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Neuropsychology exam 4
UCF PSB 4240C Exam 4
80
Psychology
Undergraduate 4
04/12/2011

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Term
Dementias
Definition
a group of disorders that cause global declines in 'cognitive' and behavioral functioning

often progressive

Causes: unkown
Term
crystallized intelligence
Definition
specialized information learned over time

most related to formal education or diverse social experiences

more education, more general info/knowledge
Term
Fluid intelligence
Definition
Novel reasoning and the efficiency of 'solving new problems' or responding to abstract ideas
Term
preserved-differentiation hypothesis
Definition
theorized: higher levels of crystallized intelligence may provide protection against function loss
Term
Both types of intelligence start to decline at age __
Definition
75
Term
Nun study
Definition
lower levels of idea complexity at ages 18-32 predicted lower cognitive functioning after 75

the lower number of complex ideas the lower cognitive function

preserved-differentiation hypothesis
Term
Scotland Study
Definition
higher IQ at age 11 predicted less likelihood for dementia at age 72

preserved-differentiation hypothesis
Term
Digit span task
Definition
repeat numbers exponentially
(1,5; 3,2,7; 6,8,9,1... etc)

Those that died before 85 loss ability to repeat numbers

those still living were able to do the same amount
Term
differential-preservation hypothesis
Definition
by keeping mentally active or by increasing mental exercise, can e ward off cognitive decline?

research does NOT support this idea
Term
Cortical Atrophy
Definition
linear decrease in weight and volume across the lifespan

First recognizable signs:
Widening of ventricles
Neuronal shrinkage rather than loss of whole neurons
Gray matter shows linear decline (cell bodies)
White matter inverted U-shape
'Fluid intelligence'
Term
Brain structures more vulnerable to effects of aging
Definition
Hippocampus
Frontal lobes (fluid intelligence)
Specific association areas of the temporal and parietal lobes

Relatively preserved structures:
Occipital and somatosensory cortices
Term
Swedish Study
Definition
85% of elderly adults' brains showed minimal evidence of cerebral atrophy

Over 85, gray matter atrophy is apparent on CT and MRI scan

Genetic and environmental factors:
Stress- BAD- hippocampal volume
Aerobic exercise- GOOD- brain less damages by atrophy
Term
Mild Cognitive Impairment
Definition
a particular cognitive domain thats being impaired but not broad enough to be labeled dementia

Risk factor for dementi
Term
Amnestic subtype - MCI
Definition
Mild cognitive impairment related solely to memory
Term
Defining Dementia
Definition
Very broad; disagreement

A behavioral syndrome, whereas AS is a particular disease
Many different diseases and medical conditions can cause dememntia
Various subtypes usually relate to suspected disease, cause, or primary site of damage
Term
Degenerative dementias
Definition
Cortical
Subcortical
Mixed

causes:
Vascular infections, toxic conditions, and other medical issues
Term
diagnostic criteria of dementia
Definition
no one set of criteria

Must include memory impairment
DSM-IV-TR requires additional area of cognitive impairment

'Loss of intellectual functioning and multiple areas of cognitive functioning'
Term
Difference between Cortical/subcortical dementia
Definition
Cortical: primarily affect the cerebral cortex (gray matter)
Alzheimer's

Subcortical: mostly affect the white matter, as well as gray matter beneath the cortex
Huntington's and Parkinson's

Major area of damage, not exclusive
generally affect a big part of brain and cannot be defined as simply cortical or subcortical
Term
Progressive/Static dementia
Definition
Progressive: continuously worsen
dementias that result for a disease process

Static: steady state
neurotoxic substance

Both can have insidious or acute onset

Vascular Dementia- stepwise progression
Term
Delerium
Definition
May be misdiagnosed as dementia

altered state of consciousness
grossly confused and disoriented to their surroundings
caused by specific organic problems; overmedication, acute worsening medical conditions
Term
Alzheimer's disease
Definition
An Irreversible, progressive, cortical dementia, not due to an identifiable cause, that is characterized by neuropathologic markers including neurofibrillary tangles and senile plaques

8th leading cause of death in elderly (>65)
1% 65-75
6-8% over 85
10-30% over 85 have AD
Those diagnosed with dementia = 50% have AD
Term
Alzheimer's info
Definition
No single cause
linked to increased age

