Dickson Dennis W
Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Int J Clin Exp Pathol. 2009 Aug 25;3(1):1-23.
Neurodegenerative diseases are characterized by selective and progressive loss of specific populations of neurons, which determines the clinical presentation. The same neuronal populations can be affected in a number of different disorders. Given that the clinical presentation reflects the particular population of neurons that are targets of the disease process, it is clear that for any given clinical syndrome, more than one neurodegenerative disease can account for the clinical syndrome. Because of this clinical ambiguity, for the purpose of this brief review neurodegenerative disorders are classified according to the underlying molecular pathology rather than their clinical presentation. The major neurodegenerative diseases can be classified into amyloidoses, tauopathies, alpha-synucleinopathies and TDP-43 proteinopathies.
神经退行性疾病的特征是特定神经元群体的选择性和进行性丧失,这决定了临床表现。相同的神经元群体可能在多种不同疾病中受到影响。鉴于临床表现反映了作为疾病过程靶点的特定神经元群体,显然对于任何给定的临床综合征,不止一种神经退行性疾病可导致该临床综合征。由于这种临床模糊性,在本简要综述中,神经退行性疾病是根据潜在的分子病理学而非临床表现进行分类的。主要的神经退行性疾病可分为淀粉样变性病、tau蛋白病、α-突触核蛋白病和TDP-43蛋白病。