Neuropathological Conditions and Clinical Presentations Flashcards
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Which neurodegenerative disease is defined by the hallmark pathology of amyloid-beta plaques and tau-containing neurofibrillary tangles alongside progressive memory loss?
Answer: Alzheimer's disease
Alzheimer's disease is defined by extracellular amyloid-beta plaques and intraneuronal neurofibrillary tangles, accompanied by progressive episodic memory impairment.
Which clinical feature most distinguishes Lewy body dementia from Alzheimer's disease on neuropsychological evaluation?
Answer: Fluctuating cognition and well-formed visual hallucinations
Lewy body dementia is characterized by fluctuating cognitive arousal, spontaneous well-formed visual hallucinations, and parkinsonism, features not typical of Alzheimer's disease.
Which subcortical structure shows the most prominent early atrophy in Huntington's disease, contributing to characteristic cognitive and motor symptoms?
Answer: Caudate nucleus
Huntington's disease causes early degeneration of the caudate nucleus and striatum, producing choreiform movements and executive dysfunction.
In behavioral variant frontotemporal dementia (bvFTD), which cognitive domain is typically most impaired early in the disease course?
Answer: Executive function and social behavior regulation
bvFTD preferentially affects prefrontal circuits, resulting in early prominent executive dysfunction, disinhibition, and social comportment deficits before significant memory impairment.
A patient with Parkinson's disease without dementia undergoes neuropsychological testing. Which cognitive profile is most characteristic?
Answer: Executive dysfunction and slowed processing with relative memory preservation
Parkinson's disease produces a subcortical cognitive profile characterized by executive dysfunction, psychomotor slowing, and visuospatial difficulties with relative preservation of memory encoding.
Which condition is characterized by rapidly progressive dementia, myoclonus, periodic sharp wave complexes on EEG, and detection of 14-3-3 protein in cerebrospinal fluid?
Answer: Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease is a fatal prion disease presenting with rapidly progressive dementia, myoclonus, and CSF biomarkers including 14-3-3 protein.
Compared to Alzheimer's disease, vascular dementia most characteristically shows which pattern of cognitive decline and neuropsychological profile?
Answer: Stepwise decline with prominent executive dysfunction and relatively less severe amnesia
Vascular dementia typically shows stepwise cognitive decline corresponding to cerebrovascular events, with frontal-subcortical executive deficits more prominent than the episodic amnesia seen in AD.