Women 2x as likely
longer life expectancy

More education less likely to develop AD
cognitive reserve

No clear genetic component
chromosomes 1,14,21 (down syndrome)
protein ApoE4
Term
Down Syndrome and AD
Definition
people with Down syndrome who reach 50 or above will get Alzheimer's disease
Term
Diagnostic problem of Alzheimer's disease
Definition
definite diagnosis of AD cannot be made until autopsy

highly over diagnosed
64.5% concordance rate of autopsy to clinical diagnosis (35% diagnoses false)
Term
Major brain structures affected by Alzheimer's
Definition
Cortical (brain shrinkage) + subcortical limbic system structures (hippocampus and amygdala)
Major pathways to and from hippocampus

Primary motor and sensory areas and basal ganglia spared
Term
Neurofibrillary tangles
Definition
accumulated Tal proteins within cytoplasm of swollen cell bodies that destroys them

AD
Term
Senlie plaques
Definition
clump-like deposits of beta-amyloid (amyloid plaques, also on chromosome 21) located around synapses
destroy synapse

AD
Term
Acetlycholine (ACh)
Definition
synthesized in the basal forebrain cholinergic complex (BFCC)
lie in basal forebrain
axons project to hippocampus and cortex

devestation of BFCC neurons depress brain levels of ACh (60-90%)
Term
Alzheimers and neuroimagimg
Definition
CT & MRI
cerebral atrophy; not sensitive or specific enough for AD diagnosis

mainly used to rule out other causes
Term
Declarative memory
Definition
explicit, verbalizable, and accessible to conscious awareness
deficit in new declarative learning (anterorgrade amnesia) noticed first
Encoding, consolidation, retrieval
Both verbal and visual
Term
Alzheimers memory info
Definition
Breakdown of semantic knowledge

Brain contain "semantic networks" when activated lead to recognition
-Wing, beak, fly... BIRD

Includes progressive disintegration of these associational networks
Term
Alzheimer's memory, spared systems
Definition
Simple short-term memory
- Not working memory
- until later stages

Visual and motor procedural memory
Term
Alzheimer's language and speech
Definition
anomic aphasia: word finding and naming difficulties

progression
- comprehension difficulties
- global aphasia
Term
Alzheimer's visuospatial and intellectual functioning
Definition
middle stage; sometimes before
more noticeable in novel environments
copying and remembering complex abstract figures

Intellect
General decline in cognitive functioning
- not all at the same rate
- abstract thinking impaired
- crystallized int remains intact until later
- somewhat slower processing speed, not as extreme as subcortical dementias
Term
Alzheimer's executive functioning
Definition
Preservation- getting stuck on the same idea
Poor planning
Metacognitive awareness- lose sight
- genuinely unaware of presence or severity
- Need constant supervision
Term
Alzheimer's orientation, attention, level of consciousness awareness
Definition
orientation generally intact
not altered state of consciousness
selective attention stay intact until later
- more complex attention decline in early stages
Term
Alzheimer's motor and sensory functions
Definition
basic vision and hearing stay intact
Olfaction declines early
simple motor speed and strength persist until later stages
-more complex skilled coordination declines earlier
Term
Alsheimer's mood, emotion, personality, and insight
Definition
Personality changes
Often first referred for psychiatric issues

Depression
Worthlessness, cognitive difficulties, fatigue, loss of energy, depressed mood, trouble thinking
Differential diagnosis difficult (40% overlap)
- depressed patients exaggerate memory problems
- dementia minimize loss of insight
Term
Alzheimer's treatment
Definition
No current available treatments can reverse, halt, or notably slow the progression of Alzheimer's

treatments for cognitive enhancement
- ACh agonists
-- weak ability to slow progression
Term
Alzheimer's cognitive, behavioral, psychiatric symptom control
Definition
Antidepressants and minor tranquilizers
- side effects
Restructure environment
- supervision
Nursing homes
Family members
- 'day care' programs
Term
Parkinson's Disease
Definition
progressive diseas process characterized by a dopamine deficiency of the substantia nigra

results in resting tremors and other motor symptoms

may cause subcortical dementia
Term
Parkinsonism
Definition
characterized by the symptoms but not an actual disease

could be caused by: drugs, encephalitis, toxins, injury

Muhammad Ali
Term
Parkinson's Disease entomology
Definition
Cause is unknown
selectively affects the substantia nigra and dopaminergic systems of the brain

1% of pop of US older than 50
incidence of new cases increasing with age
More common in males
Term
PD; dementia
Definition
Age related

24-31% of those with PD meet criteria
12% in their 50s
70% older than 80
Term
Neuropathology of PD
Definition
degeneration of dopaminergic cells and pigmentation in the substantia nigra

