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25 Cards in this Set
- Front
- Back
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What brain region is affected by Alzheimer's Dementia?
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Cortical Regions
Affect: Frontal, Temporal, Parietal Spares: Occipital |
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What are the symptoms and course of Alzheimer's Dementia?
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* Progressive cognitive impairment
2) Loss of formation of new memories 3) Progresses to multiple domains |
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What are the pathologic hallmarks of Alzheimer's Dementia?
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1) Excessive Aβ peptide production and deposition of amyloid
2) Tau changes with neurofibrillary tangles (NFTs) |
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Are there genetic abnormalities associated with Alzheimer's Dementia?
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* Most AD is sporadic
* Auto. dominant mutations in Presinilin 1 & 2 |
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What are the symptoms and course of Parkinson's disease?
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1) Progressive motor impairment
2) Bradykinesia 3) Resting Tremor 4) Rigidity 5) Late-stage dementia |
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What are the neuronal systems affected in Parkinson's Disease?
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Parkinson's results in
1) Loss of DOPAMINERGIC neurons in the Substantia Nigra 2) Degeneration of the Nigrostriatal Pathway |
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What are the microscopic changes associated with Parkinson's?
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Substantia Nigra neurodegeneration with LEWY BODY formation.
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Is there a genetic component to Parkinson's?
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Most cases are sporadic. Rare mutations in alpha-synuclein.
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What's the pathology?!?
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Neurofibrillary Tangles Associated with Alzheimer's Disease
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What's the pathology?!?
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Lewy Body associated with Parkinson's Disease
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What are the two major neurodegenerative diseases affecting the Basal Gangia?
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1) Parkinson's
2) Huntington's |
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What are the symptoms and course of Huntington's Disease?
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SXS: Chorea (dance-like movements), dementia, and psychosis
Onset: variable, early-middle adulthood |
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What are the gross changes in Huntington's?
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1) Loss of the Caudate Nucleus
2) Secondary frontal lobe atrophy |
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Describe the genetics of Huntington's Chorea
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HC is an autosomal dominant disease, inherited with anticipation. Expansion of CAG trnucleotide repeats (>37) results in disease.
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What is the typical microscopic appearance of Huntington's pts?
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Gliosis
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What is the most common motor neuron disease?
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Amyotrophic Lateral Sclerosis
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How does ALS present clinically?
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ALS presents with both upper and lower motor neuron disability. There are several variants of the disease.
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What is major feature of ALS?
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Loss of descending upper motor neurons in the spinal cord
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What are the microscopic features of ALS?
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1) Loss of motor neurons and ASTROGLIOSIS in ANTERIOR HORN, motor cortex and brainstem
2) Bunina and Lewy-like Inclusions 3) Degeneration of the LATERAL CORTICOSPINAL TRACT |
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Is there a genetic correlation with ALS?
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* 10% caused by Cu/Zn Superoxide Dismutase Gene distruption
* 90% Sporadic |
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What are the three sources of Creutzfeld-Jakob Disease?
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1) Sporadic
2) Transmitted (iatrogenic) 3) Autosomal Dominant (mutation in Prion Precursor Protein) |
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What's the clinical course of Creutzfeld-Jakob Disease?
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Rapidly progressive dementia with movement disorders and myoclonus.
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Are there diagnostic tests for CJD?
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The diagnostic tests are ambiguous. Dx by pathology.
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What are the three pathologic features of CJD?
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1) Spongiform changes in neurons and processes
2) Astrogliosis 3) Amyloid Plaques of PrP fragments |
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What's the pathology?!?
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Spongiform Changes in CJD
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