6- Degenerative Disease

Question 1

understand common symptoms and presentation of patients with delirium

Answer 1

1) fluctuating consciousness
2) impaired attention (can’t focus on salient aspects)
3) incoherent speech
4) toxic/metab causes

Question 2

common symptoms and presentation of patients with dementia

Answer 2

normal LOC

trouble with memory,
visuospatial skills, complex cognition, emotion or personality INTEREFERES WITH usual social/occup function

deterioration of global intellectual ability

Question 3

Features of Alzheimer’s disease

Answer 3

early memory and visuospatial (can’t put on clothes or can’t find new places) (problems

Question 4

early memory and visuospatial problems

Answer 4

Alzheimer’s disease

Question 5

Features of Frontotemporal dementia

Answer 5

early behavioral, executive, and or language deficits

inappropriate sexual comments
motor/cognition retained

Question 6

early behavioral, executive, and or language deficits

Answer 6

frontotemporal dementia

Question 7

features of parkinson’s disease

Answer 7

rest tremor, rigidity, bradykinesia

Question 8

tremor, rigidity, bradykinesia

Answer 8

parkinson’s disease

Question 9

features of lewy body dementia

Answer 9

early parkinsonian features
psychosis
fluctuating consciousness
]visual hallucinations

decr DA and ACh

lewy bodies + synuclein

Question 10

early parkinsonian features
psychosis (fixed false belief)
fluctuating consciousness
]visual hallucinations

Answer 10

lewy body dementia

Question 11

progressive supranuclear palsy

Answer 11

bradykinesia
rigidity
falls (earlier than parkinson’s)
abnormal vertical eye movements (lose volitional eye movements)

Question 12

bradykinesia
rigidity
falls
abnormal vertical eye movements

Answer 12

progressive supranuclear palsy

Question 13

features of als

Answer 13

weakness and atrophy
fasciuclations
both UMN and LMN signs

Question 14

weakness and atrophy
fasciuclations
both UMN and LMN signs

Answer 14

als

Question 15

features of huntington’s disease

Answer 15

dementia (acquired sociopathy,
substance abuse
driving problems

depression

chorea

Question 16

dementia
depression
chorea

Answer 16

huntington’s disease

Question 17

features of creutzfeldt-jakob disease

Answer 17

rapidly progressive dementia

myoclonus (high velocity twitching movements faster than volitional movements)

prion disease

Question 18

rapidly progressive dementia

myoclonus

Answer 18

creutzfeldt jakob disease

Question 19

define prion

where is it found in most human tissues?

disease due to?

Answer 19

small infectious pathogen with protein but no nucleic acid

found in normal CNS

conformational change in protein

Question 20

common features of prion disease

method of spread

age of onset?

Answer 20

ataxia
abnormal movements
visuospatial/exec dysfunction, cognitive problem

sporadic, heritable, and transmissible

usu 55-65 y/o

Question 21

how does vCJD differ from typical sporadic CJD

Answer 21

1) younger age at present

2) slower progression

Question 22

describe kuru

transmitted by

Answer 22

early tremor
ataxia
dementia late in course

transmitted by ritual cannibalism

Question 23

early tremor
ataxia
dementia late in course

Answer 23

kuru

Question 24

describe gerstmann syndrome

Answer 24

AD

clumsiness
incoordination
gait ataxia

variation within family

Question 25

AD

clumsiness
incoordination
gait ataxia

Answer 25

gerstmann syndrome

Question 26

fatal familial insomnia

onset age?

Answer 26

AD

35-60 y/o

insomnia, memory loss, confusion, hallucinations

Question 27

insomnia, memory loss, confusion, hallucinations

Answer 27

fata familial insomnia

Question 28

define neurodegenerative dz

Answer 28

spontaneous death of neurons

either due to intra/extracellular abnormal protein accum

Question 29

clinical presentation of neurodegenerative disease

Answer 29

1) dementia = memory, language, exec fxn
depression, apathy, sociopathy

2) movement disorders= bradykinesia, rigidity, chorea, tremor
3) motor problems = weakness, atrophy

Question 30

diseases under dementia

Answer 30

1) alzheimer’s
2) FTD
3) lewy body
4) huntington’s
5) CJD

Question 31

diseases under movement

Answer 31

1) parkinson’s
2) PSP
3) CJD
4) huntington’s

Question 32

diseases under motor problems

Answer 32

1) ALS

2) ALS + FTD

Question 33

Genetics of alzherimer’s

young onset ( less than 40) most cases are ____

young onset

Answer 33

usu sporadic

presenilin 1 mutation

trisomy 21

Question 34

tau protein (tangles) accum in which diz

synuclein means what?

ubiquitin means what?

polyglutamine means what?

prion protein means?

Answer 34

1) FTD and PSP
2) parkinson’s
3) ALS
4) Huntington’s
5) CJD

Question 35

ACh changes assoc with what?

DA changes assoc with what?

serotonin assoc with what?

Answer 35

Alzheimer’s and Lewy body

parkinson’s and lewy body

FTD

Question 36

classic findings of alzheimer’s neuropath

Answer 36

NEED BOTH
neuritic (amyloid plaques and neurofibrillary tangles

diffuse atrophy (hydrocephalus ex vacuo)

spares deep structures

Question 37

neuritic (amyloid plaques and neurofibrillary tangles

diffuse atrophy (hydrocephalus ex vacuo)

spares deep structures

Answer 37

alzheimer’s

Question 38

4 different FTD pathologies

if have tau-reactive intraneuronal inclusions then

Answer 38

1) neurofibrillary tangles (tau deposits)
2) ubiquitin inclusions

–> pick bodies

dementa wtihout histo features (decr neuron and cortex)

Question 39

Huntington’s disease assoc with what gross brain features

inheritance?

genetics?

Answer 39

marked caudate atrophy

AD

incr CAG repeats on chromosome 4

Question 40

only neurodegenerative disease that is transmissible

Answer 40

CJD