Term
| define degeneration disease of CNS, what are the two basic types |
|
Definition
loss of neurons in gray matter
cortex gray matter: dementia
basal ganglia gray matter: movement disorders |
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Term
| what are the 8 kinds of dementia, what is #1 and #2 |
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Definition
infectious
vascular #2
metabolic
toxin
brain tumor
alzheimers
frontaltemporal (Pick's disease)
lewy body |
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Term
| 6 causes of infectious dementia |
|
Definition
prions
HIV
leukoencephalopathy
viral encephalitis
neurosyphilis
chronic meningitis |
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Term
| 3 causes of vascular dementia |
|
Definition
HTN
athlerosclerosis
vasculitis |
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Term
| effect of vascular dementia, complication |
|
Definition
| decreased blood causes hypoxia and multiple infarctions which can progress to subdural hematoma |
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Term
| 4 causes of metabolic dementia |
|
Definition
thiamine deficiency
B12 deficiency
niacin deficency (Pellagra)
endocrine disorders |
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|
Term
| what are the two diseases thiamine deficiency causes |
|
Definition
wernicke encephalopathy
korsakoff syndrome |
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Term
| wernicke encephalopathy: 3 symptoms, 2 complications |
|
Definition
confusion, abnormal eye movement, ataxia
hemorrhage and necrosis in mamillary bodies |
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|
Term
| korsakoff syndrome: cause, symptoms, reason for symptoms |
|
Definition
untreated wernickes, irreversible
lesion in medial dorsal nuclei of thalamus causes loss of memory |
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|
Term
| 4 causes of toxin dementia |
|
Definition
murcury
lead
manganese
bromides |
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|
Term
| what is the most common cause of dementia |
|
Definition
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|
Term
| how is alzheimers disease diagnosed 3 |
|
Definition
exclusion of other dementia
imaging
pathology |
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|
Term
| explain the normal processing of amyloid and the alzheimers disease processing |
|
Definition
amyloid protein receptors (unk function) are used and broken down by A SECRETASE into amyloid a which is degraded
when broken down by B SECRETASE it makes amyloid B which cannot be degraded and deposits on CORTEX GRAY MATTER |
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|
Term
| explain the normal progression of location of amyloid B deposition 3 |
|
Definition
| enterohinal cortex > hippocampal formation and isocortex > neocortex |
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|
Term
| what are the two types of alzheimer's disease |
|
Definition
|
|
Term
| what are the two causes of early alzheimers (explain each) |
|
Definition
familial: mutation in PRESENILIN 1 (Y SECRETASE PRODUCTION)
down syndrome: amyloid precursor protein IS ON CH21 so now there are THREE COPIES making it more likley to cause amyloid B production (45yo) |
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|
Term
| what is soporadic alzheimers caused by |
|
Definition
donno
associated with age (40%>85yo) |
|
|
Term
| what are three genes that are associated with alzheimers, what does there presence indicate |
|
Definition
apoE4: INCREASED RISK causes conversion of a secretase into B secretase causing more amyloid B production
apoE2: DECREASED RISK
SORL1: DEFICIENCY alters trafficing of amyloid precursor protein INCREASES RISK |
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|
Term
| 6 earlier / mid stage changes in alzheimers |
|
Definition
memory loss with SLOW ONSET AND WITHOUT LOC OR NEUROLOGICAL DEFICITS begning with RECENT MEMORY
disorientation
aphagia
cognitive decline
behavior change
personality change |
|
|
Term
| what are the 3 signs of alzheimers in 5-10y |
|
Definition
|
|
Term
| what do people with alzheimers usually die of |
|
Definition
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|
Term
| what are 4 morphology changes in dementia |
|
Definition
diffuse cerebral atrophy
neurotic plaques
cerebral amyloid angiopathy
neurofibrillary tangles |
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|
Term
| 3 signs of diffuse cerebral atrophy |
|
Definition
gyri narrow
sulci widen
ventricles dilate due to loss of mass (HYDROCEPHALUS EX VACUO) |
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|
Term
| how do neurotic plaques effect the brain in small and large quantities |
|
Definition
amyloid B deposits extracellular on neuronal processes and alters neurotransmission causing neuro toxicity
large deposits cause neuron death, inflammation, and altered communication between parts of the brain |
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|
Term
| what is the function of Tau protein |
|
Definition
| microtubule organizer in cells |
|
|
Term
| what causes neurofibrillary tangles |
|
Definition
