Week 6 Flashcards
What are the 3 ways to divide progressive dementia?
- cortical dementia
- subcortical dementia
- mixed dementia
What is the most prevalent dementia in the population?
AD
Who is clinical evaluation of dementia usually done by?
Geriatrician, neurologist, medical practitioner. Clinical neuropsychologist opinions is essential.
___ diagnosis is important in dementia
Early
Information obtained to make a dementia diagnosis includes:
- present history
- past/social history
- cognitive assessment
- brain imaging
- other medical testing
What is one advantage of early diagnosis in dementing cognitions
- provide a diagnostic answer and education for the patient and family
- provide strategies
Instead of ‘dementia’, what is usually diagnosed according to the DSM 5?
neurocognitive disorder
What is the first diagnostic criteria in major neurocognitive disorder?
evidence of significant cognitive decline from a previous level of performance
What is the second diagnostic criteria in major neurocognitive disorder?
Impairment needs to interfere with independence in everyday activities
What is the third diagnostic criteria in major neurocognitive disorder?
Cognitive deficits do not occur exclusively in the context of a delirium
What is the fourth diagnostic criteria in major neurocognitive disorder?
Cognitive deficits are not better explained by another rental disorder (e.g., MDD or schizophrenia)
What is the next step in diagnosing major neurocognitive disorder?
- specify type of dementia
- specify with or without behavioural disturbance
- specify if it produces milk difficulties with instrumental activities of daily living
What is cortical dementia?
Characterised by preferential neuronal loss to cortical regions of the brain
Involvement of the subcortical regions in dementia is rare, or if it does occur it occurs in:
late stages of the disease
What are some risk factors for dementia in early life?
less education (7%)
What are some risk factors for dementia in midlife?
- hearing loss
- hypertension
- alcohol
- obesity
- TBI
What are some risk factors for dementia in later life?
- smoking
- depression
- social isolation
- diabetes
- physical inactivity
Because of associated risk factors with dementia throughout life, what percentage of dementia is potentially modifiable?
40%
What percentage accounts for the risk unknown in dementia onset?
60%
Who first described Alzheimers disease?
Alois Alzheimer
How do we refer to AD as today?
an irreversible cortical progressive dementia
What were the symptoms of the first ever AD patient?
- hiding things
- severe behaviour change
- suspicious of husband
There is no single identifiable cause for AD. However:
- incidence increases with age (strongest predictor)
2. twice as many women develop AD
Average duration of AD is what?
8-10 years
What is the mean age of sporadic AD?
mean age of onset of 80 years
Familial Alzheimer’s is rare, but early onset AD at:
More than 50 years of age
What are the two key features of AD?
- the disease target specific regions of the brain
2. the disease targets structures which then sustain massive cell loss
Cortical atrophy in AD patients are more evident in:
frontal, temporal and parietal lobes
Cortical ___ results in ____ in AD patients:
thinning
ventricular enlargement
What are two histological markers of AD in the brain:
- amyloid/senile plaques
2. neurofibrillary tangles (TAU protein)
Neurofribillary tangles (as associated with AD) appear throughout the brain, but are disproportionately concentrated in which areas?
temporal parietal areas and hippocampal complex
Where are senile plaques (as involved in AD) mainly located?
most likely to concentrate in frontal and temporal regions of the brain and in the hippocampal complex
What are some signs and symptoms with the onset of AD?
- failing recent memory
- depression and irritability
- occasionally a seizure
- one or more language problems
- confusion when sudden disruption to work or social life occurs
As AD progresses, what happens to various cognitive symptoms?
- apraxias become increasingly prominent
- agnosias become increasingly evident
- disfluency becomes more apparent in speech
- in end stage, neuropsychological functions can no longer be measured. Primitive reflexes reemerge.
The progression of AD resembles a ___ of development from ____. With __ being lost first and a progression to a ___ state.
reversal
infancy to childhood
higher level skills
reflexive
In combination with cognitive changes, there are a range of ___ and ___ changes in AD patients
behavioural
personality
What are some behavioural and personality changes associated with AD?
- clinging to caregiver
- poor self care
- depression
- wandering
- agitation
- suspiciousness and paranoia
Frontal lobe dementia typically affects which areas of the brain?
atrophy in the frontal and temporal lobes
The cerebellum and ___ are unaffected in frontal lobe demential patients.
brainstem
What are the two subtypes of frontotemporal dementia?
