Dementia and HD Flashcards
What is dementia
cognitive impairment that affects the ability to think, remeber, and reason
what is dementia typically characterized by?
memory impairment, as well as difficulty in the domains fo language, motor activity, object recognition, and distrubance of executive function
What is executive function?
ability to plan, organize, and abstract
reasoning, problem solving, judgment and cognitive flexibility
What is memory cognitive domain ?
retain information and utilize it later for adaptive purposes
What is cognitve domain language ?
execute verbal function including spontaneous speeh, speech reptition and comprehension, naming, reading, writing
What is the cognitive domain visuospatial?
accurately perceive and understand the visual relationships between objects and space
What is cognitive domain attention
focus on specific piece of information for a sustained period of time while suppressing awaareness of other competing distractions
What are the signs and symptoms of dementia?
memory loss that disrupts daily life
challenges in planning or solving problems
difficulty completing familiar tasks
confusion with time or place
trouble understanding visual images and spatial relationships
new problems with words in speaking or writing
misplacing things, inability to retrace steps
decreased poor judegement
withdrawal from work or social activities
changesin mood and personality
What are the behavior and psychological symptoms of dementia?
aggression, agitation, apathy, deoression, dis-inhibition, mood lability, repetive questioning, sleep distrubances, socially inappropriate behaviors, wandering
What are the AD Risk factors?
genetics, hypertension, high cholesterol, dietary, cultural factors
What are AD protective factors?
regular physical activity fruits and vegetables intellectually stimulation activites leisure time physical activity at midlife is assoicated with a decreased risk of dementai and AD later in life vitamin C, coenzyme Q10, and folate
What is the definition of delerium
acute onset, cognitive flucutuations over hours or days
impaired consciousness and attention
altered sleep cycles
What is the definition of cognitively impaired, no dementia (CIND)?
clinical syndrome with deficits in memory or other cognitive abilities that have minimal impact on day to day functioning and does not meet criteria for dementia
What is mild cognitive impairment (MCi)
clinical subsyndrome of CIND. Amnestic or nonamnestic
What is Alzheimer dementia
dementia syndrome that has gradual onset and slow progression and is best explained as caused by Alzheimer disease
What is Alzheimer disease?
brain disease characterized by plaques, tangles, and neuronal loss
What are the subcortical types of dementia?
dementia with Lewy bodies (DLB)
Huntington’s disease
What is dementia with lewy bodies (DLB(
Lewy body proteins are present in the brain stem, depleting dopamin, causing PD symptoms
What is Huntington’s disease
hereditary disorder that causes degeneration in the brain resulting in movement disorder and cognitive delcine
What are the cortical dementiates?
Azheimers vacular dementia frontotemporal dementa wernicke-Korsakofff's syndrome pugilistic dementia (CTE)
What is frontotemporal dementia?
degeneration of nerve cells in the frontal or temporal lobes of the brain
those with ALS typically develop this type in mid to late stage
What is vascular dementia
caused by stroke or partially blocked blood flow
2nd most common type
What defines Alzheimer’s dementia
neurofibrillary tangels and neuritic plaques with amyloid
60-80% of dementias
What is chronic traumatic encephalopathy (CTE)
progressive neuro degeneration caused by
history of repetitive hits to the head
length of exposure to head impacts
CTE can only be diagnosed after death through brain tissue analysis
How many people are diagnosed with MCI each year?
10-12%
How many times likely are AA to have Alzheimer’s as white americans?
twice as likey
At what age is early onset AD?
prior to age 65, many in their 40s and 50s
When was AD discovered?
1901
What is the pathology of AD?
abnormal amounts of beta-amyloid are cleaved from the amyloid precurosr protein (APP) and released into the circulation
beta-amyloid fragments come together in clumps to form plaques that attach to the neuorn
microglia react to the plaque, and an inflammatory response results
What are neurofibrillary tangles?
tau proteins provide structural suppport for the neuron microtubules.
chemical changes in the neuron produce structural changes in tau proteins
this results in twisting and tangling
What is the symptom triad of normal pressure hydrocephalus (NPH)?
