Quick Start: Creutzfeldt–Jakob Disease Definition Creutzfeldt–Jakob disease (CJD) is one of several prion transmissible neurodegenerative diseases characterized by a rapid cognitive decline that can progress to akinetic mutism over weeks. Creutzfeldt–Jakob disease is the most common type of prion disease;…
Quick Start: Chronic Traumatic Encephalopathy Definition and etiology Chronic traumatic encephalopathy is a progressive neurodegenerative disease associated with repetitive brain trauma. Pathology includes perivascular tau deposition. Cognitive and behavioral symptoms, in order of prevalence at presentation Memory impairment Executive dysfunction…
Quick Start: Normal Pressure Hydrocephalus Definition Normal pressure hydrocephalus (often referred to as NPH) is a relatively rare disorder characterized by enlargement of the ventricles, a gait disorder, cognitive impairment, and incontinence. It is thought to result from low-grade scarring…
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…