![]() ![]() Neurodegenerative diseases share many fundamental processes associated with progressive neuronal dysfunction and death, suchĪs proteotoxic stress and its attendant abnormalities in ubiquitin–proteasomal and autophagosomal/lysosomal systems, oxidative Although neurodegenerative diseases are typically defined by specific protein accumulations and anatomic vulnerability, The current diagnostic gold standard is neuropathological evaluation at autopsy (see Table 1 for summary). Few patients have pure syndromes, with most having ![]() Neurodegenerative disorders can be broadly classified by their clinical presentations, with extrapyramidal and pyramidal movementĭisorders and cognitive or behavioral disorders being the most common. In this review, we detail the human pathology of select neurodegenerativeĭisorders, focusing on their main protein aggregates. The specific anatomical patterns observed at autopsy. That abnormal protein conformers may spread from cell to cell along anatomically connected pathways, which may in part explain The protein abnormalities in these disorders have abnormal conformational properties. The most common neurodegenerative disorders are amyloidoses, tauopathies, α-synucleinopathies, and TDP-43 proteinopathies. (e.g., frontotemporal degenerations, extrapyramidal disorders, or spinocerebellar degenerations), or principal molecular abnormality. Neurodegenerative diseases can be classifiedĪccording to primary clinical features (e.g., dementia, parkinsonism, or motor neuron disease), anatomic distribution of neurodegeneration Neurodegenerative disorders are characterized by progressive loss of selectively vulnerable populations of neurons, whichĬontrasts with select static neuronal loss because of metabolic or toxic disorders.
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