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intellectual impairment, mood and behavior changes, memory loss. most common neurodegenerative cause of dementia can use radiographic tools to see brain atrophy.
gross and microscope structures, alzheimers
atrophy of frontal, temporal, parietal, hydrocephalous ex vacuo. will also see neurofibrilary tangles*. neurotic plaques *& amyloid angiopathy*
who always gets alzheimers
downs syndrome pts. trisomy 21. get after 45
neurofibrillary tangles
in alzheimers. bundles of filaments, encircling the nucleus \'flame cells\' *assoc with tau protein*
neuritic plaques
spherical collections of dilated neuritic processes. alpha.beta. peptide : primary component of neuritic plaques regular ppl have neuritic plaques but alzheimers have more than normal
amyloid angiopathy
always present in alzheimers. deposition of ab peptides in cerebral vessel walls
other alzheimers assoc physical characteristics
granulovascular degeneration, neuronal vacuoles containing granules. hirano bodies, ovoid extracellular bodies with parscrystalline arrays of beaded filaments
amyloid precursor protein. sometimes abnormally broken down to ab peptides. maybe a mutation in gene (gene 21), maybe causes downs and alzheimers
mutation causes inc in ab peptide, causes early onset familial alzheimers
apolipoprotein E
mutation inc risk of alzheimers and is responsible for decreasing age of onset for alzheimers
pick disease
progressive dementia. knife edge frontal and temporal lobe atrophy. they are very angry
pill rolling tremor. stooping posture. damage to nigro-striatal pathway (can be caused by mptp, DA antagonist), multiple system atrophy
parkinsons associations
substantia nigra and locus ceruleus loss. there are lewy bodies. tx with l-dopa with carbadopa.
progressive supranuclear palsy
movement disorder with dementia (some ppl think they are chronic alcoholics) verticle gaze palsy.speech probs. midbrain neuron loss and globose neurofibrillary tangles.
multiple system atrophy
neurodegenerative disorders affecting multiple neuron pathways. actually in oligodendrocytes. combo of shy-drager syndrome, striatonigral degeneration, and olivopontocerebellar atrophy.
striatonigral degeneration
SN neuron loss, caudate and putamen loss too. No lewy bodies. **doesn\'t respond to L-dopa
shy drager syndrome
combines parkinson & striatonigral degeneration. destroy of intermediolateral column of spinal cord. causes impotence, orthostatic hypotension. sweat & salivary probs
olivopontocerebellar atrophy
inf olive, pons, and purkinje cells of cerebellum results in ataxia mostly autosomal dominant
huntington chorea
hyperkinetic mvmts. loss of gaba neurons (basal ganglia, subthalamic nn). HD gene codes for it. huntingtin protein. CAG repeats. caudate , putamen GP, frontal cx, and parietal cx atrophy.
fredrich ataxia
AR. begins in childhood. clumsiness, dysarthria. loss of proprioception, temp, pain. GAA repeat for protein frataxin dorsal root ganglion atrophy. enlarged heart. loss of dentate nn of cerebellum.
ataxia telangiectasia
AR. ppl die early. eye mvmt disorders. ataxia. dysarthria. capillary growth in periphery. ppl with this don\'t have dna repair mech so get lots of cancer :(
ataxia telangiectasia gene probs
abnormal ATM gene. no dna repair. inc risk of cancer
ataxia telangiectasia physical loss?
loss of purkinje, granular cells. degeneration of DC, spinocerebellar tracts & anterior horn cells, peripheral neuropathy. lymph nodes, thymus & gonads & anterior horn
neurodegenerative disease
progressive loss of neurons. if primarily cerebral cx (dementia). if primarily subcortical- mvmt disorders. loss of paychosocial function
upper and lower motor neuron disease. probs with SOD1 gene (makes superoxide dismutase). cn nn degrades
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