LATEST PATHOLOGY - NEUROPATHOLOGY
CERTIFICATION EXAM OFFERED BY
AMERICAN BOARD OF PATHOLOGY (ABMS) |
COMPLETE EXAM Q&A WITH RATIONALES
1. A 65-year-old man presents with progressive
memory loss, visuospatial deficits, and personality
changes over 5 years. At autopsy, the brain shows
diffuse cortical atrophy with temporal lobe
predominance. Microscopy reveals neurofibrillary
tangles (NFTs), neuritic plaques, and cerebral
amyloid angiopathy (CAA). Immunohistochemistry for
tau shows 3-repeat and 4-repeat isoforms. What is
the most likely diagnosis?
A) Pick disease
B) Alzheimer disease (AD)
C) Dementia with Lewy bodies (DLB)
D) Frontotemporal dementia (FTD)
Correct answer: B
Rationale: Alzheimer disease is defined by neuritic
plaques (amyloid-beta) and neurofibrillary tangles
(hyperphosphorylated tau, both 3R and 4R). The
clinical course (5+ years) and atrophy pattern are
consistent. Pick disease (A) has Pick bodies (3R tau).
,2. A 55-year-old man presents with rapidly
progressive dementia, myoclonus, and ataxia over 4
months. EEG shows periodic sharp wave complexes.
MRI shows hyperintensity in the basal ganglia and
cerebral cortex on DWI (cortical ribboning). What is
the most likely diagnosis?
A) Alzheimer disease
B) Creutzfeldt-Jakob disease (CJD)
C) Lewy body dementia
D) Progressive multifocal leukoencephalopathy (PML)
Correct answer: B
Rationale: Sporadic CJD (prion disease) presents
with rapidly progressive dementia (months),
myoclonus, ataxia, and characteristic EEG (PSWCs).
MRI shows cortical ribboning (DWI hyperintensity in
cortex) and basal ganglia involvement. PRNP
mutations (genetic CJD) or acquired forms possible.
3. A 68-year-old man with a history of Parkinson
disease (PD) for 10 years develops visual
hallucinations, fluctuating cognition, and falls. At
autopsy, the brain shows Lewy bodies (alpha-
synuclein positive) in the brainstem (substantia nigra)
,and diffusely in the cortex (temporal, frontal). What is
the most likely diagnosis?
A) Parkinson disease
B) Dementia with Lewy bodies (DLB)
C) Multiple system atrophy (MSA)
D) Progressive supranuclear palsy (PSP)
Correct answer: B
Rationale: Dementia with Lewy bodies (DLB) is
defined by dementia occurring before or within 1
year of parkinsonism (or dementia with cortical Lewy
bodies). Pathologically, Lewy bodies are widespread
in cortex and brainstem. Parkinson disease (A) has
Lewy bodies restricted to brainstem (substantia
nigra).
4. A 60-year-old man presents with vertical gaze
palsy, axial rigidity, and falls early in the disease
course (within first year). At autopsy, the brain shows
neuronal loss, gliosis, and neurofibrillary tangles
(tau-positive) in the subthalamic nucleus, globus
pallidus, and substantia nigra. The tau isoform is
predominantly 4-repeat. What is the most likely
diagnosis?
A) Progressive supranuclear palsy (PSP)
B) Corticobasal degeneration (CBD)
, C) Multiple system atrophy (MSA)
D) Pick disease
Correct answer: A
Rationale: PSP (Steele-Richardson-Olszewski
syndrome) presents with vertical supranuclear gaze
palsy, axial rigidity, early falls, and tau pathology (4R
tau) in subthalamic nucleus (tufted astrocytes). CBD
(B) has asymmetric apraxia and tau pathology
(astrocytic plaques).
5. A 55-year-old woman presents with asymmetric
limb apraxia (alien limb phenomenon), cortical
sensory loss, and dystonia. At autopsy, the brain
shows frontoparietal atrophy with ballooned neurons
(tau-positive) and astrocytic plaques in the cortex
and basal ganglia. Tau isoform is 4-repeat. What is
the most likely diagnosis?