Midbrain structure; connects with basal ganglie
Fluidity of overlearned and semiautomatic motor movements
Loss of dopamine cells leads directly to the PD symptoms
Term
Lewy bodies
Definition
small granular structures found in dying cells located in the substantia nigra

They are either Indicators of general disease process
or
Markers of cell death

not specific to PD
Term
Clinical presentation of PD
Definition
insidious and slow onset

Vague aches and pains
General malaise
Irritable or depressed

Motor symptoms
weaness in arm or leg
problems holding a pen
voice quality becomes softer/more monotone
facial expression appears flat
Term
Motor symptoms of PD: Negative symptoms
Definition
Bradykinesia: poverty of movement
-slowed and reduced magnitude
-semiautomatic movements may appear almost frozen
Masked faces: emotionless face
Festinating slow gait: small rapid shuffling
Slowed speech
Decreased voice amplitude
Hypokinesia: reduced motor initiation
Micrographia: slowed and small writing
Term
Motor symptoms of PD: Positive symptoms
Definition
Resting tremor: pill rolling, rhythmic shaking
-Generally starts in one hand
-Stops with voluntary movement; sleep
-Increases with concentration, alertness, nervousness
-Not predictable; bursts

Rigidity: a tightening of muscles and joints
-Cogwheel rigidity: ratchet tigthening
-Stooped posture
-Poor balance
Term
Cognitive deficits of PD
Definition
Cognitive profile is heterogeneous
-Depends largely on presence of dementia
Term
PD: visuospatial deficits
Definition
Many but not all patients have problem
-Most commonly reported
-Research is conflicting
-Heterogenous population; hard to compare

-Difficulty orienting themselves to space
--Basal ganglia dysfunction responsible
Term
PD: executive functioning
Definition
Perveveration- difficulty changing mental sets
-continue to use same strategy, even when not working
(trying to find shoes so keep looking in the same place)

Difficulty maintaining mental sets
-finally shift sets; trouble maintaining that new set, and revert back to original set

Temporal sequencing problems
-difficulty in time-stamping events
-memory of event sequencing is disturbed

Appear to be related to deficits in the premotor area and the projections to the frontal lobe from the basal ganglia
Term
PD: speech/language
Definition
No classic signs of aphasia
No difficulty with grammar or sentence structure
General language processing and comp intact
70% of patients show problems with articulation and neuromechanical aspects of speech
-decreased voice amplitude
-dysphonia
-tachyphemia
compulsive word or phrase repitition
Term
dysphonia
Definition
PD

decreased voice emotional expression
-monotone voice
Term
tachyphemia
Definition
PD

sporadic accelerated bursts of speech
Term
PD: Memory
Definition
Memory deficits NOT on the level found with alzheimer's

**Declarative memory: problems with using strategies for effective organization and retrieval of information**
-impaired free recall
-normal performance when they have a cue

nondeclarative learning is generally not impaired unless it requires motor skills or executive functioning
Term
PD: Mood, emotion, personality, insight
Definition
Large portion experience depression
- more so than with patients with other diseases
Unclear if the depression is due to:
- Brain changes associated with illness
- As a result of medications used to treat illness
Term
PD: treatments
Definition
1860's use of anticholinergic drugs
- extracted from plant source
mechanism of action was unknown
blocked the action of ACh
side effects: dry mouth, blurred vision, confusion, slurred speech, visual hallucination

Pallidotomies

Thalotomies

Deep brain stimulation
Term
Pallidotomies
Definition
First surgical treatment for PD
-alleviate symptoms of the "malfunctioning circuitry" in the basal ganglia through heat induced surgical lesioning of the global pallidus
--motor improvements
--prior to CTs and MRIs- precision not good
--Negative side effects likely
Term
L-dopa
Definition
1961- miracle drug
- naturally occuring precursor for dopamine
-- directly metabolizes into dopamine
-- surgeries fell to the wayside
-- effectively alleviates tremor, bradykinesia, and rigidity

