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Alzheimer’s Disease and Other Dementia Related Disorders
Jason Schillerstrom, [email protected]
Learning ObjectivesList the diagnostic criteria for Major Neurocognitive
Disorder.
Describe cognitive deficits across multiple domains
Describe the clinical, pathological, and neuropsychological features associated with Neurocognitive Disorder due to Alzheimer’s disease.
Distinguish between Neurocognitive Disorder due to Alzheimer’s disease, cerebrovascular disease, Lewy Body disease, and frontotemporal lobar degeneration.
Major Neurocognitive Disorder1. Evidence of significant cognitive decline from a previous level of performance in one or more cognitive domains based on:
◦ Concern of the individual, a knowledgeable informant, or the clinician that there has been a significant decline in cognitive function; and
◦ A substantial impairment in cognitive performance, preferably
documented by standardized testing or, in its absence, another qualified clinical assessment.
2. The cognitive deficits interfere with independence in everyday activities (i.e. at a minimum, requiring assistance with complex instrumental activities of daily living such as paying bills or managing medications). 3. The cognitive deficits do not occur exclusively in the context of a delirium. 4. The cognitive deficits are not better explained by another mental disorder.
Major Neurocognitive DisorderSpecify whether due to: Alzheimer’s disease frontotemporal lobar degeneration Lewy body disease vascular disease traumatic brain injury substance/medication use HIV infection, prion disease Parkinson’s disease Huntington’s disease another medical condition
Major Neurocognitive DisorderSpecify:1. Without behavioral disturbances2. With behavioral disturbances: if the cognitive disturbance is accompanied by a clinically significant behavioral disturbance such as psychosis, mood disturbance, agitation, or apathy.
Specify:1. Mild: difficulties limited to instrumental activities of daily living2. Moderate: difficulties with basic activities of daily living3. Severe: fully dependent
Key Points #1The diagnosis of neurocognitive
disorders is based on clinical presentation.◦There are no “dementia labs”.◦There is no dementia imaging study.◦Laboratory and imaging are used to
“rule out” reversible causes.
There must be clinically significant functional impairment.
Cognition – Complex AttentionCognitive Domain Definition Examples of Symptoms or
ObservationsComplex Attention Sustained
attention, divided attention, processing speed
Major -Difficulty in environments with multiple stimuli. -Easily distracted. Difficulty holding new information. -All thinking takes longer than usual.
Minor -Requires more double checking than previously. -Thinking is easier when not distracted by radio, TV, etc…
Cognition – Learning and MemoryCognitive Domain Definition Examples of Symptoms or
ObservationsLearning and Memory
Immediate memory, recall memory, remote memory, implicit learning
Major -Repeats self in conversation. -Asks same questions again and again. -Cannot keep track of a short list of item.
Minor -Increasingly relies on lists. -Needs occasional reminders.-Loses track of whether bills have been paid.
Cognition – LanguageCognitive Domain Definition Examples of Symptoms or
ObservationsLanguage Expressive
language, word finding, receptive language
Major -Often uses general terms such as “that thing” and “you know what I mean”. -Prefers pronouns. -Echolalia. -Mutism.
Minor -Noticeable word finding difficulty. -Grammatical errors.
Cognition – Perceptual MotorCognitive Domain Definition Examples of Symptoms or
ObservationsPerceptual Motor Visuoperception,
visuospatial, praxisMajor -Difficulty with
using tools, driving, navigating in familiar environments.
Minor -May need to rely more on others for directions. -May find self lost when not concentrating. -Less precise in parking.
Cognition – Social CognitionCognitive Domain Definition Examples of Symptoms or
ObservationsSocial Cognition Recognition of
emotions, theory of mind
Major -Clearly out of acceptable range.-Insensitivity to social standards in conversation. -Focuses excessively on topic despite others disinterest.
Minor -Less able to read facial expressions. -Decreased empathy. -Increased extraversion or introversion.
Cognition – Executive FunctionCognitive Domain Definition Examples of Symptoms or
ObservationsExecutive Function Planning,
organizing, decision making, overriding habits, mental flexibility
Major -Abandons complex projects. -Needs to focus on one task at a time. -Relies on others to make decisions.
Minor -Difficulty resuming a task after an interruption. -Complains of fatigue from the effort of planning. -Difficulty following conversations of multiple persons.
Key Point #2There are multiple ways to be
cognitively impaired.
Executive function is the cognition that is most strongly associated with self-care abilities and decision making capacity.
Case Example #1: HPI83yr female presents to clinic with her two
daughters.
