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a group of disorders that cause global declines in 'cognitive' and behavioral functioning
often progressive
Causes: unkown |
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crystallized intelligence |
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specialized information learned over time
most related to formal education or diverse social experiences
more education, more general info/knowledge |
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Novel reasoning and the efficiency of 'solving new problems' or responding to abstract ideas |
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preserved-differentiation hypothesis |
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theorized: higher levels of crystallized intelligence may provide protection against function loss |
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Both types of intelligence start to decline at age __ |
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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 |
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higher IQ at age 11 predicted less likelihood for dementia at age 72
preserved-differentiation hypothesis |
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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 |
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differential-preservation hypothesis |
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by keeping mentally active or by increasing mental exercise, can e ward off cognitive decline?
research does NOT support this idea |
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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' |
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Brain structures more vulnerable to effects of aging |
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Hippocampus Frontal lobes (fluid intelligence) Specific association areas of the temporal and parietal lobes
Relatively preserved structures: Occipital and somatosensory cortices |
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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 |
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Mild Cognitive Impairment |
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a particular cognitive domain thats being impaired but not broad enough to be labeled dementia
Risk factor for dementi |
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Mild cognitive impairment related solely to memory |
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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 |
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Cortical Subcortical Mixed
causes: Vascular infections, toxic conditions, and other medical issues |
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diagnostic criteria of dementia |
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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' |
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Difference between Cortical/subcortical dementia |
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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 |
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Progressive/Static dementia |
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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 |
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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 |
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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 |
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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 |
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Definition
people with Down syndrome who reach 50 or above will get Alzheimer's disease |
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Diagnostic problem of Alzheimer's disease |
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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) |
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Major brain structures affected by Alzheimer's |
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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 |
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accumulated Tal proteins within cytoplasm of swollen cell bodies that destroys them
AD |
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clump-like deposits of beta-amyloid (amyloid plaques, also on chromosome 21) located around synapses destroy synapse
AD |
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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%) |
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Alzheimers and neuroimagimg |
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Definition
CT & MRI cerebral atrophy; not sensitive or specific enough for AD diagnosis
mainly used to rule out other causes |
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explicit, verbalizable, and accessible to conscious awareness deficit in new declarative learning (anterorgrade amnesia) noticed first Encoding, consolidation, retrieval Both verbal and visual |
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Breakdown of semantic knowledge
Brain contain "semantic networks" when activated lead to recognition -Wing, beak, fly... BIRD
Includes progressive disintegration of these associational networks |
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Alzheimer's memory, spared systems |
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Definition
Simple short-term memory - Not working memory - until later stages
Visual and motor procedural memory |
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Alzheimer's language and speech |
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anomic aphasia: word finding and naming difficulties
progression - comprehension difficulties - global aphasia |
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Alzheimer's visuospatial and intellectual functioning |
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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 |
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Alzheimer's executive functioning |
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Preservation- getting stuck on the same idea Poor planning Metacognitive awareness- lose sight - genuinely unaware of presence or severity - Need constant supervision |
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Alzheimer's orientation, attention, level of consciousness awareness |
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orientation generally intact not altered state of consciousness selective attention stay intact until later - more complex attention decline in early stages |
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Alzheimer's motor and sensory functions |
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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 |
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Alsheimer's mood, emotion, personality, and insight |
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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 |
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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 |
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Alzheimer's cognitive, behavioral, psychiatric symptom control |
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Definition
Antidepressants and minor tranquilizers - side effects Restructure environment - supervision Nursing homes Family members - 'day care' programs |
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progressive diseas process characterized by a dopamine deficiency of the substantia nigra
results in resting tremors and other motor symptoms
may cause subcortical dementia |
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characterized by the symptoms but not an actual disease
could be caused by: drugs, encephalitis, toxins, injury
Muhammad Ali |
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Parkinson's Disease entomology |
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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 |
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Age related
24-31% of those with PD meet criteria 12% in their 50s 70% older than 80 |
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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 |
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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 |
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Clinical presentation of PD |
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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 |
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Motor symptoms of PD: Negative symptoms |
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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 |
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Motor symptoms of PD: Positive symptoms |
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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 |
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Definition
Cognitive profile is heterogeneous -Depends largely on presence of dementia |
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PD: visuospatial deficits |
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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 |
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PD: executive functioning |
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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 |
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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 |
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PD
decreased voice emotional expression -monotone voice |
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PD
sporadic accelerated bursts of speech |
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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 |
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PD: Mood, emotion, personality, insight |
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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 |
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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 |
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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 |
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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 |
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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 |
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lesioning a small part of the thalamus to reduce tremor in particular |
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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 |
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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 |
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twisting writhing undulating (smooth, wavelike motion) grimacing movements of the face and body |
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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 |
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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 |
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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 |
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HD: clinical presentation |
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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 |
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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 |
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currently no cure -suicide?
Focuses mainly on the relief of emotional symptoms |
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Creutzfeldt-Jakob Disease (CJD) |
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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 |
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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 |
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Gerstmann-Straussler-Scheinker syndrome (GSS) |
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CJD variant
Extremely Rare Familial Results in fatal insomnia |
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CJD variant
SE found in a tribe in New Guinea ritually cannibalizing their deat at the beginning of the 1900's |
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"Spongy" appearance: cerebellum and cerebrum
Astrogliosis: -glial cells filling in after neuronal tissue had died -effect, not cause |
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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 |
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CJD: clinical presentaion |
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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 |
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No treatment No cure
By time of ID often too late |
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No treatment No cure
By time of ID often too late |
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