Dementia Flashcards
Degeneration in the cerebral cortex leads to what?
Alzheimers
Picks disease
Creutzfeldt jakob disease
Degeneration in the Basal Ganglia and brain stem leads to what?
Parkinsons
Multi System atrophy
Huntingtons
Degeneration in the spinocerebellar region leads to what?
Spinocerebellar ataxia
Degeneration in the motor neurones leads to what?
Motor Neurone Disease
What is dementia?
Pathological acquired and persistent generalised disturbance in higher mental function.
List some primary causes of dementia.
Alzheimers
Huntingtons
Picks disease
List some secondary causes of dementia.
Multi infarct dementia Infection Trauma Drugs Toxins Vitamin deficiencies
Epidemiology of Alzheimer’s
Most common dementia
F:M 2:1
What are some genetic risk causes of Alzhiemer’s?
Amyloid Precursor Protein
Presenilin 1 and 2
In what condition is there an increased risk of Alzhiemer’s?
Trisomy 21 - Down syndrome
Presence of Amyloid Precursor Protein
What is the cause of death in most Alzheimer’s cases?
Secondary cause due to insidious impairment of higher function
What does the brain of someone with Alzheimer’s look like?
Decreased size and weight Cortical Atrophy - Frontotemporal and Parietal Widened Sulci Narrowed gyro Compensatory ventricle dilation
Compensated ventricular dilation is a cause of what in Alzheimers?
Secondary Hydrocephalus
On a microscopic level what does the brain tissue of someone with Alzheimer’s show?
Simple neuronal atrophy
Gliosis
Neurofibrillary tangles
Neuritic plaques
What are neurofibrillary tangles?
Microtubules within the cytoplasm contain Tau proteins
What make neuritic plaques?
Aβ amyloid plaques
What produces Aβ amyloid?
Cleavage of the Amyloid precursor protein
Describe the clinical presentation and history of someone with levy body dementia.
Fluctuating levels of attention/cognition
Hallucinations
Late onset memory loss
Motor features of Parkinsonism
Conditions affecting where in the brain will result in Parkinsonism?
Nigro-striatal dopaminergic pathways
What are lewy bodies?
Eosinophilic dense body surrounded by a halo of radiating fibrils
What causes lewy body dementia?
Degeneration of substantia nigra
Dopaminergic pathways are broken down
The brain of someone with lewy body dementia will show.
Pallor where pigmented substantia nigra was located.
Reactive gliosis
What is hunitingtons disease?
Inherited Autosomal dominant disease leading to rapidly progressive motor and cognitive disturbances.
What are the main symptoms of Huntington’s?
Chorea Myoclonus Clumsiness Slurred speech Depression Irritability Apathy Dementia later on
How many CAG repeats are present in normal population?
<28
How many CAG repeats are present when the disease is expressed?
> 35
What regions of the brain undergo atrophy? HD
Basal Ganglia
Caudate nucleus
Putamen
Later fronto-parietal
On a microscopic level how are the tissues of the brain affected by Huntingtons?
Neuronal atrophy of striatal neurones within Basal ganglia
Astrocytic gliosis
Loss of striatal neurones within basal ganglia results in what?
Loss of motor inhibition
Frontotemporal dementia is also known as….
Picks disease
What is Picks disease?
Progressive dementia with onset in middle life around 50-60 years
What are the main symptoms of Picks disease?
Personality and behavioural changes
Speech and communication issues
Eating habits change
Reduced attention span
What is the mean time from onset of symptoms to death in Picks disease?
7 years
What does the brain of someone with Picks disease look like?
Extreme atrophy of cerebral cortex
Neuronal loss and gliosis
Picks cells
What are pick cells?
Swollen neurones
Intracytoplasmic filaments inclusion (Picks bodies)
Describe someone who classical presents with Multi Infarct Dementia.
> 60 male with history of hypertension
What is the pathology of Multi Infarct Dementia?
Succesive cerebral infarction lead to a growing area of cell death and damage leading to cognitive dysfunction.
At what volume of brain destruction does Multi Infarct Dementia present?
50-100mls of brain tissue
Why are people suffering from of Multi Infarct dementia more prone to suffer depression and anxiety?
Due to stepwise progression of the disease they are far more aware of their own cognitive deficits.
How can you differentiate Multi Infarct Dementia from Alzheimer’s?
Abrupt onset with a stepwise progression
History of hypertension of strokes
Evidence of a stroke on CT or MRI
A large vessel infarct will usually affect..
A whole hemisphere
A small vessel infarct will usually affect…..
Central subcortical distribution
What is the usual cause of a small vessel infarct?
Longstanding hypertension
Arteriosclerosis
What is the usual cause of large vessel infarcts?
Atheroma
What is Prion disease an example of?
Creutzfeldt jakob Disease
Creutzfeldt Jakob Disease are examples of what?
Neurodegenerative Proteinopathy
What are the four subtypes of Prion disease?
Sporadic
Varient
Iatrogenic
Genetic
Describe Sporadic Prion disease.
Onset in 60’s
Rapid onset dementia
4 months to death
Describe Varient Prion disease/
Onset in the 20’s
Painful sensory disturbances
Neuropsychiatric decile
14 months to death
What are some causes of Variant prion Disease?
