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Dementia - An 'Umbrella' Term

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Zoé Powell

on 18 February 2014

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Transcript of Dementia - An 'Umbrella' Term

Dementia
Dementia is often described as an 'Umbrella' term as it encompasses a wide range of symptoms. Although it mostly describes the symptoms that occur when the brain is affected by certain disease or conditions.

These symptoms can vary widely between each type of dementia.

An individual with dementia would have to have two or more of the following impaired core mental functions:

Memory
Communication and Language
Ability to Focus and Pay Attention
Reasoning and Judgement
Visual Perception
The variable types of dementia signifies that memory illnesses can differ in their cause, symptomatology, and pathology.


This differentiation between each type of dementia is important in the diagnosis and treatment of a specified mental illness. Although in the majority of cases, a correct diagnosis can not be made until postmortem.
What is dementia?
Risk Factors Involved
Types of Dementia
Alzheimer's Disease
Vascular Dementia
Dementia with Lewy Bodies
Fronto-temporal dementia
Most common type of Dementia. The majority of individuals that are diagnosed with AD are old than 65 years of age. Currently there are approximately 820,000 with LOAD.

There is a less prevalent type of AD (EOAD) that does occur in those that are younger than 65. It more uncommon, accounting for only 5-10% of all Alzheimer's cases. However, 13% of cases with EOAD are familial AD (have a genetic predisposition), this has great implications for research carried here.
Symtomatology:
Similar types of symptoms occur in those that have early-onset Alzheimer's disease. These signs include unusual memory loss, particularly when remembering recent events and the names of people and things.

As the disease progresses, individuals can exhibit more mood swings, inability to preform complex tasks.
Causes
The cause of AD is still mostly unknown except for the cases where genetic differences have been identified. Although, there environmental factors are also considered to contribute to the cause of Alzheimer's.
Genetic:

Autosomal dominant inheritance (Usually EOAD)
Mutations on the: APP gene (^ production of beta amyloid plaques), presenilins 1 & 2 (change ratio of amyloid and tangles)

Sporadic AD - mixture of environment and genetic factors (EOAD - 4%/LOAD - 95%)
Most common risk gene in APOE - Between 40 - 80% of people with AD possess at least on APOE4 allele
Genetic Varient - Polymorphism on SNP Between 40 - 80% of people with AD possess at least on APOE4 allele

Environmental:
Overweight
Unhealthy Diet
High Alcoholic intake
Lack of Exercise

Cholinergic Hypothersis
AD caused by the reduced synthesis of the neurotransmitter acetylcholine.

Amyloid Hypothesis
Accumulation amyloid plaques between nerve cells in the brain
Beta amyloid is snipped from an amyloid precursor protein
In healthy brains, these substances are normally broken down an eliminated.
Hereditary - APOE4 (sporadic LOAD) less effective break down
Evidence : Trisomay 21 universally exhibit AD due to APOE on chromosome 21

Tau Hypothesis
Neurofibrillary tangles are insoluble twisted fibers found inside the cell, primarily consisting of tau proteins.
These form microtubules, which normally help to transport nutrient
In those with Alzheimer's the tangles become hyperphosphrelated
Causes cell death.


The second most common type of Dementia.
Signs and signals are almost identical to Alzheimer's disease. Although after onset, a stepwise, as opposed to gradual, progression is typical.
Causes
Vascular (multi-infarct) dementia irreversible form of dementia. The onset is caused by a vascular lesion in the brain, these are most likely to be hemorrhagic lesions in the anterior cerebral artery, parietal lobes and thalamus

It is also likely that n individual can have both vascular dementia and vascular disease.

Risk Factors include; hypertension, smoking, hypercholesterolemia, diabetes, cardiovascular disease.
Pathology
Pathology
Noticeable lesions and artery damage. Accumulation of various substances such as lipid deposits and clotted blood.

Atrophy of white tissue and calcification of arteries
Is the third most common type of dementia. It is closely associated with Parkinson's disease. It is characterized anatomically by the presence of Lewy bodies, only properly diagnosed after death.
Signs, Symptoms and Cause:

Core features include:
Fluctuating cognition with great variations from day to day and hour to hour.
Recurrent visual hallucinations
motor features of Parkinson's Disease
Sensitivity to neuroleptic and antiemetic medications (affect the dopaminergic and cholingergic systems).

Causes are not yet understood, but here is a genetic link with the gene PARK11. As with Alzheimer's most cases appear sporadically. Similarly, risk of LBD is increased with the inheritance of the APOE4 gene.
Pathophysiology:

Lewy bodies (alpha-synuclein) is a protein that is abundant in the brain.

May regulate the release of dopamine, which is critical in controlling voluntary/involuntary movements.

The lewy bodies protein is also encoded by the the same gene that makes the amyloid fragment - hence why you 'd see amyloid plaques as well.

Effects of aging on memory but not AD:
Forgetting things occasionally
Misplacing items sometimes
Minor short-term memory loss
forgetting that memory lapses happened

Early Stages:
Absent-mindedness
Forgetting appointments
Slight changes seen by close loved ones
Some confusion in situations outside the familiar

Middle Stages:
Deeper difficulty remembering recently learned information
Deepening confusion in many circumstances
Speech impairment
Repeat the same conversation

Late Stages:
More aggressive or passive
Some loss or self-awareness
Debilitating cognitive deficit
More abusive, anxious or paranoid
Tangles and Protein deposits
Brain Cell Loss
Brain Atrophy, Neurotransmitter
Symptoms
A less common type of dementia. It refers to the damage caused to the frontal lobes in the brain that control emotion and behaviour.

It is a significant cause of dementia for those under the age of 65.
Signs and Symptoms
Behavioural Varient:
Disinhibitions (inappropriate, sometimes sexual, comment).
Apathetic (socially withdrawn,no longer able to take care of themselves.

Progressive nonfluent aphasia:
Breakdown of speech fluency and articulation.

Semantic dementia
Differs from nonfluence aphasia as they'd have normal phonology and syntax but difficulty with naming and understanding the meaning of words.

FTD individuals also struggle with compulsive behaviours such as binge eating and change of food preference.

Also experience loss of cognitive skills in planning and organizing.
Korsakoff Syndrome
A Chronic memory disorder characterized by a server deficiency of vitamin B-1, specifically Thiamine.

Nerve cells are particularly sensitive to a thiamine deficient.


In Korsakoff syndrome there is not enough thiamine to help produce glucose (source of energy), therefore the brain cells cannot function.

Most often caused by alcohol misuse.
Why Alcohol Misuse?
Evidence suggests that ethanol interferes directly with thiamine uptake int the intestinal tract.
In rats, it has been shown that for those exposed to higher levels of alcohol incurred neurological problems that result from thiamine deficiency.
Also common in those who have serve malnutrition or have had an eating disorders.

Risk Factors Include :
Chemotherapy
Dialysis
Extreme Dieting
Age
Genetic Factors
Causes:
An accumulation of Tau protein (Tauopathy), major scarring (gliosis) and cell destruction and death in the frontal and/or temporal parts of the brain.
Umbrella Term
Frontal Variant Fronto-Temporal Dementia
Frontal Temporal Dementia
Pick's Disease
Fronto-Temporal Dementia with Parkinsonism
Primary Progressive Aphasia
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