Neurologists Exam Neurologists Exam Neurodegenerative & Movement Disorders 2 — Questions and Answers
Question 1: Which cognitive domain is characteristically impaired earliest in Alzheimer's disease?
- Episodic memory (short-term recall) (Correct answer)
- Procedural memory
- Visuospatial ability
- Language fluency
Correct answer: Episodic memory (short-term recall)
Alzheimer's disease typically begins with episodic memory impairment reflecting early hippocampal and entorhinal cortex involvement.
Question 2: Frontotemporal dementia (FTD) is distinguished from Alzheimer's disease by which early clinical feature?
- Prominent personality and behavioral changes (Correct answer)
- Early episodic memory loss
- Visual hallucinations
- Postural instability
Correct answer: Prominent personality and behavioral changes
FTD primarily affects frontal and temporal lobes causing early behavioral disinhibition, apathy, and personality change before significant memory loss.
Question 3: Progressive supranuclear palsy (PSP) is best characterized by which unique clinical finding?
- Vertical gaze palsy (downgaze) and falls (Correct answer)
- Resting tremor and cogwheel rigidity
- Early dementia with visual hallucinations
- Chorea and psychiatric symptoms
Correct answer: Vertical gaze palsy (downgaze) and falls
PSP classically presents with vertical supranuclear gaze palsy (especially downgaze), axial rigidity, and early falls backward.
Question 4: Cerebellar ataxia, polyneuropathy, and autonomic failure occurring together in a middle-aged adult most suggests which condition?
- Multiple system atrophy (MSA-C) (Correct answer)
- Friedreich's ataxia
- Hereditary spastic paraplegia
- CADASIL
Correct answer: Multiple system atrophy (MSA-C)
MSA-C combines cerebellar ataxia with autonomic failure (orthostatic hypotension, bladder dysfunction) reflecting combined olivopontocerebellar and autonomic degeneration.
Question 5: Which treatment is FDA-approved specifically for chorea in Huntington's disease?
- Tetrabenazine (or deutetrabenazine) (Correct answer)
- Levodopa
- Donepezil
- Riluzole
Correct answer: Tetrabenazine (or deutetrabenazine)
Tetrabenazine (and its deuterated form deutetrabenazine) deplete presynaptic monoamines by inhibiting VMAT2, reducing choreiform movements in HD.
Question 6: Corticobasal syndrome (CBS) is characterized by asymmetric parkinsonism combined with which distinctive cortical sign?
- Alien limb phenomenon (Correct answer)
- Resting tremor
- Vertical gaze palsy
- Visual hallucinations
Correct answer: Alien limb phenomenon
The alien limb phenomenon—involuntary, goal-directed limb movements that feel foreign to the patient—is a hallmark cortical sign of CBS.
Neurologists Exam Neurologists Exam Neurodegenerative & Movement Disorders 2
Which cognitive domain is characteristically impaired earliest in Alzheimer's disease?