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Path Degenerative CNS
KYCOM Block 13
52
Pathology
Graduate
01/27/2014

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Cards

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
Term
what are the 8 kinds of dementia, what is #1 and #2
Definition
infectious
vascular #2
metabolic
toxin
brain tumor
alzheimers
frontaltemporal (Pick's disease)
lewy body
Term
6 causes of infectious dementia
Definition
prions
HIV
leukoencephalopathy
viral encephalitis
neurosyphilis
chronic meningitis
Term
3 causes of vascular dementia
Definition
HTN
athlerosclerosis
vasculitis
Term
effect of vascular dementia, complication
Definition
decreased blood causes hypoxia and multiple infarctions which can progress to subdural hematoma
Term
4 causes of metabolic dementia
Definition
thiamine deficiency
B12 deficiency
niacin deficency (Pellagra)
endocrine disorders
Term
what are the two diseases thiamine deficiency causes
Definition
wernicke encephalopathy
korsakoff syndrome
Term
wernicke encephalopathy: 3 symptoms, 2 complications
Definition
confusion, abnormal eye movement, ataxia
hemorrhage and necrosis in mamillary bodies
Term
korsakoff syndrome: cause, symptoms, reason for symptoms
Definition
untreated wernickes, irreversible
lesion in medial dorsal nuclei of thalamus causes loss of memory
Term
4 causes of toxin dementia
Definition
murcury
lead
manganese
bromides
Term
what is the most common cause of dementia
Definition
alzheimers disease
Term
how is alzheimers disease diagnosed 3
Definition
exclusion of other dementia
imaging
pathology
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
Term
explain the normal progression of location of amyloid B deposition 3
Definition
enterohinal cortex > hippocampal formation and isocortex > neocortex
Term
what are the two types of alzheimer's disease
Definition
early
sporadic
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)
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
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
mute
immobile
disabled
Term
what do people with alzheimers usually die of
Definition
pneumonia or infection
Term
what are 4 morphology changes in dementia
Definition
diffuse cerebral atrophy
neurotic plaques
cerebral amyloid angiopathy
neurofibrillary tangles
Term
3 signs of diffuse cerebral atrophy
Definition
gyri narrow
sulci widen
ventricles dilate due to loss of mass (HYDROCEPHALUS EX VACUO)
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
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
Term
why do clinical signs begin before clinical presentation in alzheimers
Definition
Tau protein causes neurofibrillary tangles
Term
cause of frontotemporal dementia
Definition
TAU protein aggregates in ROUND balls in cytoplasm of neurons in frontal and temporal lobes
Term
signs of picks disease
Definition
changes in behavior and decreased language precede normal dementia signs
Term
signs of lewy body dementia
Definition
parkinsons disease with EARLY DEMENTIA (within 1y) and HALLUCINATIONS
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
Term
how do people from parkinsons disease usually die
Definition
trauma from falls
Term
how can you diagnose parkinsons
Definition
L-DOPA improves symptoms
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
5 signs of huntingtons
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
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
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
15y
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