neuro12

Question 1

classic pathology for alzheimer’s disease

Answer 1

neurofibrillary tangles (NFTs) and senile plaques in the cerebral neocortex and hippocampus; loss of neurons in the cerebral cortex and gliosis

Question 2

factors assoc with increased risk of alzheimers

Answer 2

female, age, history of head trauma, and Down syndrome

Question 3

gene assoc with alzheimers

Answer 3

ApoE4 on chrom 19 assoc with both early and late onset AD

Question 4

pathogenesis of alzheimers

Answer 4

amyloid beta precursor protein mutations cause increased amyloid beta production with subsequent aggregation in neurons;

Question 5

sundowning

Answer 5

seen in alzheimers; worsening psych manifestations during the evening and night; patient usually dies within 5-10 years

Question 6

possible early diagnostic markers of alzheimers

Answer 6

elevated tau protein and low A-beta-42 in the CSF

Question 7

MRI in alzheimers

Answer 7

may show up to 40% reduction in size of hippocampus, amygdala, and thalamus

Question 8

what are NFTs

Answer 8

intracellular accum of phosphorylated tau protein

Question 9

what are senile plaques

Answer 9

extracellular deposits of amyloid surrounded by dystrophic axons

Question 10

treatment for alzheimers

Answer 10

acetylcholinesterase inhib like donepezil or rivastigmine, as well as memantine ,an NMDA receptor antag

Question 11

prophylaxis for alzheimers?

Answer 11

use of vitamin E, NSAIDs, and estrogens has been proposed; no good data

Question 12

average length of time from symptom onset to diagnosis for alzheimers

Answer 12

2 to 3 years

Question 13

diagnosis of vascular dementia

Answer 13

presence of dementia and two or more of the following: focal neuro signs; onset that is abrupt, stepwise or stroke-related; or brain imaging showing multiple strokes, lacunes, or extensive deep white matter changes

Question 14

vascular dementia is assoc with what

Answer 14

Binswanger disease (microvascular disease), a lacunar state, and large strokes

Question 15

dementia with lewy bodies clinical picture

Answer 15

looks like parkisonism but with visual hallucinations;

Question 16

dementia with lewy bodies has a marked worsening with what drug

Answer 16

haloperidol and other neuroleptics

Question 17

patholgic hallmark of dementia with Lewy bodies

Answer 17

Lewy body (eosinophilic intracellular inclusion of alpha synuclein), but can also have NFTs and amyloid plaques like in AD

Question 18

difference between Lewy body dementia and PD

Answer 18

both have Lewy bodies; in PD, they are in the substantia nigra; in Lewy body dementia they are in the cortical neurons

Question 19

management of lewy body dementia

Answer 19

low dose of atypical neuroleptics like risperidone and quetiapine

Question 20

three key distinguishing features of dementia with Lewy bodies

Answer 20

fluctuations of alerteness, visual hallucinations, and an extraordinary sensitiviey to neuroleptics

Question 21

life span in dementia with Lewy bodies

Answer 21

usually in 10 to 15 years

Question 22

Steele-Richardson-Olszewski syndrome AKA

Answer 22

progressiv supranuclear palsy

Question 23

main features of progressive supranuc palsy

Answer 23

supranuc ocular palsys (failure of vertical gaze), dysarthria, dysphagia, extrapyramidal rigidity, gait ataxia, and dementia; falls and gait abnormalities;

Question 24

other feautres of progressive supranuclear palsy

Answer 24

dementia may occur early or develop later; frontal lobe abnormalities predominate. Patients may become apathetic

Question 25

diagnostic eval of progressive supranuc palsy

Answer 25

atrophy of the dorsal midbrain, globus pallidus, and subthal nuc

Question 26

characteristic path of progressive supranuc palsy

Answer 26

NFTs, neuronal loss, and gliosis in many subcortical structures

Question 27

Huntington disease- lesions where

Answer 27

basal ganglia

Question 28

symptoms of HD appear when

Answer 28

between ages of 35 and 45

Question 29

triad of huntington's

Answer 29

chorea, behavioral changes or personality disorder (frequently OCD), and dementia

Question 30

diagnosis of HD

Answer 30

atrophy of the caudate on brain imaging and demonstration of more than 40 CAG repeats in the HD gene on chrom 4

Question 31

pathology of HD

Answer 31

severe destruction of the caudate and putamen (striatal GABA-ergic neurons) and loss of neurons in the cerebral cortex (layer 3)

Question 32

pathology of HD- next

Answer 32

; the HD gene leads to aberrant processing of cell rpoteins with formation of deposits in the nucleus and activation of intracellular mechanisms of death

Question 33

treatment for HD

Answer 33

pharmacologic management of dementia and chorea with dopaminergic antags, including neuroleptic drugs

Question 34

life expectancy in HD

Answer 34

10 to 20 years after onset

Question 35

can parkinsons disease have dementia

Answer 35

yes, can cause subcortical dementia; about 30% of patients with PD have dementia

Question 36

frontotemporal lobar dementia

Answer 36

significant alterations in personaluty, social behavior, and language

Question 37

how does FTLD present initially

Answer 37

cognitive and behavioral deficits other than memory loss

Question 38

types of FTLD

Answer 38

frontotemporal dementia; nonfluent aphasia, and semantic dementia

Question 39

molecular pathology of FTLD

Answer 39

abnormalities in tau protien or in a protein called TDP-43

Question 40

prion-related diseases that cause dementia

Answer 40

Creutzfeld-akob disease; Gerstmann-Straussler-Scheinker syndrome; and fatal familial insomnia

Question 41

pathology of prion diseases

Answer 41

spongy degeneration, neuronal loss, gliosis, and astrocytic proliferation resulting from accum in the brain of a mutated protease-resistant prion protein

Question 42

most common prion disease

Answer 42

Creutzfeld-jakob disease

Question 43

pyramidal sign vs extrapyramidal sign

Answer 43

pyramidal is voluntary motor; extrapyramidal is invol motor like basal gang and cerebellum; damage to extrapyramidal system results in dyskinesias

Question 44

characteristics of dementia of CJD

Answer 44

rapidly progressive dementia with pyramidal signs, myoclonus, cerebellar or extrapyramidal signs, and periodic sharp waves on EEG; focal neuro signs

Question 45

MRI in CJD

Answer 45

evolving cortical and basal gang abnormalities during the course of the disease

Question 46

CSF in CJD

Answer 46

typically normal, but the presence f protein 14-3-3 is relatively sens and specific for CJD

Question 47

prognosis for CJD

Answer 47

no treatment; most die within a year

Question 48

HIV-associated dementia complex

Answer 48

patients report memory problems, difficulty with concentration, and poor attention; MRI shows cortical and subcortical atrophy

Question 49

metabolic causes of dementia

Answer 49

Wilson disease, hypothyroidism, vit B12 def, hypercalcemia, Addison disease

Question 50

characteristics of B12 def

Answer 50

dementia, SC, and peripheral nervous system disfunction; neuropathy and memory loss; anemia and sore tongue

Question 51

treatments for alzheimers

Answer 51

cholinesterase inhib (donepezil or rivastigmine or galantamine) or NMDA receptor antag (memantine)

Question 52

REM sleep behavior disorder

Answer 52

clonazepam is treatment; often seen in dementia with lewy bodies