Exposure to BSE
Bovin Spongiform Encephalopathy
Describe Iatrogenic Prion disease.
Onset in the 30’s
Cerebellar or visual onset
Multifocal neurological decline
>2 years to death
What are some causes of Iatrogenic Prion disease.
Exposure to hGH (Human Growth Hormone)
less common now
Why is Iatrogenic Prion disease caused by hGH less common now?
hGH is no longer extracted from cadavers but produced in a lab.
What is Limbic Encephalitis ?
Inflammatory antibody mediated encephalitis
What are the symptoms of Limbic Encephalitis?
Short term memory deficits
+/- seizures
+/- behavioural changes
A CSF sample of someone with limbic encephalitis will show?
Antibodies
What can limbic encephalopathy be linked to?
Underlying malignancy or autoimmune condition
What is the treatment for Limbic Encephalopathy?
Treatment of tumour if present
Immunosuppression
What causes Alzhiemer’s?
Disruption of cholinergic pathway and synaptic loss
How do extracellular Amyloid plaques cause Alzheimer’s?
Disrupt normal cell function
Induce Apoptosis
How do intracellular neurofibrillary tangles cause the symptoms in Alzheimer’s?
Inhibit the cytoskeleton
Cell death
If someone presents with Alzheimer’s before 65 what is its likely aetiology?
Genetic
How does a genetic Alzheimers present?
Atypical presentation
What is an atypical presentation of Alzheimers?
Visuospatial disturbances
Primary progressive aphasia
What causes visuospatial disturbances?
Posterior Cortical Atrophy
What types of Primary Progressive Aphasia are there?
Lopogenic - Difficuilty thinking of words
Semantic - Naming
Non fluent - effortful speaking
If someone presents with Alzheimers after 65 what is its likely aetiology?
Sporadic
Environmental > Genetic
How does a sporadic Alzheimers present?
Usual pattern off forgetfulness.
An MRI of Alzheimers shows….
Atrophy of temporal and parietal lobes
A SPECT of an Alzheimers patient will show…
Reduced temperoparietal metabolism
What does the CSF of an Alzheimers patient show?
Decreased amyloid
Increase TAU ratio
What investigations are undertaken in a suspected Alzheimers case?
MRI
SPECT scan
CSF sample
Amyloid ligand imaging
What is the treatment for Alzheimers?
Acetylcholine boosters - Cholinesterase inhibitor
NMDA blocker
Give an example of an NMDA blocker used in Alzheimers?
Memantine
Give an example of a Cholinesterase inhibitor used in Alzheimers?
Rivastigmine
Donepezil
In an Alzheimers patient what else should be treated?
Address any vascular risk factors
When does Frontotemporal dementia usually present?
Early <65
What is the usual cause of Frontotemporal dementia?
Neurodegenerative proteinopathy
Aggregation of protein leads to cell death
List three proteins in order of occurrence that can cause a frontotemporal dementia.
TAU
TDD-43
Ubiquitin
What are the three presentations of a frontotemporal dementia?
Behavioural variant
Early frontal features
Early loss of insight
What behavioural variant is common in frontotemporal dementia?
Primary progressive aphasia
What are the frontal features that present in frontotemporal dementia?
Disinhibition
Lack of apathy
Loss of empathy
Compulsive behaviours
What is needed in order to assess whether a patient has lost their insight into oneself?
A collateral history
What investigations are undertaken in a suspected Frontotemporal dementia?
MRI
CSF
SPECT
What is to be seen on an MRI with frontotemporal dementia?
Atrophy of frontal lobes
What is to be found within the CSF of a patient with frontotemporal dementia?
raised TAU
Normal amyloid
What is to be seen on a SPECT with frontotemporal dementia?
Reduced frontal and temporal lobe metabolism
What is the management for Frontal Temporal Dementia?
Trazodone / Antipsychotics for behavioural features
Safety Management - access to money internet etc
Structured activities
Power of attorney
Attached a specialist nurse
A subcortical presentation of Vascular dementia due to small vessel infarction will present with?
Reduced attention
Executive dysfunction
Slowed processing
What is the management of Vascular Dementia?
Vascular risk factors are treated.
+/- cholinesterase inhibitors
What investigations are undertaken in Lewy body dementia?
DaT- Dopamine transport imaging
Alpha synuclein ligand imaging
CSF - alpha synuclein in CSF
What is the treatment for lewy body dementia?
Low dose Levodopa
Cholinesterase inhibitors
Someone with Parkinson’s Disease Dementia presents with these physical symptoms.
Bradykinesia, Rigidity, Tremor
Someone with parkinsons disease dementia present with these cognitive symptoms.
Dementi Reduced attention Slowness of processing Impaired visuospatial function Hallucinations
What is the management for Parkinsons Disease Dementia?
Small does Levodopa
Cholinesterase inhibitors
What is the management for Huntingtons?
No curative options
Mood stabilisers
Nurse specialist
If someone is over 65 with no additional neurology where are they referred?
old age psychiatry
If someone is under 65 with unusual features where are they referred?
Neurology
