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47 Cards in this Set
- Front
- Back
which neurodegenerative diseases involve the cortex?
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alzheimer's diseease
Frontotemporal dementia (Picks) Lewy body dementia |
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which neurodegenerative diseases involve the basal ganglia and brainstem?
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Parkinson's disease
Huntington disease Multi system atrophy Progressive supranuclear palsy |
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which neurodegenerative disease involves the cerebellum and spinal cord?
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spinocerebellar degeneration
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which neurodegenerative diseases involve the upper and lower motor neurons?
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Amyotrophic Lateral Sclerosis
Spinal Muscular Atrophy |
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what is dementia pugilistica?
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a type of neurodegenerative disease or dementia caused by repeated concussions or sub-concussive blows or both
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what is the most common age group affected by alzheimer's disease?
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over 65
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what chromosomes are involved in Alzheimer's disease? what does each chromosome code for?
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chromosome 1 - presenilin 2 (PS2)
chromosome 14 - presenilin 1 (PS1) chromosome 19 - APOE4 chromosome 21 - amyloid precursor protein (APP) |
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what is the theory as to why patients with down syndrome develop early alzheimer's disease?
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down syndrome is trisomy 21. chr 21 codes for APP. too many chr 21 = too much APP = development of plaques
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where do you seen atrophy in the brain of patients with alzheimer's disease?
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mainly the temporal lobe (hippocampus and amygdala), but also frontal and parietal lobes
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how will atrophy appear grossly on a brain?
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widened sulci and narrowed gyri
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what are the 2 structures essential for diagnosis of alzheimer's disease?
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neurofibrillary tangles and neuritic plaques
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where the neurofibrillary tangles located? neuritic plaques?
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- tangle are intracellular
- plaques are extracellular |
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T or F: neurofibrillary tangles can occur in normal aging.
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TRUE
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what is cerebral amyloid angiopathy?
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accumulation of A beta peptide in small arteries, arterioles, and veins of the cortex and leptomeninges that cause the vessels to weaken and may cause non-traumatic intraparenchymal hemorrhage
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what is the major cause of lobar intraparenchymal hemorrhage?
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cerebral amyloid angiopathy
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how will beta amyloid protein appear on polarized congo red stain?
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apple green biferingence
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which enzyme cleaves APP in a normal brain?
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alpha secretase
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which enzyme(s) cleave APP in an alzheimer's patient's brain?
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beta secretase
gamma secretase |
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which allele of APOE is neuroprotective? which allele of APOE is associated with an increased risk of alzheimer's?
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neuroprotective - APOE2
alzheimer's - APOE4 |
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what are the two main neurochemical changes in alzheimer's disease?
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reduction of Acetylcholinesterase and choline acetyltransferase in the neocortex esp. temporal lobe
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what is the most identifiable finding in the brain of a down syndrome patient with alzheimer's disease?
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narrowing of the superior temporal gyrus
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what chromosome is linked to frontotemporal dementia with parkinsonism?
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chromosome 17
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why do behavioral problems manifest first in FTD and memory problems later?
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FTD affects the frontal lobe first and then hits the temporal lobe later
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a patient dies in your hospital and you are asked to perform a autopsy. when you remove the brain you notice it has a "knife's edge appearance" of the gyri. the attending tells you another name for the disease this person died from is "walnut brain". what is the disease?
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frontotemporal dementia or Picl's disease
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where exactly would you find a pick body?
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in the cytoplasm of neurons
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what is the pathologic change in parkinson's disease?
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destruction of the substantia nigra
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where is the substantia nigra located in the brain?
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midbrain
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what is the name of the inclusion body associated with parkinson's disease? where do you find it?
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- lewy body
- found in the cells of the substantia nigra that produce neuromelanin |
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a patient comes into your office and has parkinsonism and vertical gaze palsy. what is the most likely diagnosis?
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Progressive supranuclear palsy
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progressive supranuclear palsy affects what age group? which sex is more affected?
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40 - 60 y/o
males > females |
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a patient has cognitive decline, visual hallucinations, parkinsonisms, and a history of falls. what is the most likely diagnosis?
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dementia with lewy bodies
- look for the history of falls |
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what is the pathological difference between dementia with lewy bodies and parkinson's disease?
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DLB will have lewy bodies in the substantia nigra AND the cortex
Parkinson's will have lewy bodies only in the substantia nigra |
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what is the name of the stain that is needed to see cortical lewy bodies?
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alpha synuclein (so if you see this stain the disease must be dementia with lewy bodies)
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what chromosome is the huntingtin protein located on?
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chromosome 4
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Huntington's disease is caused by a chromosomal mutation that does what?
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causes trinucleotide repeats specifically CAGs
the larger the number of repeats the earlier the onset and more severe the disease |
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what is meant by anticipation?
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the next generation will have the disease earlier and more severely
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what is the pathology of huntington's disease?
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atrophy of the striatum (putamen and caudate)
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what is the key feature(s) on an MRI of Huntington's disease?
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- ventricular enlargement
- atrophy of the striatum |
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where in the CNS does ALS damage?
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anterior horn cell destruction and degradation of the corticospinal tract
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what chromosome is associated with ALS?
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chromosome 21
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how will ALS look under the microscope?
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destruction of neurons and replacement by astrocytes
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what age group does ALS affect most often? males or females?
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40 y/o
more common in males |
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how will a person with ALS present clinically?
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- muscle atrophy
- hyper-reflexia |
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a baby presents with "floppy baby" to the ED. you admit the baby and she gets progressively worse. within a few months the baby dies of respiratory failure. autopsy showed an autosomal recessive disease that destroyed only the anterior horn cells of the spinal cord. what is the most likely diagnosis?
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Spinal Muscular Atrophy (Werdnig-Hoffman disease)
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what is the most common prion disease in humans?
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Creutzfeldt-Jacob disease
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what is the "defect" in a prion and how does it affect the host?
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normal prion proteins are folded in the alpha helical form but abnormal prion proteins are folded in a beta pleated sheet form.
they cause other prion proteins to convert from the alpha helical form to the beta pleated sheet form |
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how will CJD clinically manifest?
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-rapidly progressing dementia
- myoclonus - characteristic spiking EEG - 14-3-3 CSF protein |