6- Degenerative Disease Flashcards
understand common symptoms and presentation of patients with delirium
1) fluctuating consciousness
2) impaired attention (can’t focus on salient aspects)
3) incoherent speech
4) toxic/metab causes
common symptoms and presentation of patients with dementia
normal LOC
trouble with memory,
visuospatial skills, complex cognition, emotion or personality INTEREFERES WITH usual social/occup function
deterioration of global intellectual ability
Features of Alzheimer’s disease
early memory and visuospatial (can’t put on clothes or can’t find new places) (problems
early memory and visuospatial problems
Alzheimer’s disease
Features of Frontotemporal dementia
early behavioral, executive, and or language deficits
inappropriate sexual comments
motor/cognition retained
early behavioral, executive, and or language deficits
frontotemporal dementia
features of parkinson’s disease
rest tremor, rigidity, bradykinesia
tremor, rigidity, bradykinesia
parkinson’s disease
features of lewy body dementia
early parkinsonian features
psychosis
fluctuating consciousness
]visual hallucinations
decr DA and ACh
lewy bodies + synuclein
early parkinsonian features
psychosis (fixed false belief)
fluctuating consciousness
]visual hallucinations
lewy body dementia
progressive supranuclear palsy
bradykinesia
rigidity
falls (earlier than parkinson’s)
abnormal vertical eye movements (lose volitional eye movements)
bradykinesia
rigidity
falls
abnormal vertical eye movements
progressive supranuclear palsy
features of als
weakness and atrophy
fasciuclations
both UMN and LMN signs
weakness and atrophy
fasciuclations
both UMN and LMN signs
als
features of huntington’s disease
dementia (acquired sociopathy,
substance abuse
driving problems
depression
chorea
dementia
depression
chorea
huntington’s disease
features of creutzfeldt-jakob disease
rapidly progressive dementia
myoclonus (high velocity twitching movements faster than volitional movements)
prion disease
rapidly progressive dementia
myoclonus
creutzfeldt jakob disease
define prion
where is it found in most human tissues?
disease due to?
small infectious pathogen with protein but no nucleic acid
found in normal CNS
conformational change in protein
common features of prion disease
method of spread
age of onset?
ataxia
abnormal movements
visuospatial/exec dysfunction, cognitive problem
sporadic, heritable, and transmissible
usu 55-65 y/o
how does vCJD differ from typical sporadic CJD
1) younger age at present
2) slower progression
describe kuru
transmitted by
early tremor
ataxia
dementia late in course
transmitted by ritual cannibalism
early tremor
ataxia
dementia late in course
kuru
describe gerstmann syndrome
AD
clumsiness
incoordination
gait ataxia
variation within family
AD
clumsiness
incoordination
gait ataxia
gerstmann syndrome
fatal familial insomnia
onset age?
AD
35-60 y/o
insomnia, memory loss, confusion, hallucinations
insomnia, memory loss, confusion, hallucinations
fata familial insomnia
define neurodegenerative dz
spontaneous death of neurons
either due to intra/extracellular abnormal protein accum
clinical presentation of neurodegenerative disease
1) dementia = memory, language, exec fxn
depression, apathy, sociopathy
2) movement disorders= bradykinesia, rigidity, chorea, tremor
3) motor problems = weakness, atrophy
diseases under dementia
1) alzheimer’s
2) FTD
3) lewy body
4) huntington’s
5) CJD
diseases under movement
1) parkinson’s
2) PSP
3) CJD
4) huntington’s
diseases under motor problems
1) ALS
2) ALS + FTD
Genetics of alzherimer’s
young onset ( less than 40) most cases are ____
young onset
usu sporadic
presenilin 1 mutation
trisomy 21
tau protein (tangles) accum in which diz
synuclein means what?
ubiquitin means what?
polyglutamine means what?
prion protein means?
1) FTD and PSP
2) parkinson’s
3) ALS
4) Huntington’s
5) CJD
ACh changes assoc with what?
DA changes assoc with what?
serotonin assoc with what?
Alzheimer’s and Lewy body
parkinson’s and lewy body
FTD
classic findings of alzheimer’s neuropath
NEED BOTH
neuritic (amyloid plaques and neurofibrillary tangles
diffuse atrophy (hydrocephalus ex vacuo)
spares deep structures
neuritic (amyloid plaques and neurofibrillary tangles
diffuse atrophy (hydrocephalus ex vacuo)
spares deep structures
alzheimer’s
4 different FTD pathologies
if have tau-reactive intraneuronal inclusions then
1) neurofibrillary tangles (tau deposits)
2) ubiquitin inclusions
–> pick bodies
dementa wtihout histo features (decr neuron and cortex)
Huntington’s disease assoc with what gross brain features
inheritance?
genetics?
marked caudate atrophy
AD
incr CAG repeats on chromosome 4
only neurodegenerative disease that is transmissible
CJD
