Dementia Flashcards
What is dementia?
a progressive and largely irreversible syndrome that is characterised by a widespread impairment of mental function
Disease o the medial temporal lobe, hippocampus, amygdala and limbic system lead to what?
disorders in memory and hallucinations
What is disease of the temporal neocortex associated with?
receptive dysphasia and automatisms
Disease of the parietal lobe leads to what?
impairment of visuospatial skills and integration of sensory inputs, leading to sensory agnosias and apraxias
What does disease of the frontal lobes lead to?
abnormalities in several behavioural domains - impairment of judgement, abstract reasoning, strategic planning, emotional restraint and control of appetite
What does disease of the occipital lobe lead to?
failure of visual sensory systems
Name 4 types of neurodegenerative dementias
- Alzheimer’s
- Lewy Body
- Frontotemporal
- Huntington’s disease
Which type of neurodegenerative dementia is Parkinson’s disease associated with?
Lewy body dementia
Can treatment cure vascular dementia?
no, but treatment can slow down progression
How quickly does prion disease progress?
rapid progression (untreatable) - patients often die within 2-3 months of presentation
What are the classic presentations of cortical dementia?
higher cortical abnormalities - dysphasia, agnosia, apraxia
Is Alzheimer’s cortical or sub-cortical?
cortical
How would subcortical dementia classically present?
apathetic, forgetful and slow, associated with other neurological signs and movement disorders
Which neurodegenerative diseases are associated with synucleinopathies?
- Parkinson’s disease
- Dementia with Lewy Bodies
- MSA
Which neurodegenerative diseases are associated with ubiqionopathies?
- MND and MND/dementia
- semantic dementia
What is the triad of symptoms presenting in normal pressure hydrocephalus?
- dementia
- gait disturbance
- urinary incontinence
How does transmissible spongiform encephalopathies present under a microscope?
tissue looks like a sponge
What would be noticeable in an MRI of someone with sporadic CJD?
non-specific changes in basal ganglia
What is the characteristic EEG in a patient with CJD?
triphasic waves on repeat testing
What is a ‘pulvinar sign’?
characteristic abnormality seen in the posterior thalamic region
What disease is a pulvinar sign highly sensitive and specific for?
variant CJD
What would you use to treat autoimmune encephalitis?
steroids
Define cognition
sum of brain function which allows us to integrate in the environment
What are amyloid proteins?
insoluble fibrous proteins aggregates sharing specific structural traits
What are tau proteins?
a group of proteins that stabilise microtubules in neurons
How many isoforms of tau proteins are there?>
6
What is ubiquitin?
a small regulatory protein that has been found in almost all cells of eukaryotic organisms
What is the function of ubiquitin?
directs proteins to compartments in the cells and can attach to proteins and label them for destruction
What is the function of the limbic system?
arousal, emotion, motivation, attention and memory
Describe a simple microcircuit in the brain
neuron > synapse > neurotransmitter > action potential
What are the two functional classifications of neurodegenerative disorders?
cognitive (AD) and movement (PD)
which protein can produce Lewy bodies when mutated?
alpha-synuclein
What is oxidative stress?
imbalance in cell due to excess oxygen free radicals (causes neuronal cell death)
what is a normal brain weight? What would you expect in someone with AD?
1200-1400 g
AD: 900-1100g
In which areas would you expect to see most atrophy in the brain in AD?
- hippocampus
- temporal lobe
- parietal lobe
- frontal lobe
- cingulate gyrus
what does atrophy in deep white matter cause?
ventricular dilation
What skills will someone lose in significant hippocampal atrophy?
inability to learn new things and to retain new facts
Do tangles or plaques show correlation to clinical severity?
tangles
How many Braak and Braak stages are there?
6
Where would the most significant atrophy be seen in dementia with Lewy body?
amygdala, cingulate gyrus, temporal, parietal and frontal lobes
What is the most striking feature seen in a brain with Lewy bodies?
pale substantia nigra
What is the pathology of Binswanger’s disease?
blood vessels cause microinfarction of the white matter
What is Pick’s disease?
frontotemporal dementia
In Pick’s disease, what would you expect to see under the microscope?
ballooned neurones due to them being tau +ve and ubiquitin +ve