A) Progressive supranuclear palsy (PSP)
B) Corticobasal degeneration (CBD)
C) Pick disease
D) Argyrophilic grain disease
Correct answer: B
Rationale: Corticobasal degeneration (CBD) presents
with asymmetric apraxia (alien limb), cortical sensory
CERTIFICATION EXAM OFFERED BY
AMERICAN BOARD OF PATHOLOGY (ABMS) |
COMPLETE EXAM Q&A WITH RATIONALES
1. A 65-year-old man presents with progressive
memory loss, visuospatial deficits, and personality
changes over 5 years. At autopsy, the brain shows
diffuse cortical atrophy with temporal lobe
predominance. Microscopy reveals neurofibrillary
tangles (NFTs), neuritic plaques, and cerebral
amyloid angiopathy (CAA). Immunohistochemistry for
tau shows 3-repeat and 4-repeat isoforms. What is
the most likely diagnosis?
A) Pick disease
B) Alzheimer disease (AD)
C) Dementia with Lewy bodies (DLB)
D) Frontotemporal dementia (FTD)
Correct answer: B
Rationale: Alzheimer disease is defined by neuritic
plaques (amyloid-beta) and neurofibrillary tangles
(hyperphosphorylated tau, both 3R and 4R). The
clinical course (5+ years) and atrophy pattern are
consistent. Pick disease (A) has Pick bodies (3R tau).
,2. A 55-year-old man presents with rapidly
progressive dementia, myoclonus, and ataxia over 4
months. EEG shows periodic sharp wave complexes.
MRI shows hyperintensity in the basal ganglia and
cerebral cortex on DWI (cortical ribboning). What is
the most likely diagnosis?
A) Alzheimer disease
B) Creutzfeldt-Jakob disease (CJD)
C) Lewy body dementia
D) Progressive multifocal leukoencephalopathy (PML)
Correct answer: B
Rationale: Sporadic CJD (prion disease) presents
with rapidly progressive dementia (months),
myoclonus, ataxia, and characteristic EEG (PSWCs).
MRI shows cortical ribboning (DWI hyperintensity in
cortex) and basal ganglia involvement. PRNP
mutations (genetic CJD) or acquired forms possible.
3. A 68-year-old man with a history of Parkinson
disease (PD) for 10 years develops visual
hallucinations, fluctuating cognition, and falls. At
autopsy, the brain shows Lewy bodies (alpha-
synuclein positive) in the brainstem (substantia nigra)
,and diffusely in the cortex (temporal, frontal). What is
the most likely diagnosis?
A) Parkinson disease
B) Dementia with Lewy bodies (DLB)
C) Multiple system atrophy (MSA)
D) Progressive supranuclear palsy (PSP)
Correct answer: B
Rationale: Dementia with Lewy bodies (DLB) is
defined by dementia occurring before or within 1
year of parkinsonism (or dementia with cortical Lewy
bodies). Pathologically, Lewy bodies are widespread
in cortex and brainstem. Parkinson disease (A) has
Lewy bodies restricted to brainstem (substantia
nigra).
4. A 60-year-old man presents with vertical gaze
palsy, axial rigidity, and falls early in the disease
course (within first year). At autopsy, the brain shows
neuronal loss, gliosis, and neurofibrillary tangles
(tau-positive) in the subthalamic nucleus, globus
pallidus, and substantia nigra. The tau isoform is
predominantly 4-repeat. What is the most likely
diagnosis?
A) Progressive supranuclear palsy (PSP)
B) Corticobasal degeneration (CBD)
, C) Multiple system atrophy (MSA)
D) Pick disease
Correct answer: A
Rationale: PSP (Steele-Richardson-Olszewski
syndrome) presents with vertical supranuclear gaze
palsy, axial rigidity, early falls, and tau pathology (4R
tau) in subthalamic nucleus (tufted astrocytes). CBD
(B) has asymmetric apraxia and tau pathology
(astrocytic plaques).
5. A 55-year-old woman presents with asymmetric
limb apraxia (alien limb phenomenon), cortical
sensory loss, and dystonia. At autopsy, the brain
shows frontoparietal atrophy with ballooned neurons
(tau-positive) and astrocytic plaques in the cortex
and basal ganglia. Tau isoform is 4-repeat. What is
the most likely diagnosis?
A) Progressive supranuclear palsy (PSP)
B) Corticobasal degeneration (CBD)
C) Pick disease
D) Argyrophilic grain disease
Correct answer: B
Rationale: Corticobasal degeneration (CBD) presents
with asymmetric apraxia (alien limb), cortical sensory
