Neurodegenerative Diseases Flashcards
How are neurodegenerative diseases typically/traditionally classified?
• According to clinical presentation
• Alzheimers
○ Early memory and visuospatial problems
• Frontotemporal dementia
○ Early behavioral, executive and language problems
• Parkinsons
○ Tremor, rigidity, bradykinesia
• Lewy
○ Early parkinsonian features, psychosis, fluctuating consciousness
• Progressive supranuclear palsy
○ Bradykinesia, rigidity, falls, abnormal vertical eye movements
• Amyotrophic lateral sclerosis
○ Weakness and atrophy, fasciculations, and the combination of both upper and lower motor neuron signs
• Huntingtons
○ Dementia, depression, chorea
• Creutzfeldt-jakob
○ Rapidly progressive dementia with myoclunus
What are the classic signs of CJD?
• Creutzfeldt-jakob
○ Rapidly progressive dementia with myoclunus
What are the classic signs of huntington’s?
• Huntingtons
○ Dementia, depression, chorea
What are the classic signs of ALS?
• Amyotrophic lateral sclerosis
○ Weakness and atrophy, fasciculations, and the combination of both upper and lower motor neuron signs
what are the classic signs of progressive supranuclear palsy?
• Progressive supranuclear palsy
○ Bradykinesia, rigidity, falls, abnormal vertical eye movements
what are the classic signs of lewy body disease?
• Lewy
○ Early parkinsonian features, psychosis, fluctuating consciousness
what are the classic signs of parkinsons?
• Parkinsons
○ Tremor, rigidity, bradykinesia
what are the classic signs of alzheimers?
• Alzheimers
○ Early memory and visuospatial problems
what are the classic signs of frontotemporal dementia?
• Frontotemporal dementia
○ Early behavioral, executive and language problems
Neurodegenerative diseases seem to be variations on a theme. What is the theme?
- Theme - spontaneous death of neuronal populations
- The location of the neurons determine the clinical presentation
- Remember that neuronal location is tied to neuronal function
What protein is implicated in frontotemporal dementia and progressive supranuclear palsy?
- Abnormal tau metabolism
* Neurofibrillary tangles?
Synuclein is a protein that when you see it you should think…?
- Parkinsons
* Lewy body diseases
Ubiquitin in the context of neurodegenerative disease makes you think…?
- ALS
* Amyotrophic lateral sclerosis
Disturbances in ach neuronal transmission makes you think:
- Alzheimers and lewy body
* Though there are other pathways disturbed in each of the neuorodegen diseases
Serotonergic dysfucntion makes you think:
- Frontotemporal dementia
* Though there are other pathways disturbed in each of the neuorodegen diseases
Disturbances in dopamine transmission makes you think:
- Parkinsons and lewy body
* Though there are other pathways disturbed in each of the neuorodegen diseases
What are the neuropathology findings of: Alzheimers
• Amyloid plaques and neurofibrillary tangles
• Dx requires the presence of both of these changes on pathologic examination
• Location is cortexa nd hippocampus with sparing of deeper structures
Gross - diffuse atrophy
What are the neuropathology findings of: FTD - frontotemporal dementia
- This is less of a single disease and more of a collection of pathology
- Some have neurofibrillary tangles
- Others have predominately ubiquitin inclusions
- Some have tau reactive intra-neuronal inclusion
- Some have CDP-43 deposition
- Many have no findings at autopsy - “dementia lacking distinct histological features”
What are the neuropathology findings of: Huntingtons
- Neurodegeneration has a predilection for the caudate nucleus
- Produces a characteristic pattern of atrophy that can be seen with neural imaging by CT or MRI
