Neurodegenerative diseases, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), and amyotrophic lateral sclerosis (ALS) are major public health issues in the aging population. The central nervous system undergoes considerable degeneration for years before the onset of cardinal neurodegenerative symptoms such as resting tremors in PD (1). Failure to identify highly disease-correlated risk factors or biomarkers delays the initiation of neuroprotective treatment; several clinical trials on disease modification in neurodegenerative disorders have failed because of this. The lack of reliable outcome-predicting biomarkers hinders the development of disease modification approaches (2). Epidemiological studies have presented significant risk factors for and biomarkers of neurodegenerative diseases that can be detected before diagnosis, such as mild cognitive impairment (MCI) for AD (3, 4) and rapid eye movement sleep behavioral disorder (RBD) for PD (5). Vascular risk factors accelerate AD progression (6); diabetes is associated with rapid deterioration in PD (7). However, this knowledge does not facilitate robust prediction of neurodegenerative diseases and outcomes or early initiation of neuroprotective treatment in clinical trials. In this special issue, we have gathered eight original articles regarding biomarkers of neurodegenerative diseases. A review article explores the role of neuroimaging in neurodegenerative diseases. Articles in this special issue address three topics: clinical disease biomarkers for neurodegenerative diseases, biomarkers from animal models of neurodegenerative diseases, and biomarkers as therapeutic indicators.