presented with a 15-year history of chronic constipation. At age 68, he experienced a nearcomplete loss of his sense of smell. Recently, he has developed an unusual sleep disorder, characterized by abrupt and at times combative behavior during the night that has resulted in injury to his spouse on two separate occasions; he is referred to a sleep disorders clinic.For most physicians, this medical history would not immediately bring Parkinson’s disease (PD) to mind. Yet, accumulating clinical and pathological evidence suggests it should. The traditional “textbook” description of PD defines the disease as a progressive disorder characterized by the triad of rigidity, bradykinesia, and rest tremor. A large body of evidence suggests that these features are due to degeneration of the dopaminergic nigrostriatal system. Although these clinical features typically lead a patient to the door of the neurologist, it is now increasingly clear that parkinsonism is only one aspect of a multifaceted and complex disorder. Furthermore, it is increasing clear that at least some of these nonparkinsonian features can precede the onset of parkinsonism by years and perhaps even decades. This essay argues that parkinsonism represents the disease as seen through the somewhat myopic lens of the neurologist, whereas in reality, parkinsonism only represents the tip of the iceberg that makes up this diverse disease (Fig 1). If one accepts this view, then the time has come to radically rethink the complex of features that constitute the disorder, which might be better viewed as a “centrosympathomyenteric neuronopathy.” The implications of this thesis are of more than academic importance. A failure to reevaluate how we view this disease at a time when our knowledge about its clinical and pathological features is expanding rapidly will adversely affect not only efforts to improve the diagnosis and management of the patients that we care for, but also is likely to impede research