Degenerative path Flashcards
What is a common theme of degenerative neuro disease?
protein aggregates that are resistant to degradation via the ubiquitin-proteasome system
histologically recognized as cellular inclusions
What is the MC type of AD?
sporadic late onset type
What are the three stages of AD?
- early - insidious impairment of higher intellectual fx, alterations in mood and behavior
- later - progressive disorientations, memory loss, loss of math skills and learned motor skills, aphasia
- final - develop profound disability, may become incontinent, mute and or unable to walk
What are the two main processes of AD?
- extracellular deposition of amyloid Abeta
- senile plaques
- intracellular accumulation of tau protein
- neurofibrillary tangles
this H&E stain shows…
senile plaques
What are we looking at?
beta amyloid immunostain
What are we looking at?
neurofibrilary tangles - flame shape or globose tangles
What are we looking at?
NFT on Bielschowsky stain, commonly found in cortical and other neurons
What is seen in about 90% of AD?
this increases the risk of infarcts and leukoencephalopathy
cerebral amyloid angiopathy
- deposition of Abeta amyloid in the walls of small vessels of the brain results in narrowing of the lumen and loss of smooth muscle
What is this called? What disease is it seen in?
walnut brain
Pick Disease
In AD, when there is compensatory ventricular enlargement after cortical atrophy, this is called…
hydrocephalus ex vacuo
What is the prototypical frontotemporal lobar degenerative tauopathy?
What are associations with it?
Pick Disease
‘knife-blade’ atrophy of frontal and temporal lobes (walnut brain), which may be asymmetrical
What do I see microscopically in Pick Disease?
- Pick cells (left) - ballooned neurons with dissolution of chromatin
- Pick bodies (right) - tau-positive spherical cytoplasmic inclusions in neuron cell bodies
The pathogenesis of PD is…
accumulation and aggregation of alpha synuclein
What is the diagnostic hallmark of PD?
What are they immunoreactive for?
Lewy bodies
ubiquitin and alpha-synuclein