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33 Cards in this Set

  • Front
  • Back
A definition of dementia should include the following criteria
Cognitive impairment is acquired
Impairment involves multiple domains of cognitive function
No impairment of arousal
What is apraxia
inability to perform learned movements that is not due to weakness/incoordination or failure to comprehend (in the differential for dementia)
Average duration of Alzheimer's
8-12 yrs
Major findings in the 3 clinical stages of Alzheimer's
Stage I- memory disturbances, indifference, irritability
Stage II- aphasia, indifference, incontinence
Stage III- intellectual functions effected, worsening language, motor disturbance
Amyloid precursor protein is on gene ___ and contributes to ___ cases of Alzheimer's
Chromosome 21, <.01% of AD
Proteinopathy most significant in AD is related to
Alpha Beta peptides which are endocytosed, elicit a response in astrocytes and microglia and are toxic
Gross pathology of Alzheimer's disease shows
atrophy with hydrocephalus ex vacuo
*pronounced atrophy inthe medial temporal lobe
Neurofibrillary tangles are composed of
hyper-phosphorylated forms of tau (a microtubular associated protein)
ubiquiting, and AlphaBeta Protein (precursor of APP)
Neuritic plaques are found in which disease and what are they
Alzheimer's
consist of dystrophic neurites and mitochondria
Heavy involvement of neuritic plaques in AD are in which -

Which notable regions are spared-
Association cortex, posterior cingulate gyrus, and amygdala

Spared- primary motor, sensory, and visual
What are Hirano bodies and where are they found
Eosinophilic intracytoplasmic inclusions found in pyramidal neurons of hippocampus
Hirano bodies are immunoreactive for
actin
Where is granulovacuolar degeneration found and how is their immunoreactivity?
pyramidal neurons of the hippocampus
Tubulin-like immunoreactivity
Where in the brain should you look for Lewy bodies in Alzheimer's
Anterior cingulate gyrus

can also look in midbrain substantia nigra
Pick's disease is what underlying pathology?
Tauopathy
Frontotemporal dementia and perkinsonism are classified as---
Taupathies
Compare presentation of frontotemporal dementia to AD
memory deficit may not be present in FTD initially (like it is in AD) and FTD is more behavioral and language related
Do Frontotemporal dementias have plaques and tangles
No, at least not in same levels as AD
FTDP-17 is-
a group of autosomal dominant progressive neurodegenerative syndromes
Alpha-synuclein is involved in what function
neurofilament chaperones
Describe classical Lewy Bodies
Brainstem type, spherical with eosinophilic core and pale halo
Describe Cortical Lewy Bodies
Small neurons in cortical layers V and VI, less clearly defined than classical, lack halo
Cortical Lewy Bodies stain for phosphorylated neurophilaments, ubiquiting, synuclein but DO NOT stain for
tau
In dementia with Lewy Bodies what is the time course of the memory impairment like
It may not be prominent in early stages
Compared to AD, vascular dementia has an earlier or later onset?
Vascular onset is earlier
Describe the prion protein in the disease state
The alpha helix changes to Beta sheet, it is resistant to digestion and induces conformational change in normal PrP molecules
What are clinical syndromes of CJD
non-specific malaise followed by neurological signs, dementia, pyramidal signs, end stage is vegetative with seizures
What laboratory test may be abnormal with CJD
14-3-3 protein
What changes does CJD cause histologically in the cortex
microvacuolation of cortex
Name 4 nutritional deficiencies that can cause dementia
Thiamine, B12, B6, Niacin
What is a clinical syndrome of Thiamine deficiency
delirium, ataxic gait, abnormal eye movements with nystagmus
Describe Korsakoff's Syndrome
results from untreated Wernicke's encephalopathy
Causes retrograde amnesia, atnerograde amnesia, confabulation
l
Where is the loss of neurons in Korsakoff's Syndrome
Medial Dorsal Nucleus of Thalamus