Myelinating Disorders, Seizures and Neurocutaneous disorders Flashcards
What kind of people have increased risk of developing Alzheimer?
Down Syndrome
MCC dementia?
Alzheimer
APP presenilin-1, presenilin-2: risk of?
early onset (1,2 year old kids)
With ApoE4 is there increased or decreased risk?
Increased
What gross findings do you see in Alzheimer Dementia?
Widespread cortical atrophy. Narrowing of gyri and widening of sulci
Alzheimer amyloid can deposit where? causing what?
amyloid angiopathy=> intracranial hemorrhage
What is the problematic protein?
Αβ (amyloid-β)
With Alzheimer disease, you see a neurofibrillary tangle, what is made of?
intracellular, hyperphosphorylated tau protein=>insoluble cytoskeleton elements
Frontotemporal (pick’s disease) vs Alzheimer?
Frontotemporal-pick’s disease=> earlier onset (50-60)+personality changes
Alzheimer=> (60+)+memory changes
In pick’s disease you get pick’s bodies; what are they?
silver-staining spherical tau protein aggregates
you pick your spherical boogers
Early onset dementia and visual hallucinations
Lewy Body Dementia
“haLewycinations”
Rapidly progressive (weeks to months) dementia with myoclonus (“startle myoclonus”)?
CJD
In CJD, prion PrP C becomes? What is the consequence?
C=>SC
=>B-pleated sheet resistant to proteases
CJD prions have what EEG?
Spiked wave complex on EEG
2nd most common cause of dementia in elderly?
Multi-infarct (aka vascular) dementia
