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Neurodegeneration
Dr. Ziegler Neurological Disease - Neurodegenerative 1 & 2
| Term | Definition |
|---|---|
| neurodegeneration | diffuse and progressive neuronal cell death via necrosis or apoptosis |
| anterograde or retrograde neurodegeneration | cell bodies degenerate > dendrites, axons follow (anterograde) axon terminals degenerate > cell bodies follow (retrograde) |
| defect in degradative pathways | proteosomal, lysosomal, auto-phagy leads to intracellular inclusions (Tay-Sach's, Parkinson's, Lewy body dementia) |
| glutamate excitotoxicity | glutamate receptors act as sodium and calcium channels that are excitatory (depolarizing) -- too much NMDA receptor mediated calcium influx can lead to activation of necrosis or apoptosis |
| necrosis | mitochondrial impairment, no caspase activation, cytolysis: toxic region |
| apoptosis | cell shrinkage, no cytolysis |
| Parkinson's disease | neurodegeneration of substantial nigra, dopamine neurons, and nigrostriatal pathway 1. cortical input to striatum is intact 2. gradual loss of dopamine (enhances indirect pathway, impairs direct) 3. increased GPi, decreased thalamus & cortex |
| s/s Parkinson's | resting tremor: seen only when not in motion hypokinesia/bradykinesia: festinating (difficulty initiating walking) impaired facial expressions: masked facies postural instability: risk of falling cogwheel rigidity: gear-like jerky movements |
| lewy bodies | inclusions of alpha-synuclein protein |
| Huntington's disease "HD" | neurodegeneration of striatum > selective loss of D2 receptor expressing GABA indirect, involuntary hyperkinesia and chorea dance + impaired voluntary movement *inherited!! >> trinucleotide CAG repeat disorder (polyglutamine repeats) |
| pathogenesis of HD | loss of function and gain of function ; length of repeated in genes increases with each generation in family and is associated with earlier onset |
| rigid/hypokinesisa subtype HD | general loss of striatal GABA affecting both direct and indirect pathways *increased GPi, decrease thalamus and cortex (resembles Parkinson's) |
| Wilson's disease | genetic, progressive disease of liver and basal nuclei > autosomal recessive mutation *Kayser-Fleishcer rings!!, wing beating tremor, dyskinesia |
| cerebral palsy | weakness and impaired motor coordination, acquired or congenital, diffuse |
| amyotrophic lateral sclerosis (ALS) | corticospinal and corticonuclear affects: motorneurons ONLY *age-related disorder, sporadic NOT genetic, diffuse, insidious and progressive |
| spinal muscular atrophy (SMA) | motoneuron disease that affects only corticospinal system!! > LMNs only! *pediatric disorder, genetic AR, diffuse, insidious and stable |
| SM1 - survival motorneuron 1 | SMA is AR that has the effect of deletion of which gene? |
| Werndig-Hoffman | SMA type 1, onset in infancy (less than 6 mo) - lethal |
| SMA type 2 | onset in 1-2 years (limited life span) |
| Kugelberg-Welander | SMA type 3, onset in adolescence (reduced lifespan) |
| SMA type 4 | adult onset, RARE |
| Creutzfeldt-Jakob disease | aka prion disease; mutant form of protein undergoes change that makes it neurotoxic and acts as an enzyme to convert more normal protein to the prion (resistant to proteases) |
| transmission of Creutzfeldt-Jakob | neurosurgery tools, ingestion of contaminated food |
| fronto-temporal dementia | aka Pick's; dying neurons with spherical intracellular inclusions cognitive, non-fluent aphasia, behavioral deficits *insidious and progressive |
| posterior cortical atrophy | syndrome of the visual system *adult onset, atypical distribution of another neurodeg. disease like Alzhiemer's or CJD - visual field defect, diffuse in occipital and/or temporal + parietal lobes - insidious and progressive |
| characterizations of Alzheimer's | - cognitive decline (memory loss of recent > memory loss of older > impaired orientation) - post-mortem histopathology: amyloid plaques & neurofibrillary tangles |
| distribution of Alzheimer's | initial: hippocampus, entorhinal > memory for recent spread: temporal > older memories progression: dementia in cerebrum/brain > impaired orientation |
| neurotoxic amyloid protein (Abeta) in Alzheimer's | APP is degraded into Abeta which aggregates into amyloid plaques and can disrupt function of other proteins (such as sodium-potassium pump) |
| neurofibrillary tangles in Alzheimer's | tau proteins become hyperphosphorylated and destabilize the microtubules > cell death of cytoskeleton! - debris from destroyed dendrites/axons form extracellular neurofibrillary tangles |
| presinilins & ApoE | presinilins: protein subunits of secretase in Alzheimer's ApoE: not specific to Alzheimer's but can have interactions presnilins & Abeta |
| AChEI and Memantine | Alzheimer's treatment memantine: NMDA glutamate receptor antagonist |
Created by:
kycokate
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