1: Cognitive neurology Flashcards
What is cognition?
Mental action of acquiring and understanding knowledge
Using thought, experience and senses
What is the general definition of dementia?
Significant cognitive decline
interfering with daily activities
not explained by other processes
Dementia tends to be a (sudden / progressive) disease.
progressive decline in cognition
Which percentage of over 65s have been diagnosed with dementia?
Around 7%
What is one of the biggest risk factors for developing dementia?
Increasing age
Which infections can cause dementia?
HIV
Syphilis
Prion disease (i.e contact with the misfolded protein)
What acute neurological diseases cause a decline in cognition?
Viral encephalitis
Head injury
Stroke
What are the symptoms of transient global amnesia?
Amtegrade amnesia (difficulty laying down new memories)
Retrograde amnesia (loss of short term memories)
but preserved knowledge of self
Transient global amnesia typically lasts how long?
4 - 6 hours
Never more than 24 hours
What can trigger transient global amnesia?
Emotions
Changes in temperature
Which type of amnesia is short-lived compared to transient global amnesia but tends to be a recurrent problem?
Transient epileptic amnesia
Which brain structure is found in the temporal lobe and has a role in learning and memory?
Hippocampus
What is the clinical name for ‘everyday forgetfulness’ which impacts daily living?
Functional cognitive impairment
What is the course of functional cognitive impairment (‘everyday forgetfulness’)?
Fluctuating
What are the courses of
a) functional cognitive deficit
b) dementia?
a) Fluctuating
b) Progressive decline
What disorders need to be excluded in forgetful patients?
Dementia
Mood disorders
What is a prion disease?
Disease caused by misfolded proteins
What is a well-known prion disease causing rapid brain degeneration?
Creutzfeldt-Jakob disease (CJD)
In which age of patient does CJD tend to present?
60s
What are the clinical features of sporadic CJD?
Rapid onset dementia
Myoclonus
What is the average life expectancy of a patient diagnosed with CJD?
4 months
What is the cause of CJD?
Misfolded protein
What investigations are used to diagnose CJD?
EEG
MRI brain
Lumbar puncture (CSF markers)
What is a common disease causing gradual-onset dementia?
Alzheimer’s disease
Alzheimer’s disease is a ____ condition causing dementia.
neurodegenerative
What are the clinical features of Alzheimer’s disease?
Forgetfulness
Apraxia
Visual problems
At what age do patients tend to develop Alzheimer’s disease?
> 65
Younger patients who are diagnosed with Alzheimer’s disease tend to have ___ symptoms.
atypical
visual disturbances. aphasia
What are the atypical symptoms of Alzheimer’s disease?
Visual disturbances (feeling like you’re going to step into a pit)
Aphasia (difficulty communicating, naming things)
How is Alzheimer’s disease investigated?
MRI brain - atrophy of temporal lobes
Lumbar puncture - amyloid in CSF sample (not specific)
What neurotransmitter is inhibited in Alzheimer’s disease?
ACh
Which two classes of drugs can give symptomatic relief in Alzheimer’s disease?
Anticholinesterase inhibitors (e.g donepezil, rivastigmine) - ACh thought to be involved
NMDA antagonists - remember that NMDA is involved in excitatory neurotransmission
What is a disease causing early-onset (i.e < 65) dementia with behavioural symptoms?
Frontotemporal dementia
What is another name for frontotemporal dementia?
Pick’s disease
What are clinical features of frontotemporal dementia?
Loss of inhibition
Apathy
Lack of empathy
Obsessive, compulsive behaviour
Which type of history can be crucial in diagnosing patients with dementia?
Collateral history
Which lobes are atrophied on the MRI of someone with frontotemporal dementia?
Frontal / temporal lobes
Patients with frontotemporal dementia should have their access to food, money and the internet closely monitored - why?
Loss of inhibitions
Which type of dementia is linked to the same processes as stroke?
Vascular dementia
What causes vascular dementia?
Atherosclerosis > Infarct of areas responsible for memory, processing etc.
Haemorrhage > Infarct “” “” “”
Which bodies, seen in Parkinson’s disease, can also cause a late-onset dementia?
Lewy bodies
Which protein is implicated to cause Lewy body dementia?
Alpha synuclein
What are the clinical features of Lewy body dementia?
Declining cognition
Visual hallucination
Extra-pyramidal ‘Parkinsonian’ symptoms - tremor, hypokinesia, rigidity
Which scan is used to measure dopamine uptake from the basal ganglia and can support a diagnosis of Parkinson’s disease or Lewy body dementia?
DaT scan
What is the name for a dopamine precursor which can be given to reduce symptoms in PD and Lewy body dementia?
Levodopa
Which two diseases, involving Lewy bodies and alpha synuclein, can occur together?
Parkinson’s disease
Lewy body dementia
Which disease, involving CAG triplet repeats, can cause early-onset dementia in patients aged 30 - 60?
Huntington’s disease
Which gene is affected in Huntington’s disease?
Huntingtin
What are the symptoms of Huntington’s disease w/ dementia?
Chorea (rhythmic muscle contractions)
Decline in cognition
How is Huntington’s disease treated?
Symptomatically
Who tends to manage dementia in
a) under 65s
b) over 65s?
a) Neurology
b) Psychiatry
Who tends to see patients with rapid-onset dementia or dementia with unusual symptoms?
Neurology
How is cognition assessed?
ACE-III screen
Which types of dementia are associated with
a) Lewy bodies
b) Alpha synuclein
c) CAG repeats
d) prions
e) tau proteins?
a) Dementia with Lewy bodies (and Parkinson’s disease)
b) “ “
c) Huntington’s disease
d) Creutzfeldt-Jacob disease
e) Alzheimer’s disease