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47 Cards in this Set

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  • Back
which neurodegenerative diseases involve the cortex?
alzheimer's diseease
Frontotemporal dementia (Picks)
Lewy body dementia
which neurodegenerative diseases involve the basal ganglia and brainstem?
Parkinson's disease
Huntington disease
Multi system atrophy
Progressive supranuclear palsy
which neurodegenerative disease involves the cerebellum and spinal cord?
spinocerebellar degeneration
which neurodegenerative diseases involve the upper and lower motor neurons?
Amyotrophic Lateral Sclerosis
Spinal Muscular Atrophy
what is dementia pugilistica?
a type of neurodegenerative disease or dementia caused by repeated concussions or sub-concussive blows or both
what is the most common age group affected by alzheimer's disease?
over 65
what chromosomes are involved in Alzheimer's disease? what does each chromosome code for?
chromosome 1 - presenilin 2 (PS2)
chromosome 14 - presenilin 1 (PS1)
chromosome 19 - APOE4
chromosome 21 - amyloid precursor protein (APP)
what is the theory as to why patients with down syndrome develop early alzheimer's disease?
down syndrome is trisomy 21. chr 21 codes for APP. too many chr 21 = too much APP = development of plaques
where do you seen atrophy in the brain of patients with alzheimer's disease?
mainly the temporal lobe (hippocampus and amygdala), but also frontal and parietal lobes
how will atrophy appear grossly on a brain?
widened sulci and narrowed gyri
what are the 2 structures essential for diagnosis of alzheimer's disease?
neurofibrillary tangles and neuritic plaques
where the neurofibrillary tangles located? neuritic plaques?
- tangle are intracellular
- plaques are extracellular
T or F: neurofibrillary tangles can occur in normal aging.
TRUE
what is cerebral amyloid angiopathy?
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
what is the major cause of lobar intraparenchymal hemorrhage?
cerebral amyloid angiopathy
how will beta amyloid protein appear on polarized congo red stain?
apple green biferingence
which enzyme cleaves APP in a normal brain?
alpha secretase
which enzyme(s) cleave APP in an alzheimer's patient's brain?
beta secretase
gamma secretase
which allele of APOE is neuroprotective? which allele of APOE is associated with an increased risk of alzheimer's?
neuroprotective - APOE2

alzheimer's - APOE4
what are the two main neurochemical changes in alzheimer's disease?
reduction of Acetylcholinesterase and choline acetyltransferase in the neocortex esp. temporal lobe
what is the most identifiable finding in the brain of a down syndrome patient with alzheimer's disease?
narrowing of the superior temporal gyrus
what chromosome is linked to frontotemporal dementia with parkinsonism?
chromosome 17
why do behavioral problems manifest first in FTD and memory problems later?
FTD affects the frontal lobe first and then hits the temporal lobe later
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?
frontotemporal dementia or Picl's disease
where exactly would you find a pick body?
in the cytoplasm of neurons
what is the pathologic change in parkinson's disease?
destruction of the substantia nigra
where is the substantia nigra located in the brain?
midbrain
what is the name of the inclusion body associated with parkinson's disease? where do you find it?
- lewy body

- found in the cells of the substantia nigra that produce neuromelanin
a patient comes into your office and has parkinsonism and vertical gaze palsy. what is the most likely diagnosis?
Progressive supranuclear palsy
progressive supranuclear palsy affects what age group? which sex is more affected?
40 - 60 y/o

males > females
a patient has cognitive decline, visual hallucinations, parkinsonisms, and a history of falls. what is the most likely diagnosis?
dementia with lewy bodies

- look for the history of falls
what is the pathological difference between dementia with lewy bodies and parkinson's disease?
DLB will have lewy bodies in the substantia nigra AND the cortex

Parkinson's will have lewy bodies only in the substantia nigra
what is the name of the stain that is needed to see cortical lewy bodies?
alpha synuclein (so if you see this stain the disease must be dementia with lewy bodies)
what chromosome is the huntingtin protein located on?
chromosome 4
Huntington's disease is caused by a chromosomal mutation that does what?
causes trinucleotide repeats specifically CAGs

the larger the number of repeats the earlier the onset and more severe the disease
what is meant by anticipation?
the next generation will have the disease earlier and more severely
what is the pathology of huntington's disease?
atrophy of the striatum (putamen and caudate)
what is the key feature(s) on an MRI of Huntington's disease?
- ventricular enlargement
- atrophy of the striatum
where in the CNS does ALS damage?
anterior horn cell destruction and degradation of the corticospinal tract
what chromosome is associated with ALS?
chromosome 21
how will ALS look under the microscope?
destruction of neurons and replacement by astrocytes
what age group does ALS affect most often? males or females?
40 y/o

more common in males
how will a person with ALS present clinically?
- muscle atrophy
- hyper-reflexia
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?
Spinal Muscular Atrophy (Werdnig-Hoffman disease)
what is the most common prion disease in humans?
Creutzfeldt-Jacob disease
what is the "defect" in a prion and how does it affect the host?
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
how will CJD clinically manifest?
-rapidly progressing dementia
- myoclonus
- characteristic spiking EEG
- 14-3-3 CSF protein