Daughters are concerned:◦ that their mother repeats her conversations◦ cannot remember the names of her grandchildren◦ became confused and disoriented when shopping
at a local mall.
The patient’s husband died one year ago and daughters are surprised how much they have to help their mother.
Past HistoryNo past psychiatry history.
Only medical issue is hypertension (treated with hydrochlorothiazide)
Retired teacher, 55yr marriage, 2 children
No clinically significant substance use history.
Case #1: Neuropsychological Testing
Age: 83 yearsGDS: 2/15 MIS: 6MMSE: 18CLOX1: 7CLOX2: 7EXIT25: 36
Alzheimer’s Disease Insidious onset and gradual
progression of impairment in one or more cognitive domains.
Subtypes include ‘early onset’ (65 years of age or below) vs. ‘late onset’ (>65 years of age).
Alzheimer’s Association StagingStage 1: No impairment
◦The person does not experience any memory problems.
◦No evidence of symptoms of dementia.
Stage 2: Very mild cognitive decline◦The person may feel as if he or she is having
memory lapses — forgetting familiar words or the location of everyday objects.
◦But no symptoms of dementia can be detected during a medical examination or by friends, family or co-workers.
Alzheimer’s Association StagingStage 3: Mild Cognitive Decline
◦Noticeable problems coming up with the right word or name.
◦Trouble remembering names of new people.◦Having noticeably greater difficulty
performing tasks in social or work settings.◦Forgetting material that one has just read.◦Losing or misplacing a valuable object.◦ Increasing trouble with planning or
organizing.
Alzheimer’ Association StagingStage 4: Moderate Cognitive
Decline◦Forgetfulness of recent events.◦Greater difficulty performing complex
tasks, such as planning dinner for guests, paying bills or managing finances.
◦Forgetfulness about one's own personal history (usually medical).
◦Becoming moody or withdrawn, especially in socially or mentally challenging situations.
Alzheimer’s Association StagingStage 5: Moderately severe cognitive
decline◦Unable to recall their own address or
telephone number or the high school or college from which they graduated.
◦Become confused about where they are or what day it is.
◦Need help choosing proper clothing for the season or the occasion.
◦Still remember significant details about themselves and their family.
◦Still require no assistance with eating or using the toilet.
Alzheimer’s Association StagingStage 6: Severe cognitive decline
◦Lose awareness of recent experiences and surroundings.
◦Difficulty remembering the name of a spouse or caregiver.
◦Need help dressing properly and may, without supervision, make mistakes such as putting pajamas over daytime clothes or shoes on the wrong feet.
◦Need help handling details of toileting.◦Experience major personality and behavioral
changes, including suspiciousness and delusions.
Alzheimer’s Association StagingStage 7: Very severe cognitive
impairment◦Lose the ability to respond to their
environment and to carry on a conversation. ◦Need help with much of their daily personal
care, including eating or using the toilet. ◦They may also lose the ability to smile, to
sit without support and to hold their heads up.
◦Reflexes become abnormal. ◦Muscles grow rigid. ◦Swallowing impaired.
Well Elderly
AD Affects Both ECF and Constructions
AD Pathology
http://www.umsl.edu/~homecare/brain1.PDD.jpg
AD Pathology
Tangle
Plaque
FDA Approved MedicationsAcetylcholinesterase Inhibitors
◦donepezil (Aricept)◦rivastigmine (Exelon)◦galantamine (Razadyne)◦tacrine (Cognex)
memantine (Namenda) – NMDA antagonist
Summary of FDA Approved Medications
All have demonstrated efficacy by the chosen outcome measures.
However, the effect size is small.
Few patients actually show improvement.
Some outcome measures are less relevant.
Exercise.
Case Example #2: HPI 73yr man presents to clinic with his wife.
She expresses concern for her husband stating, “I think he’s depressed. He just sits in his chair all day doing nothing. I have to do everything.”
He no longer manages finances and he needs assistance with his medications.
He denies feeling depressed and doesn’t understand wife’s concerns.
Past HistoryTakes medication for diabetes,
hypertension, and elevated cholesterol.
Had heart catheterization for coronary artery disease 5 years ago.
Retired produce salesman, married to current wife 22 years, 3 adult children.
Smokes one pack per day. Drinks 6-12 beers per week.
Case #2: Neuropsychological Testing
Age: 73 years
GDS: 2/15 MIS: 8MMSE: 26CLOX1: 4CLOX2: 9EXIT25: 30
Vascular DementiaEvidence for decline is prominent in
complex attention and frontal-executive function.
Memory is less impaired relative to loss of executive function.
Focal neurological signs
Evidence of hypertension, valvular heart disease, vascular disease, atrial fibrillation.
