Myelinating Disorders, Seizures and Neurocutaneous disorders Flashcards
(35 cards)
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
Autoimmune inflammation and demyelination of oligodendrocytes?
MS
Who does MS most commonly affect?
20-30 year old white woman who has eye symptoms
What is the SIN in MS?
Scanning speech
Intention tremor (also Incontinence and
Internuclear ophthalmoplegia=>Bilateral MLF)
Nystagmus
What Dx of MS?
Oligoclonal bands
MS is associated with?
HLA-DR2
two words in MS
MS symptoms are seen after taking a?
Hot Shower
With MS, how will patients eye react to light if the affected eye is the right?
Light on Right=>No change
Light On Left=>Both eyes constrict
destroys Schwann cells?
GBS
What do you see with GBS?
Increased CSF protein with normal cell count=>albuminocytologic dissociation
