case 22 Flashcards
what are 3 lewy body diseases?
- parkinson’s
- parkinson’s dementia
- dementia with lewy bodies
what is the dementia definition according to the WHO?
a syndrome in which there isdeterioration in memory, thinking, behaviour and the ability to perform everyday activities.
what is the diagnostic criteria for dementia written by NICE?
patient must have impairment in at leasttwo cognitive domains
(memory, language, behaviour, and visuospatial or executive function) leading tosignificant functional decline (enough to affect ADLs) thatcannot be explained by another disorder or adverse effects of medication.
what are the 4 most common types of dementia in order?
1) Alzheimer’s (>50%)
2) vascular
3) mixed
4) DLB
what is the prognosis of DLB?
average survival rate=5-8 years from onset
what are the 2 most common dementias in people under 65?
1) early onset alzheimer’s
2) fronto-temporal demenia
what is CJD caused by?
prions
what is confabulation?
the production of fabricated or misenterpreted memories. can range from subtle altercations to bizarre fabrications.
what happens in korsakoff’s dementia and what causes it?
severe memory disturbance with confabulation to compensate.
caused by severe B1 deficiency (thiamine) classically associated w alcohol dependency but also malabsorption and malnutrition.
also can be b12 deficiency
what are the features of the initial dementia/confusion screen?
history
cognitive assessment
bloods
imaging
what imaging do they use in the initial cognitive assessment?
1st line: CT/NRI
2nd line: SPECT/PET if diagnostic uncertainty.
what happens in atypical alzheimer’s?
memory problems aren’t the first symptom.
how can medial temporal lobe atrophy be assessed?
on imaging using the MTA score
in DLB what does the dementia mainly affect?
attention and executive function (planning/organising)
how does bradykinesia typically present?
hypomimia (reduction of facial expression)
monotonous/slurred speech
reduced blink rate
generalised motor slowness
what are some of the potential surgical treatments for parkinson’s?
ablative surgery (removing/reprogramming brain tissue) deep brain stimulation striatal grafts infusion of neurotropic factors gene therapy
what does neurotropic mean?
having an affinity for nerve cells
which circuit is abnormal in parkinson’s?
motor basal ganglia circuit
in deep brain stimulation for parkinson’s, which structures do you apply stimulation to?
subthalamic nucleus, globus pallidus interna, pedunculopontine nucleus (PPN)
how is huntington’s inherited?
autosomal dominant
what is the genetic cause of huntington’s?
short arm of chromosome 4
expanded CAG repeat sequence
what are some examples of misfolded proteins that cause neurodegenerative diseases?
hyperphosporylated tau (HPt)
amyloid beta (Ab)
alpha-synuclein (a-syn)
huntington protein
how many items can we store in working memory?
5-9, as long as we keep repeating them
what is the sulcus terminalis?
v shaped groove separating the oral and pharyngeal parts of the tongue
