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33 Cards in this Set

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Dementia

A loss of mental ability that leads to the inability to function in normal, everyday life. Episodes must last more than 6 months, not since birth and is not associated with an alteration or loss of consciousness (not drug induced). AKA Neurocognitive Disorders

What type of disease are dementias often associated with?

Most are progressive, meaning they get worse over time and are usually caused by actual brain mass loss and are non-reversible.

What are some dementias that we can reverse the effects of?

Vitamin B12 Deficiency, hydrocephalus and encephalopathies (infection/trama induced inflammation)

How are some ways we categorize these diseases?

We categorize them by:


1) specific psychological abnormalities


2) motor problems


3) neuropathological features (post-mortem!)

What mental abilities must be significantly impaired to be considered a dementia (Cognitive & Behavioral Symptoms)? How many must a patient exhibit before being diagnosed?

Memory


Communication & Language


Focus & Attention


Reasoning & Judgement


Visual Perception


Behavioral & Personality Changes & Psychosis.


Patients need to exhibit significant impairment in two or more of these categories.

What are some of the first symptoms to arise?

Memory loss is the most common first symptom. It starts with losing the newest memories first and moves in reverse chronological order

What are some manifestations of Communication and Language problems?

A difficulty remembering words, misuse of words (naming the wrong object), mispronounced words/slurred speech, inability to understand instructions, speak in confusing sentences (inability to articulate thoughts)

What are some manifestations of Focus & Attention problems?

Trouble planning/organizing, difficulty with challenging mental arithmetic, easily distracted from tasks, tendency to wander and become lost, increased restlessness, overall confusion about surroundings.

What are some manifestations of Reasoning & Judgement problems?

inability to make decisions (stemming from logical association i.e. unable to match clothing to season. Inability to determine the appropriateness of their response or behavior to someone else's actions or statements, poor risk assessment, inability to learn new information (different bc memory and learning are not the same process)

What are some manifestations of Visual Perception impairment?

Patients have increasing difficulty detecting:


contrast


movement


differences in color


depth


Patients also have eye movement abnormalities. Eyes can be physically fine but patients report reduced fields of vision.

What are some pre-morbid symptoms?

people get more irritable and withdrawn which can lead to depression and anhedonia (inability to take pleasure in things that once gave joy) and have less interest in their own personal care.


What are some later (more aggressive) symptoms?

increased anxiety and paranoia, lack of social skills (increased inappropriate behavior), more likely to be violent and get in arguments, increased impulsivity and an increase in repetitive behaviors. begin to have sleep disturbances. Visual and auditory hallucinations

What are the motor symptoms of Dementia?

Weakening of the muscles leads to uncoordinated movements (patients describe it like walking through water), muscle spasms and rigidity, balance problems, slowing of eye movements, increased incontinence, difficulty swallowing


lastly is an almost complete loss of movement.

What are Neuropathological Features?

Structural or functional changes in brain cell morphology, physiology or metabolism.

What are the neuropathological features of dementia that we are looking for (Primary Features)?

1) What type of brain cell? (neuron or glia?)


a) What type of neuron/glia?


2) What types of inclusion bodies (insoluble protein aggregates)


3) What region of the brain are affected?

What are inclusion bodies? How are they related to insoluble protein aggregates?

Inclusion bodies are dense electron-retractile particles of aggregated proteins, clumps of these mis-folded proteins are implicated in several dementias

Why do we constantly change and refine how we group diseases?

The more clearly we define phenotypes the easier it is to find the gene associated with the disease

What are the four main types of neurodegenerative disorders?

Alzheimer (~60-70%), Frontotemporal (10-15%), Lewy Body (10-25%) and Vascular dementias (10-20%)

What is the most common form of dementia? How many are affected? What demographics?

AD is the most common dementia affected about 5 million americans. 1 in 3 seniors dies from AD complications and 2/3 of the patients are WOMEN - women over 65 are more likely to get AD than breast cancer

Who introduced Alzheimer as a diagnosis? Who was the first patient?

First classified by german Alois Alzheimer in 1906. Diagnosed 51 female Auguste Deter with 'presinile dementia'

How were samples of Auguste D's brain analyzed in 1910? What did they tell us?

Used a new silver staining technique by Franz Nissl. They found atrophy, amyloid plaques and neurofibrillary tangles that we now MUST find to fully diagnose AD in a patient.

What are the two things we need for a 100% AD diagnosis?

You need clinical (ALIVE) and post-mortem (DEAD) information to fully diagnose AD

What is the Clinical Progression of AD

1) Pre-morbid - no symptoms


2) Very Mild


3) Mild Cog. Decline


4) Moderate Cog Decline


5) Moderately Severe Cog Decline


6) Severe Cog Decline


7) Very Severe Cog Decline

Describe 'Very Mild'

Small memory lapses

Describe 'Mild Cognitive Decline'

Difficulty coming up with words, loss of more significant items and problems planning

Describe 'Moderate Cognitive Decline'

Short term memory loss, difficulty with complex problems, depression and withdrawing

Describe 'Moderately Severe Cognitive Decline'

Memory loss of older memories, increased confusion about location, increased reasoning impairments.

Describe 'Severe Cognitive Decline'

No personal history, no recognition of friends or family, sleep disturbances start to get much worse, increased distraction/wandering.Patients at this stage need significant assistance for everyday function

Describe 'Very Severe Cognitive Decline'

loss of ability to interact with environment, eventual loss of movement, loss of facial expression and control of neck muscles

How are patients grouped?

Patients are put into 3 groups - 1) non-impaired 2) Mild Cognitive Impairment (stages 2-4) and 3) Impaired (stages 5-7). * mild impairment MAY NOT progress

90% of AD cases are from what cause?

90% of AD cases are sporatic (no family history of disease) or idiopathic (literally 'unknown cause'). AGE is the most indicating factor.

What are the cause of approximately 10% of cases of AD?

About 10% of AD cases are associated with mutations in specific genes including; APP (codes for an amyloid precusor protein), PSEN1 and PSEN2 (both code for presenilin, a protease that is a pre-precursor for amyloid precursor protein) and are always early onset.

What is the only non-familial genetic risk for AD?

A mutation in the gene Apolipoprotein (APOE) - there are 3 isoforms of this allele, epsilon3 is the most common form of the protein population-wide but APOE-4 is 10x more likely to develop AD than those with APOE-2 but it is a RISK FACTOR and does not clearly link you to the disease. 1/3 of AD patients DONT HAVE APOE-4.