17.7 Dementia & Degen Disorders Flashcards by Michelle Cho

What is the protein that carries an INcreased risk of developing AD? Why?

-Epsilon-4-ApoE-Increases APP conversion to the beta form

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What causes the suppressed expression of genes with Huntington’s disease?

Deacetylation of histones

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What is the pathophysiology of normal pressure hydrocephalus?

Unknown etiology of inability to resorb CSF in the arachnoid granulations

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What is the normal configuration of prion protein? Abnormal?

-Normal = Alpha (PrP^c)-Abnormal = beta sheet (PrP^sc)

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What acute treatment can be performed for normal pressure hydrocephalus?

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What are the characteristics of the EEG with CJD?

Spike wave complexes

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What are Lewy bodies composed of?

Alpha-synuclein

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What are the major clinical features of PD? (4)

-Tremor-Rigidity-Akinesia/bradykinesia -Postural instability and shuffling gait

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Cogwheel rigidity = ?

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What is the major pathophysiology of Huntington’s disease?

Degeneration of the GABAergic neurons in the caudate nucleus of the BG

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True or false: most cases of Alzheimer’s are inherited

False–sporadic

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What are characteristic of the hallucinations of lewy body dementia?

Vivid and benign

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Is huntington’s disease AD or AR? What is the genetic defect, and what protein is affected?

AD repeat of CAG in the huntingtin gene

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Why is it that patients with Down syndrome develop Alzheimer’s?

Chromosome 21 is where APP is—thus they express more of it

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What are the most important risk factors for the development of early AD? (2)

-Presenilin 1 or 2 gene-Down Syndrome

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What is the natural h/o CJD?

Usually results in death in less than 1 year

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What is the protein that carries a DEcreased risk of developing AD?

-Epsilon-2-ApoE

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What are the two major gray matter areas in the brain?

-Cerebral hemispheres-BG

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Are there focal neurological deficits with Alzheimer’s?

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What are the genes that increase the risk for later Alzheimer’s disease?

Epsilon-ApoE4

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What is the protein that accumulates in Pick’s disease?

-Spherical tau protein aggregates-TDP43

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Degeneration of the brainstem and BG leads to what?

Movement disorders

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What is the treatment for normal pressure hydrocephalus?

VP shunt

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What is the key gross finding of Huntington’s disease?

Loss of the caudate nucleus and resulting hydrocephalus ex vacuo

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What are the characteristics of Lewy body dementia?

-Dementia-Hallucinations-Parkinsonian features

Mask like facies = ?

What is the usual presentation of CJD? (3)

-Rapidly progressive dementia -ataxia-startle myoclonus

What is vCJD?

Exposure to bovine protein

What is the most common cause of dementia?

Alzheimers

What is the key risk factor for the development of PD?

Aging

Where does the substantia nigra project to? What neurotransmitters are released here?

-Dopamine-Striatum

What is cerebral amyloid angiopathy?

A-beta amyloid in AD deposits around blood vessels, weakening them and leading to hemorrhage

What causes the anticipation seen in HD?

Further expansion of CAG during spermatogenesis

What is the usual cause of CJD?

Sporadic--rarely arises due to exposure to prion infected human tissue

What are the two major gray matter structures of the striatum?

-Caudate-Putamen

What is the cause of Parkinson's disease?

Degenerative loss of dopaminergic neurons in the substantia nigra pars compacta of the BG

What is the drug that is known for causing PD?

MPTP

What generally causes vascular dementia?

HTN, atherosclerosis, or vasculitis

What, generally, is hydrocephalus ex vacuo?

The appearance of enlarged ventricles 2/2 loss of cerebral matter

What is the role of the caudate on the cortex?

Project GABA to inhibit movement

What are the genes that increase the risk for early Alzheimer's disease?

Presenilin 1 or 2

What is the effect of beta (PrP^sc) on other proteins in the brain?

Produces more of itself, which is not degradable

What is the pathogenesis of Alzheimer's disease?

Disruption of the degradation of APP into beta amyloid, leading to A-beta-amyloid deposition

True or false: AD is a diagnosis of exclusion

True--confirmed with pathology at autopsy

What is the effect of D1 and D2 binding on the striatum, on the cortex?

D1 binding = increased stimulationD2 = decreased inhibition

What is familial fatal insomnia?

Prion disease that causes progressive insomnia, exaggerated startle response, and death

What is the basic pathogenesis of dementia?

Loss of neurons within the gray matter

What is normal pressure hydrocephalus?

CSF builds up, resulting in dilated ventricles

What are the three classic symptoms of normal pressure hydrocephalus?

DementiaAtaxiaUrinary incontinence("wacky, wobbly, and wet")

Pill rolling tremor = ?

What is normal role of Tau protein in neurons?

Helps to organize microtubules

What is the second most common cause of dementia?

Vascular dementia

What is the natural h/o vascular dementia?

Stepwise deterioration of mental functioning

What are the three ways to acquire prion disease?

-Sporadically-Inherited-Transmitted

What are the first few symptoms of Pick's disease?

Early behavior and language symptoms, followed by dementia

What are the nerve fibers that run along the edges of the ventricles, and are stretched in normal pressure hydrocephalus (which causes s/sx)?

Corona radiata

Degeneration of what large area of the brain leads to dementia?

Cortex

What are the characteristics hallmarks of spongiform encephalopathy?

Vacuoles in the brain

What is Pick's disease (aka frontotemporal dementia)?

Degenerative disease of the frontal and temporal cortex, that spares the parietal and occipital lobes

Is the memory loss with Alzheimer's fast or slow?

-Slow memory loss-Progressive disorientation-Loss of learned motor skills-Behavior and personality changes-Mute and bedridden

Where are Lewy bodies found in lewy body dementia?

Cortices

What are the neurofibrillary tangles seen in AD?

Hyperphosphorylated Tau protein in cells

What are the neuritic plaques seen in AD?

Pink extracellular AB amyloid, surrounded by entrapped neuritic processes

What are the gross characteristics of brains with AD?

Diffuse cerebral atrophy:-Narrowing of gyri-Widening of the sulci

What is the most important risk factor for the development of sporadic AD?

Age

Early onset dementia in the setting of suspected PD, is called what?

Lewy body dementia--although it is the same pathophysiologic progress underpinning it.

What separates the caudate and the putamen on a coronal cut of the brain?

Anterior segment of the internal capsule

What is the startle myoclonus seen with CJD?

Minimal stimuli causes myoclonus

What is the area of the BG that sends either stimulatory or inhibitory signals to the cortex?

Striatum