ABCN Neuropathological Conditions and Clinical Presentations 1 — Questions and Answers
Question 1: Which neurodegenerative disease is defined by the hallmark pathology of amyloid-beta plaques and tau-containing neurofibrillary tangles alongside progressive memory loss?
- Parkinson's disease
- Alzheimer's disease (Correct answer)
- Huntington's disease
- Lewy body dementia
Correct answer: Alzheimer's disease
Alzheimer's disease is defined by extracellular amyloid-beta plaques and intraneuronal neurofibrillary tangles, accompanied by progressive episodic memory impairment.
Question 2: Which clinical feature most distinguishes Lewy body dementia from Alzheimer's disease on neuropsychological evaluation?
- Rapid forgetting on delayed recall measures
- Fluctuating cognition and well-formed visual hallucinations (Correct answer)
- Prominent early language impairment
- Isolated visuospatial deficit with intact memory
Correct 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.
Question 3: Which subcortical structure shows the most prominent early atrophy in Huntington's disease, contributing to characteristic cognitive and motor symptoms?
- Hippocampus
- Caudate nucleus (Correct answer)
- Amygdala
- Thalamus
Correct answer: Caudate nucleus
Huntington's disease causes early degeneration of the caudate nucleus and striatum, producing choreiform movements and executive dysfunction.
Question 4: In behavioral variant frontotemporal dementia (bvFTD), which cognitive domain is typically most impaired early in the disease course?
- Episodic memory encoding
- Visuospatial construction
- Executive function and social behavior regulation (Correct answer)
- Semantic memory for word meanings
Correct 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.
Question 5: A patient with Parkinson's disease without dementia undergoes neuropsychological testing. Which cognitive profile is most characteristic?
- Prominent episodic amnesia with intact executive function
- Executive dysfunction and slowed processing with relative memory preservation (Correct answer)
- Isolated aphasia with preserved cognition elsewhere
- Global cognitive decline across all domains equally
Correct 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.
Question 6: 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?
- HIV-associated neurocognitive disorder
- Creutzfeldt-Jakob disease (Correct answer)
- Progressive supranuclear palsy
- Corticobasal degeneration
Correct 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.
Question 7: Compared to Alzheimer's disease, vascular dementia most characteristically shows which pattern of cognitive decline and neuropsychological profile?
- Gradual progressive amnesia with late executive impairment
- Stepwise decline with prominent executive dysfunction and relatively less severe amnesia (Correct answer)
- Early prominent language impairment with preserved memory
- Uniform global decline without domain-specific features
Correct 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.
ABCN Neuropathological Conditions and Clinical Presentations 1
Which neurodegenerative disease is defined by the hallmark pathology of amyloid-beta plaques and tau-containing neurofibrillary tangles alongside progressive memory loss?