| TAU protein is HYPERPHOSPHORLYATED and moves from AXONS to DENTRITES and CELL BODIES causing TANGLES with fibers in the cytoplasm |
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|
Term
| why do clinical signs begin before clinical presentation in alzheimers |
|
Definition
| Tau protein causes neurofibrillary tangles |
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|
Term
| cause of frontotemporal dementia |
|
Definition
| TAU protein aggregates in ROUND balls in cytoplasm of neurons in frontal and temporal lobes |
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|
Term
|
Definition
| changes in behavior and decreased language precede normal dementia signs |
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|
Term
| signs of lewy body dementia |
|
Definition
| parkinsons disease with EARLY DEMENTIA (within 1y) and HALLUCINATIONS |
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|
Term
| cause of lewy body dementia |
|
Definition
| lewy bodies in the CORTEX! rather than basal ganglia |
|
|
Term
| what are the 4 CNS movement disorders |
|
Definition
parkinsons disease
huntingtons disease
spinocerebellar degeneration
fredreich ataxia |
|
|
Term
| explain the normal regulation of movement: 3 nuclei, how to activate, how to deactivate |
|
Definition
overview: cortex tells basal ganglia what it wants, basal ganglia processes, tells the cortex what to do which tells the muscles
basal ganglia is composed of caudate and putamen (striatum)
caudate uses GABA to INHIBIT MOTION
substantia nigra pars compacta uses dopamine to INCREASE MOTION via D1 receptors to stimulate and D2 receptors to decrease inhibition |
|
|
Term
| cause of parkinsons disease 2 |
|
Definition
degeneration of SUBSTANTIA NIGRA PARS COMPACTA due to AGING
OR
AD/AR inheritance of A-SYNECULIN mutation |
|
|
Term
| signs of parkinsons disease 8 |
|
Definition
T-tremor: pill roll at rest
R-rigidity: cogwheel
A- akinesia/bradykinesia: slow voluntary movement, blank expression, immobility in 10-15y
P-postural instability (stoop) and shuffling gait (fenestrating)
D- dementia in YEARS due to lewy bodies progressing into CORTEX |
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|
Term
| how do people from parkinsons disease usually die |
|
Definition
|
|
Term
| how can you diagnose parkinsons |
|
Definition
|
|
Term
| what are two morphological changes in parkinsons disease |
|
Definition
lewy bodies
pallor of substantia nigra and LOCUS CERULEUS |
|
|
Term
| what is a lewy body made of 4, why is it made |
|
Definition
eosinophilic, a-syneculin, neofilament, ubiqutin
defective degeneration in proteasome |
|
|
Term
| locations of lewy bodies in parkinsons 4 |
|
Definition
| substantia nigra, locus ceruleus, basal nuclei of Meynert, brain stem nuclei |
|
|
Term
| why is there pallor in substantia nigra and locus ceruleus in parkinsons |
|
Definition
| loss of pigmented neurons and gliosis |
|
|
Term
| cause of huntingtons disease |
|
Definition
| AD inheritance of trinucleotide repeat expansion (CAG:POLYGLUTAMINE) for huntington gene on 4p.16.3 THROUGH OUT BODY |
|
|
Term
| what does polyglutamine normall do, what is wrong in huntingtons, how does it cause damage and changes to movement |
|
Definition
huntington gene protein, polyglutamate, binds mitochondria and activates apoptosis causing degeneration of CAUDATE nucleus
it normally inhibits signals via GABA, damage causes increased muscle activity |
|
|
Term
|
Definition
chorea: random purpousless movements of muscles in all parts of bod
athatosis: slow involuntary snake like movements of fingers
dementia: late stage
parkisons: late stage
affect disorders: depression, suicide |
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|
Term
| what changes can be seen in the brain in huntingtons |
|
Definition
| caudate degeneration allows for more space for ventricles (esp lateral and 3rd) to expand causing ex vacule hydrocephalus |
|
|
Term
| cause of spinocerebellar degeneration |
|
Definition
| degeneration of neurons without pathological change and mild gliosis due to GAG REPEATS |
|
|
Term
| locations of spinocerebellar degeneration 4 |
|
Definition
cerebellar cortex
spinal cord
brain
peripherial nerves |
|
|
Term
| cause of fredreich ataxia |
|
Definition
| AR inheritance of GAA TRINUCLEOTIDE REPEAT EXPANSIONS IN INTRON used for FE REGULATION causes low cellular protein levels |
|
|
Term
| location of degeneration in fredreich ataxia 2 |
|
Definition
| cerebellum and multiple spinal cord tracts |
|
|
Term
| signs of fredreich ataxia 12 |
|
Definition
gait ataxia
hand clumsiness
dysarthria
diminished or absent DTR EXCEPT PLANTAR
loss of joint position and vibration sense
pain, temp, light touch diminished
hypertrophic cardiomyopathy
DM
wheelchair bound in 5y |
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|
Term
| when does huntingtons disease usually start, why isnt this a good perdictor of start time |
|
Definition
usually 40-50yo
ANTICIPATORY: occurs earlier in each generation due to further expansion of nucleotide repeat in SPERMATOGENESIS |
|
|
Term
| after the onset of huntingtons how long away is death |
|
Definition
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