Difficulty walking
decline in thinking skills
loss of bladder control
What is the mechanism for NPH?
excess CF accumulates in the brain’s ventricles
despite excess fluid, CSF pressure as measuresd during a spinal tap is often nronal
some benefits from shunt placement
What are cholinesterarse inhibitors
acetrucholine– important brain neurotrans.– helps neurons communicate
Donepezil (Ariccept)
Rivastigmine (Exelon)
Galantamine (Reminyl)
What is the total score and average score and AD score for the Montreal Cognitive Assessment (MOCA)
total score= 30
26-30 = normal
6.2= AD
What are the ranges for the mini-mental state exam (MMSE)
total score= 30
20-24= mild dementia
13-19= moderate dementia
<13 severe dementia
What is memantine medical intervention for AD?
helps with symptoms related to glutamate
blocks glutatmate and acts on the NMDA receptor
What does the Huntington protein cause?
causes neuronal degeneration and eventual neuronal death
results in higher concentrations of dopamin and norepinephrine, disrupting the complex balance of excitation and inhibition between the thalamus and theBG for smooth, coordinated movement
What does autosomal dominant trat mean?
everyone who inherits the genetic mutation will develop the disease
if parent has mutation, there is a 50/50 chance
number of CAG repears ais a more sophisticated measure of risk
What is CAG repeat and what is normal
DNA nucleotide triplet of Cytosine, Adenine, Guanine (CAG)
normal number of repeats is <26
27-35– will not develop HD but will pass risk to children
36-39, some will develop HD
40+ all will develop HD and children will have a 50% risk
>50 repeats –juvenile onset
How do you diagnose HD?
histroy clinical signs: movement, cogntive, psychiatric postive family history imaging genetic testing
What areas of the brain are most affected in HD?
cerebral cortex and basal ganglia
cortical pyramidal nneurons from motor and premotor areas
indirect pathway is affected earlier in the disease, causing chorea and involuntary movements
direct pathway is affected later in the disease and causes rigidity and bradykinesia
striatonigral pathways are affecte later in the disease
What are the motor symptoms for adult onset HD?
choreiform movements
apraxia
cerebellar signs: ataxic gait, decreased force control
motor impersistene– inability to maintain a constant vountray contraction – results in dropping object, incomplete chewing, inconsistent driving speeds
dystonia– mainfests as arm elevation while walking, tilting of trunk, internal shoulder rotation, sustained fist clenching, excessive knee flexion, foot PF and INV
What are the cognitive/behavioral symptoms for adult onset HD?
decreased judgement loss of meomory deterioration of speech and writing depression hostility feelings of incompetence decrease in IQ changes in social behavior
What are the juvenile onset differences from adult HD
initail more sever cog-beh problems
gait difficulty
speech and swallow problems
choreiform movements
rigidity soone
seizures– myocloinc type in 25% of children
later stages– ataxia and other cerebellar signs, severs dystonia
What is the most common psychiatric condition for HD
depression
25% of patients attempt suicide once
6% of deaths
What is the prognosis for disease life span for HD
15-20 years after onset of symptoms
What are the pharmaclogical treatments for HD
dopamine antagonists decrease chorea – slows movement
antisense oligonucleotide is disease modifying – reduces Huntington messenger RNA
anticonvulsants, antipsychotics, antidepressants for symptom management
What is chronic tardive dyskinesia
involuntary movements of face, tongue, lips
What is Unifed Huntington’s disease rating scale (UHDRS)
1. motor assessment 2 cognitive assessment 3 behavioral 4 independence scale 5 functional assessment 6 total functional capacity
What defines the early stage of HD
stage 1: able to perform ADLS, live at home, work
stage 2: able to perform ADLs, live at home, work at lower level, needs assistance for finances
What defines the middle stage of HD
stage 3: needs minA for ADLs, cannot do IADLs, unable to work, can live at home with support
What defines the late stae of HD
Stage 4: needs MODA, may live in care facility
stage 5: needs maxA for ADLs, likely total care facility
What are common abnormalities in eye movements in HD
Saccades: decreased velocity, undershooting of target, latency in initiation of movement
gaze fixation abnormalities
visual tracking has saccadic tracking of smooth pursuit
visual distractibility
What can falls be attritbuted to in 30% of people with AD?
lack of perception of where their bodies are in space and their inability to move around objects
> What does the GEMS: brain change model focus on
rather than focusing on person’s loss when there is brain change, seeing individuals as persons, unique, and capable encourages a care partnership and is cor of this model
What does saphire GEM stand for
not experiencing dementia
What does diamonds stand for
Clear, sharp, rigid, inflexible and likes a habited routin
What does emerald GEM
not clear and shar
direction and tim frame is off
think they are fin
increased mistakes
What does Amber
caught in a moment of time, no caution, no safety, inconsistenct behavior
low high sensory needs
What does Ruby stand for
no fine motor, but gross strength is still there
What does pearl stand for
lose gross motor, ruled by reflexes
What doe sphysical activity do for dementia?
at least twice per week is associated with reduced risk of dementia
physical activity may alter Tau accumulation, synaptic function and number, restore neurogenesis, increase neurotrophin levels, positively alter inflammation and immunity, affect circadian rhyths, and improve cognition in those with AD
What is a behavioral strategy for those with dementia?
redirection
validation
What are the four Ps of PT managment
prevention
predcition
plasticity
participation