- behavioural variant frontotemporal dementia
2. primary progressive aphasia
What are the 2 different types of PPA?
- semantic dementia
- logopenic aphasia
- progressive non fluent aphasia
What are one of the first signs of frontal lobe dementia
silliness, socially disinhibited behaviour, poor judgemnet
What are the middle stages of frontal lobe dementia characterised by?
blunted affect, progressive apathy, significant cognitive function
What can we expect to see in the late stages of frontal lobe dementia?
mute and display motor rigidity. Typically ends in terminal vegetative state.
What is the typical disease duration of frontal lobe dementia?
2-17 years
Frontal lobe dementia affects what percentage of all cases of dementia?
12%
Huntingtons disease is a ____ dementia.
progressive subcortical
HD affects ___ people in 100, 000
5-10
Is there a known treatment or cure for HD?
no
What gene is HD linked to?
ITI5 on chromosome 4
Onset of HD symptoms usually when what age?
30-40
Survival is how many years following onset of symptoms?
10-20
HD is always what in terms of outcome?
fatal
Who first described HG?
George Huntington aged 22 years
HD is a hereditary condition, the only known autosomal dominant condition. What does this mean?
Affects 50% of children with one parent with the disorder
What is Huntingtons disease also called, Huntingtons Chorea?
symptoms of involuntary, spasmodic, often tortuous movements that ultimately become disabling.
On average, if one parent has the Huntingtons disease and one doesn’t:
2 out of 4 children will get the disease
If one has the HD illele:
they WILL develop HD
Regarding the neuropathology of HD, primary neuropathological change is bilateral deterioration of the ___
caudate nucleus - deterioration of multiple systems involving white matter structures and areas of the cerebellum
The caudate nucleus is a component of the ____. It is involved in:
basal ganglia
timing, ordering, sequencing of movement patters
Caudate sends projections to and from the ___ and ___ areas of the brain.
Limbic
prefrontal
By the end of HD, what percentage of frontal lobe shrinkage volume is there?
20-30%
HD may also affect other areas of the basal ganglia such as:
putamen, areas of the striatum and other limbic structures.
What are some aspects of the cognitive profile in HD?
- eye movements
- decreased attention span
- memory deficits
- deteriorating speech production
- impaired visuo-spatial orientation
- impaired behaviour regulation
- planning and organisation deficits
What is the second largest type of dementia?
multi-infarct (vascular) dementia
Multi-infarct dementia results from:
Multiple infarction of brain tissue, from repeated strokes or blockages to blood vessels.
There are multiple ways that multi infarct dementia differs from AD. What are some of these?
- onset is acute and rapid
- history of risk factors for stroke
- better memory sometimes than AD
- reduced verbal production
- cognitive deficits precede personality changes
- frontal compromise earlier
Vascular dementia is heterogenous. what does this mean?
The deficits depend on the location of damage
What is Creutzfeldt Jakob Disease?
- extremely rare
- rapid and progressive
- brain turns into a spongelike consistency, holes appear
- present in sheep, cows, humans
In the Fore people of New Guinea (cannibals), how is Creutzfeldt Jakob disease transmitted?
tradition of eating their dead. Females and children typically eat the brains, injecting infectious diseases
What is sporadic CJD?
most common, occurs despite no risk factors being apparent
What is variant CJD (mad cow disease)
infection via tainted meat containing neural tissue
What is familial CJD?
extremely rare, only found in a handful of families, results in fatal insomnia
What is iatrogenic CJD?
transmitted via neural transplants, or other medical procedures
Why is CJD hard to identify?
- doesn’t produce unusual symptoms
- slow virus
- camouflaged by cells
CJD targets the:
cerebellum as well as the cerebrum
Astrogliosis occurs as a ____ of the CJD not the ___
result
cause
Mertz identified CJD in sheep and found:
twisted sticklike fibres in cells using electron microscope. Unique to CJD
What are the hallmarks of CJD?
- uncoordinated movements
- druken stagger
- speech is slurred and inarticulate
- victim cannot swallow and may die of starvation
- visual function alters
Emotional and cognitive symptoms are seen in CJD are:
- mood disorders
- fatigue
- sleeping difficulties
- attention and concentration problems