Neurodegenerative Pathology Flashcards
Genetics are not a common etiology of Alzheimer’s in those older than _____.
85
How does cortical atrophy appear on histologic exam?
Astrocytic gliosis (status spongiosus)
Neurofibrillary tangles appear on what kind of stain?
A silver stain (same one used by Alois Alzheimer’s)
Note: Silver stains do not pick up beta-amyloid plaques –only neurofibrillary tangles
The modern version of the silver stain is _____________.
immunohistochemical staining for tau and amyloid (which can detect pretangles)
Neurofibrillary tangles are composed of ___________.
hyperphosphorylated tau protein
Neurofibrillary tangles reside where?
Within the cytoplasm
Where do neurofibrillary tangles start (anatomically)?
In the transentorhinal cortex –the lower, lateral portion of the hippocampus
The amorphous material in the center of plaques is ___________.
beta-amyloid
What is a neurite?
A dead axon, “blown up” with tau protein
What is amyloid angiopathy?
Deposition of amyloid within blood vessels that stain in Congo staining; not diagnostic of Alzheimer’s, but very commonly seen – also seen in those without Alzheimer’s
The ABC system for describing the pathology of Alzheimer’s is ______________.
Amyloid deposition
Braak neurofibrillary stage
CERAD neuritic plaque density score
Final score breaks down to not, low, intermediate, high burden of disease.
Frontotemporal dementia can present as one of two forms: _______________.
progressive aphasic (40%) or behavioral (60%)
What protein does frontotemporal dementia present with?
TDP-43 = TAR-DNA binding Protein (this is ubiquitin positive tau, also found in ALS)
What cellular deposit is sometimes found (sparsely) in the anterior horn neurons of those with ALS?
Bunina bodies and ubiquitin-reactive inclusions
What protein accumulates in those with Parkinson’s?
Alpha-synuclein
The second-most common cause of dementia is ___________.
Lewy body dementia (this is when they present with dementia and then develop Parkinson’s)
Lewy body dementia causes profound atrophy in the __________ system.
limbic
On H&E staining, how do beta-amyloid plaques appear?
As amorphous pink globules with microglia and astrocytes radiating outward
Pick’s disease is a type of frontotemporal dementia that presents with ___________.
aphasia
Again, what are the four key features of Parkinson’s disease?
Hypokinesia/bradykinesia
Resting tremor
Cogwheel rigidity
Postural instability
What are some sources of non-idiopathic Parkinson’s?
Trauma (dementia pugilistica –Muhammad Ali)
Flu (particularly the Spanish flu of 1918)
MPTP (a contaminant of street drugs)
Familial Parkinsonism
Other than the substantia nigra, what structures are damaged in the brains of those with Parkinson’s?
The locus ceruleus
What presentation is typical of those with Lewy body dementia?
Progressive cognitive decline
Fluctuating states of consciousness and attention
Visual hallucinations (usually well-formed)
Spontaneous features of Parkinson’s
Those with Huntington’s have more than the usual amount of ______ CAG repeats on chromosome ____.
11 - 34; 4
__________ transmission of the Huntington’s gene leads to earlier onset.
Paternal
