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31
Neuronal diseases III
27
Pharmacology
Undergraduate 2
01/12/2017

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Cards

Term
What is ALS-PDC?
Definition
caused by beta-methylamino l-alanine
Term
What is Pick's disease?
Definition
a frontotemporal dementia
2-3% of all dementias
20-25% of all frontotemporal dementias
Term
What is the onset and duration of Pick's disease?
Definition
onset 56-58 years (but wide range)
duration 8-11 years
Term
Is Pick's disease genetic?
Definition
no known genetic factor
45% of patients have first degree relative with frontotemporal dementia
Term
What are the symptoms of Pick's disease?
Definition
- personality changes
- speech
- emotional changes
- can develop voracious appetite
Term
Describe Pick's pathology
Definition
- frontotemporal atrophy
- neuronal cell loss
- asrtogliosis (astrocytic scar tissue where neurones have been lost)
- swollen neurones contain 'Pick bodies'
Term
What are Pick bodies?
Definition
intracellular inclusion bodies made of cellular proteins
Term
What causes Picks Disease?
Definition
unknown
Term
What changes are seen/not seen in Picks disease? What does this mean?
Definition
- decreases in 5-HT receptors and in 5-HT levels
- ChAT unchanged
- CSF and brain DA unchanged
- CSF and brain somatostatin decreased

Therefore distinct and separate from Alzheimers, however over confused due to symptoms
Term
What is the most common form of dementia?
Definition
Alzheimers
Term
When does Alzheimers occur?
Definition
senile and presenile dementia (either side of 65 years)
there used to be a distinction however it is now redundant as they both show identical symptoms and pathologies
Term
Is Alzheimers genetic?
Definition
no, sporadic form predominated however familial forms do exist
Term
Describe the onset and progression of Alzheimers
Definition
onset usually >60 years
death 5-10 years from onset
Term
What is the cause of death in Alzheimers?
Definition
the individual becomes completely bedridden and could get an infection (bronchopneumonia), sepsis from bedsore and much more susceptible to haemorrhagic stroke. Additionally, inanition, as they often require someone to feed them.
Term
Describe the pathology of Alzheimer's disease
Definition
- amyloid plaques in hippocampus
- tangles
- neurone loss
Term
Describe the plaques you can see in Alzheimers
Definition
deposits of amyloid beta protein (mainly)
surrounded by dystrophic neurites (axonal or dendritic)
they show a reactive gliosis in the area, macrophages will attack (unsuccessfully) but will produce everything necessary to produce toxicity
Term
Describe the tangles you can see in Alzheimers
Definition
'flame-like' structures within the brain
neurones that have aggregations of hyperphosphorylated Tau-proteins that form tangles within the cells
Term
Describe the neurone loss in Alzheimers
Definition
distinct progression of mainly cholinergic pathways resulting in massive reductions in nAChR and ChAT levels
Term
How does the brain physically change in Alzheimers?
Definition
- cortical atrophy
- smaller brain due to shrinking grey matter
- narrower gyri and wider sulci
- wider ventricles
- degenerated hippocampus
Term
What are the earliest symptoms of Alzheimers?
Definition
- Short term memory
- Disorientation - time then place
- Aphasia, anomia acalculia
- Apraxia
- Visuospatial orientation (gets lost)
Term
What are the later symptoms of Alzheimers?
Definition
- Loss of social skills
- psychosis + paranoia/hallucinations/delusions
- bradykinesia/rigidity
Term
What are the latest symptoms of Alzheimers?
Definition
- Mutism
- Incontinence
- Bedridden
Term
How is Alzheimers assessed?
Definition
Mini Mental State Examination

Normal score - 30
mild AD: MMSE 21 to 26
moderate AD: MMSE 10 to 20
moderately severe AD: MMSE 10 to 14
severe AD: MMSE less than 10.
Term
What causes Alzheimers?
Definition
- Genetics
- Amyloid
- Trauma
Term
Describe the genetic causes of Alzheimers
Definition
- not as common
- trisomy 21 (Down's syndrome) because they have 3 copies of gene 21 and the protein that encodes amyloid beta protein resides on chromosome 21. Causes early Alzheimers and build up of plaque.
- mutations to amyloid precursor proteins (APP) and PS-1 and PS-2 (proteins that cleave APP)
- ApoE4 is a risk factor for AD. Its an allelic variant. I you have ApoE4 you are 50% more likely to develop Alzheimers (ApoE2 makes you less likely)
Term
Describe the amyloid hypothesis of Alzheimers
Definition
The amyloid beta protein that forms plaques is neurotoxic ad therefore build up of the protein kills neurones in the brain and that gives rise to the symptoms of Alzheimers.
Term
Describe the traumatic causes of Alzheimers
Definition
- risk with head injury
- risk with hypoxic/anoxic episodes
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