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.