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The Basics
Degenerative Dx: Affects gray matter, neurons with associated 2 white matter changes
Pathogenesis usually involves protein aggregation
Neuronal loss is selective
No clear inciting event or previous deficits, diagnosis of exclusion
Locations of degenerative Dx
Cortex (Alzheimer, FTLD, Vascular Dementia)
Basal Ganglia (Parkinson, Huntington)
MNs (ALS, Werdnig Hoffman Dx)
I. Cortical Degenerative Diseases
1. Dementia: Degenerative cortical Sd characterized by higher cortical fxn without clouding of
consciousness
a. Caused by degenerative Dx, chronic infection, vitamin deficiency, toxins, diffuse injury, metabolic
disorders, mass lesions, genetic Dx
2. Alzheimer Dx (Senile Dementia of Alzheimers Type)/AD
a. Most common cause of elderly dementia, rarely symptomatic before 50
i) 1% of 60-65, 40% of 85-89
b. 5-10% cases are familial (early onset is AD)
c. Some Trisomy 21 patients have a form of AD (Ch 21 codes for cerebral amyloid)
d. Diagnose w/ clinical tests & radiographs (tissue examination is definitive)
e. Pathogenesis: Accumulation of abnormally cleaved (-secretase) A amyloid and hyperphosphorylated
Tau
f. Gross: Variable cortical atrophy in frontal, parietal, temporal lobes hydrocephalus ex vacuo
g. Micro: A amyloid plaques extracellularly, tau tangles intracellularly (extracellular after neuron death),
amyloid angiopathy (A40 amyloid deposited around vessels, can occur without Alzheimer)
i) Also found in elderly nondemented patients
ii) Tangles contain paired helices of Tau (dementia associated with synapses, tangles)
iii) Amyloid stains w/ Congo Red, shows apple-green birefringence under polarized light
iv) Granulovacuolar degeneration: Small, clear intraneuronal vacuoles (hippocampus, olfactory bulb)
that stain w/ silver stains
v) Hirano bodies: Glassy eosinophilic bodies (paracrystalline actin) in hippocampus pyramidal cells
h. Entorhinal cortical atrophy loss of spatial direction, hippocampal atrophy memory loss,
neocortical atrophy higher cortical fxn