Quick Start: Corticobasal Degeneration And Corticobasal Syndrome Definition Corticobasal degeneration is a neurodegenerative disease of the brain caused by accumulation of hyperphosphorylated 4-repeat tau isoforms. Corticobasal syndrome is a clinical diagnosis characterized by asymmetric cortical dysfunction, which may include difficulties…
Quick Start: Progressive Supranuclear Palsy Definition Progressive supranuclear palsy (often abbreviated to PSP) is a neurodegenerative disease caused by the accumulation of hyperphosphorylated tau protein isoforms in the brain. Main features include abnormalities of vertical eye movements (supranuclear palsy), along…
Quick Start: Posterior Cortical Atrophy Definition and etiology Posterior cortical atrophy (PCA) is a clinical syndrome characterized by progressive visual and visuospatial dysfunction. Most patients present between the ages of 50 and 65 years (mean age 59 years), although some…
Quick Start: Behavioral Variant Frontotemporal Dementia Definition Behavioral variant frontotemporal dementia is a progressive neurodegenerative disorder with more than a dozen different pathologies. Prevalence Behavioral variant frontotemporal dementia is found in about 5% of cases of dementia. It most commonly…
Quick Start: Primary Progressive Aphasia and Apraxia of Speech Definition and etiology Primary progressive aphasia (PPA) is a clinical syndrome characterized by progressive language dysfunction. There are three variants: Logopenic variant is most often associated with Alzheimer’s disease pathology. Semantic…
Quick Start: Dementia With Lewy Bodies Definition Dementia with Lewy bodies (DLB) is a neurodegenerative disease of the brain characterized: clinically by dementia, fluctuating attention and alertness, visual hallucinations, rapid-eye movement (REM) sleep behavior disorder, and parkinsonism, and pathologically by…
Quick Start: Vascular Cognitive Impairment and Vascular Dementia Definition Vascular cognitive impairment is the overarching term used when cognitive dysfunction is due to cerebrovascular disease (i.e., strokes). Vascular dementia (VaD) occurs when cerebrovascular disease causes cognitive dysfunction that significantly impairs…
Quick Start: Limbic-Predominant Age-Related TDP-43 Encephalopathy Definition In limbic-predominant age-related TDP-43 encephalopathy (LATE), phosphorylated transactive response DNA binding protein of 43 kDa (TDP-43) is found in neuronal cytoplasmic inclusions in the setting of neuronal loss and gliosis in the amygdala,…
Quick Start: Primary Age-Related Tauopathy Definition In primary age-related tauopathy (PART), neurofibrillary tangles accumulate in entorhinal cortex and limbic regions without amyloid plaques or other pathology. It may be a common cause of cognitive deficits in normal aging, subjective cognitive…
Quick Start: Alzheimer’s Disease Definition Alzheimer’s disease is a neurodegenerative disease of the brain characterized by progressive amnestic dysfunction with specific microscopic pathology including senile plaques and neurofibrillary tangles. Prevalence Alzheimer’s disease is the most common cause of dementia, affecting…