Vascular Dementia Affects the Executive Control of Clock-drawing
Vascular Dementia
Vascular Dementia
TreatmentMust prevent future stroke /
vascular disease:◦Aspirin◦Anticoagulants◦Exercise
Off label treatments for apathy◦Antidepressants (sertraline)◦Stimulants (methylphenidate)
Case #3: HPI 93yr female is brought to clinic by her daughter
(patient lives with daughter).
Daughter reports significant cognitive and functional decline over the past 1-2 years.
The patient reports occasionally seeing little men walking across her mantle.
She was started on antipsychotic by PCP and had severe dystonic reaction.
Daughter also reports that the patient talks and moves excessively in her sleep.
Past HistoryTakes medications for heartburn,
urinary incontinence, constipation, hypertension, and atrial fibrillation.
Reports frequent falls (3 in last year).
12th grade education, housewife, widowed 12 years.
No substance use history.
Case #3: Neuropsychological Testing
Age: 93 years
GDS: 1/15MIS: 6MMSE: 22CLOX1: 5CLOX2: 7EXIT25: 24
Lewy Body DementiaCore Features:
◦Fluctuating cognition with pronounced variations in attention or alertness.
◦Recurrent visual hallucinations that are well formed and detailed.
◦Spontaneous features of parkinsonism, with onset subsequent to the development of cognitive decline.
Lewy Body DementiaSuggestive Features
◦Meets criteria for rapid eye movement (REM) sleep behavior disorder.
◦Severe neuroleptic (antipsychotic medication) sensitivity.
ParkinsonismCognitive deficits are more
closely associated with rigidity and bradykinesia as opposed to tremor.
Parkinsonism in DLB tends to be:◦Less severe than that observed in PD◦More symmetric compared to PD◦Associated with more gait
abnormalities◦Less responsive to levodopa
(Sinemet)
Visual HallucinationsFully formed, detailed, 3-
dimensional objects, people or animals
Occur in 59%-85% of autopsy confirmed Lewy Body Dementia
Occur in early in the course of the disease (relative to AD hallucinations)
FluctuationsMimics delirium: waxing and waning of cognition,
behaviors, and arousal.
10% - 80% with poor inter-rater reliability
Differentiating DLB from AD:◦ Daytime drowsiness◦ Daytime sleep of 2 hours or more◦ Staring into space for long periods◦ Times when the patient’s ideas are disorganized, unclear
or illogical
3 out 4 has a positive predictive value of 83%
Other FeaturesREM Sleep Behavior Disorder:
augmented muscle activity and dream content; typically precedes onset of dementia, hallucinations, and Parkinsonism
Autonomic Instability
Perhaps a greater rate of decline
More responsive to acetylcholinesterase inhibitors.
Lewy Bodies
Kondi Wong, Armed Forces Institute of Pathology
Case Example #4: HPI APS called to investigate 60yr woman with self-neglect.
Squalor dwelling. No electricity, water, or sewer.
The client had a $2000 past due water bill and a ~$350 past due electrical bill.
APS facilitated a voluntary placement in a supervised setting where the client was allowed to take her dog. However, she became upset with management and decided to leave.
Would like to renovate home. Plan is to have Channel 4 News do a fundraising story for her.
Case Example #4 #1
Case #4: Neuropsychological Testing
Age: 60 years
GDS: 1/15 MIS: 8MMSE: 30CLOX1: 6CLOX2: 13EXIT25: 26
Frontotemporal Dementia – Behavioral Variant
1. Three or more of the following behavioral symptoms:a. Behavioral disinhibitionb. Apathy or inertiac. Loss of sympathy or empathyd. Perseverative, stereotyped or compulsive
behaviore. Hyperorality and dietary changes
2. Prominent decline in social cognition and/or executive abilities
3. Relative sparing of learning and memory and perceptual-motor function.
Frontotemporal Dementia – Language VariantProminent decline in language
ability in the form of speech production, word finding, object naming, grammar, or word comprehension.
Relative sparing of learning and memory and perceptual-motor function.
Frontotemporal DementiaFrontal and anterior temporal atrophy
(typically clearly detectable on CT or MRI scans), rarely parietal atrophy
Frontotemporal hypometabolism
Pick bodies (silver staining/argentophilic) intranuclear inclusions
Neuritic plaques/tangles not present
Frontotemporal Dementia
Frontotemporal Dementia
Frontotemporal Dementia
Off-Label Therapies for AgitationAntidepressants
Benzodiazepines
Antipsychotics◦Black box warning◦Increased risk of falls, stroke, and
death