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31 Cards in this Set

  • Front
  • Back
disease associated with caudate nucleus and frontal cortex degeneration
Huntington's disease
disease associated with frontal and hippocampal cortex degeneration
Alzheimer's disease
degeneration of the substancia nigra
Parkinson's
degeneration of the posterior and lateral columns
friedreich ataxia
degeneration of the betz cells in the motor cortex and anterior horn cells
ALS
symptoms associated with parkinsonism
tremor
rigidity
akinesia/bradykinesia
postural instability - forward propulsion
decreased facial expression
histologic findings associated with idiopathic parkinson disease
pallor and atrophy of the substancia nigra and/or locus ceruleus
lewy bodies in the substancia nigra and/or locus ceruleus
associated with MPTP
acute parkinsonism syndrome
Von Economo's disease
post-encephalitis parkinsonism after influenza infection
associated with involuntary choeiform movements, dementia, and psychosis
Huntington's disease
pathology of hunginton's disesae
bilateral caudate atrophy
AD disease with trinucleotide CAG repeats resulting in what
aggregation of huntingtin protein and formation of intranuclear inclusions causing Huntington's disease
two diseases associated with trinucleotide repeats
Huntington's disease
Friedreich Ataxia
manifestations of ALS
LMN - muscle atrophy and spontaneous fasciculations
UMN - weakness and spasticity
manifestations of Friedreich Ataxia
cerebellar ataxia, spasticity, and sensorimotor peripheral neuropathy
pathogenesis of friedreich ataxia
trinucleotide repeat expansion (GAA) on chromosome 9
caused by fibrosis of meninges that pinches off the dorsal root
Tabes Dorsalis
degeneration of cortical neurons in the motor cortex and ventral motor nerve root
ALS
subacute combined degeneration
B12 deficiency
atrophy of the posterior and lateral column and anterior horn
progressive deterioration of higher cognitive function with impariment of previously attained function
dementia
cognitive impairment associated with a person who has never attained certain milestones
mental retardation
cognitive impairment is temporary and not progressive
delirium
most common cause of dementia
Alzheimers
pathology seen in Alzheimers
1. Senile plaques - extracellular A beta amyloid protein aggregation
2. Neurofibrillary tangles - intracellular inclusion with paired helical filament structure composed of tau protein
3. granulovacuolar degeneration
progressive degeneration of Alzheimers
initially affects the hippocampus and temporal lobe, than affects the neocortex, and eventually the deep gray matter and brain-stem
associated with hyperphosphorylated Tau protein
Neurofibrillary tangles
pathogenesis of alzheimers
missense mutation on amyloid protein gene on chromosome 21
all patients with down's syndrome are prone to
alzheimers dementia due to chromosome 21 trisomy
where can amyloid aggregates be found
brain parenchyma - senile plaques
around vessels - amyloid angiopathy
what is associated with the degree of dementia in Alzheimers
neurofibrillary tangle densities, not senile plaques
associated with atrophy of the frontal and temporal lobes, no neurofibrillary tangles nor senle plaques
pick's dementia