Usually taken orally along with another drug called:
carbidopa: prevents it from being converted into dopamine until after it crosses into the brain
-allows 5X as much to cross BBB
Term
L-dopa deficits
Definition
10 year average
-drug becomes ineffective

dopaminergic neurons become hypersensitive

On/off syndrome
-On: bad side effects
-Off: symptoms reemerge

Bad side effects such as bad nightmares, disturbed sleep, perceptual illusions, hypomania
Term
Thalotomies
Definition
lesioning a small part of the thalamus to reduce tremor in particular
Term
Deep brain stimulation
Definition
impanting an electrode (usually to a part of thalamus) that interferes with neuronal transmission in that area
-implanted adjustable neurostimulator
-helps control the tremor
-disease still progresses
-only helps with tremor for a period of time
- still take medication
Term
Huntington's Disease
Definition
Genetic, autosomal dominant progressive subcortical dementia that inflicts devastating motor impairments in the form of chorea, as well as cognitive declines on adults in the prime of their lives

in about 5-10/100,000

Passed down by 1 parent
-offspring has 50% chance
Term
Chorea
Definition
twisting
writhing
undulating (smooth, wavelike motion)
grimacing movements of the face and body
Term
George Huntington
Definition
first described this disorder in 1872

Adults in the prime of there life "go insane," develop a tendency towards suicide, and suffer devastating motor impairment in the form of chorea

1960's begins the gene search
Term
HD gene search
Definition
Driven by family members of those suffering
-Founded organization to raise money for research
-1993 discovered the disease

Linked to gene ITI5 on chromosome 4
-too many CAG repeats on this gene in huntington's
--normal CAG: 11-34
--HD CAG: 37-100
Term
Caudate nucleus: HD
Definition
part of the striatum/basal ganglia
-controls the proper timing, ordering, and sequencing of movement patterns

Other areas of the brain also eventually degenerate

CT and MRI clearly show a loss of gray matter in the caudate, as well as a corresponding widening of ventricles
Term
HD: clinical presentation
Definition
Classic prefrontal lobe problems:
-Rigidity, perseveration, difficulty switching mental sets
-Poor executive control
--attention/concentration
low free recall, improvement with recognition cues
-Difficulty in determining their position in space (egocentric spatial disorder)
--theorized to be an ineffectiveness of the caudate nucleus readjusting to changes in spatial poisition

Emotional disturbance common

Often starts before the motor symptoms
-most common: depression, but wide range of psychiatric disturbances
-suicide rate the highest (6%) of all dememntias
-likely a predisposition, which are worsened in reaction to learning about the illness and their future fate
Term
HD: motor difficulties
Definition
Chorea
Worse with stress; disappears during sleep
Wide-based gait
Speech dysarthic (hard to understand)
-erratic in rate of production and staccato with intermittent pauses
Clumsy and uncoordinated
Term
HD: treatment
Definition
currently no cure
-suicide?

Focuses mainly on the relief of emotional symptoms
Term
Creutzfeldt-Jakob Disease (CJD)
Definition
Subcortical dementia characterized by quick progression
Cascading decline over 3-4 months
-dementia with fastest progression

Very rare: 1/1million per year
Same prevalence around the world
Does not vary across cultures
Generally 50-60 years old
Term
CJD: causes
Definition
Hypothesized to spontaneously arise as a random mutation
- transplants of affected neural tissue
- cornea transplants
- contamination via medical precedures

Recently found CJD can cross species through consumption of tainted meat containing neural tissue
-Mad cow disease
-Sheep: scrapie

Extensive spongelike holes appear in the brains
- spongiform encephalopathy (SE)
- mechanism by which brain becomes infected unkown

CJD dies out with it's victim if not passed on
Term
Gerstmann-Straussler-Scheinker syndrome (GSS)
Definition
CJD variant

Extremely Rare
Familial
Results in fatal insomnia
Term
Kuru
Definition
CJD variant

SE found in a tribe in New Guinea ritually cannibalizing their deat at the beginning of the 1900's
Term
CJD: Pathology
Definition
"Spongy" appearance: cerebellum and cerebrum

Astrogliosis:
-glial cells filling in after neuronal tissue had died
-effect, not cause
Term
Scrapie
Definition
Small, twisted, sticklike fibers
-may be the disease agent that causes brain degen
-- found in brains of CJD and Kuro

AKA prion proteins
-disease and normal prion proteins have identical DNA
--vody doesnt reconize prions as forien!
--prions are now focus of current research
Term
CJD: clinical presentaion
Definition
Psychiatric symptoms usually first, followed by motor symptoms

Movement uncoordinated (drunken stagger)
Involuntary tremors/facial grimaces
Patients no longer can swallow (starve)
Sometimes blindness

Progression of dementia occurs in less than a year
- 3 to 4 months
Term
CJD: cure
Definition
No treatment
No cure

By time of ID often too late
Term
CJD: cure
Definition
No treatment
No cure

By time of ID often too late
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