Flashcards in Path Notes Deck (32):
What is the key to pathogenesis of CNS Dementia and neurodegenerative disorders?
Abnormal protein homeostasis
Gender difference in Alzheimer's
Women slightly more likely
-odds for any one woman now exceeds breast cancer
Where do amyloid plaques tend to be more numerous?
Deep cortical layers
What are filamentous intracellular inclusions made of? What are they called?
-Neurfibrillary tangles (NFTs)
-Neuropil thrads (NTs)
What part of the brain does plaques and tangles spare?
Occipital lobe, primary motor cortex
Where can plaques and tangles be found early on in Alzheimers?
What are the 2 AB peptides?
AB 40 = bad
AB 42 = worse, seed
What is amyloid angiopathy?
AB deposits in cortical and leptomeningeal arteries and arterioles
What are Hirano bodies?
Intracellular aggregates of actin and actin-associated proteins
Where are Hirano bodies frequently found?
Hippocampal pyramidal cells
What is Tau?
microtubule associated protein
What happens to Tau in Alzheimer's?
-Lose ability to bind to microtubules
What ApoE alleles have higher/lower risk for AD?
E4 = higher risk
E2 = lower risk
What 3 Genes are associated with early onset AD
All autosomal dominant
3 Types of Frontotemporal Lobar Degeneration (FTLD):
Knife-edge gross examination of brain suggests:
Tau form of Frontotemporal Lobar Degeneration (FTLD)
Which type of FTLD is related to ALS?
Which type of FTLD is most common?
3 Hallmark symptoms of Parkinson's:
What are Lewy Bodies made of?
Difference of Progressive Supranuclear Palsy vs. Parkinson's
No Lewy Bodies in PSP
Important exam finding in PSP:
Difficulty with voluntary eye movements
Histological findings in PSP:
Exam and histological findings in Corticobasal Degernation (CBD)
Exam: jerking of limbs, dyspraxia (cognitive dysfunction)
Histology: Ballooned neuron
When does trinucleotide repeat expansion occur in Huntingtons?
Where does atrophy occur in Huntington's?
Caudate and Striate
Freidreich's ataxia age of onset:
1st or 2nd decade of life
Disease associated with Freidreich's Ataxia:
Diabetes (1/4 of patients)
Genetics of Freidreich's Ataxia:
-GAA trinucleotide repeat on 9q13 frataxin protein
Exam finding of ALS:
Proteins associated with Spinal Muscular Atrophy
-Gene needed for motor neuron survival