Understanding and Treating Dementia Understanding and Treating Dementia (Neurocognitive Disorders)(Neurocognitive Disorders)

in Intellectual Disability in Intellectual Disability

Tolisano DDS December 2013Tolisano DDS December 2013

Demographics of DementiaDemographics of Dementia

In the general population, dIn the general population, dementia affects 5-10% of those ementia affects 5-10% of those aged 65 to 74, and 40% of those over 85. aged 65 to 74, and 40% of those over 85.

It accounts for more than 50% of nursing home admissions.It accounts for more than 50% of nursing home admissions.

At least 5 million people in the United States are diagnosed At least 5 million people in the United States are diagnosed with dementia. The term is retained with the DSM-5 for with dementia. The term is retained with the DSM-5 for continuity. continuity.

Neurocognitive disorder is now the preferred terminology, Neurocognitive disorder is now the preferred terminology, especially with impairments secondary to other conditions especially with impairments secondary to other conditions that affect younger individuals (e.g., TBI).that affect younger individuals (e.g., TBI).

Relationship between Dementia and Intellectual Disability

Longevity has increased for people including those with ID. As greater numbers are surviving into older age, there is a higher risk of developing dementia.

This is uniquely true for those with certain genetic disorders, such as Down syndrome who have four times the risk of developing Alzheimer’s disease.

  Overall, the age-related prevalence of dementia in

persons with intellectual disability is similar to the general population.

Assessment of Dementia in Intellectual Disability

Assessment is complex due to confounds, such as pre-existing cognitive impairment, physical difficulties, and mental health comorbidity.

This may result in dementia progressing before the initial diagnosis is made.

Early recognition and intervention are key.

Assessment of Dementia in Intellectual Disability

There is no consensus about the optimal test battery to use in detecting dementia in persons with intellectual disability. 

No reliable means of determining etiology. Although there have been advances in neuroimaging, the best confirmation of dementia remains by autopsy.

The diagnosis is a process of recognizing and accounting for the decline from the individual’s previous or baseline level of functioning.

This underscores the importance of establishing an individual’s premorbid capabilities prior to the onset of perceived changes.

Brain RegionsBrain Regions

General Signs and Symptoms of Dementia

Appreciable disturbance in higher cortical functions:- Memory - Calculation - Thinking - Orientation - Language - Reasoning- Comprehension - Learning - Judgment- Skill sets

Onset is often gradual.

Course is chronic, progressive, and irreversible.

However, in certain phases, the decline may be static.

Consciousness is not clouded.

General Signs and Symptoms of Dementia

Impaired cognition is often accompanied by deterioration in emotional control, social behavior, and motivation.

Motor problems may occur at different stages, depending on the type of dementia. For example, they occur early in vascular dementia and late in Alzheimer's disease.

Decline in activities of daily living, such as washing, eating, and toileting often depend upon the setting in which the individual lives, especially in the context of Down syndrome.

The Basics of The Basics of ScreeningScreening for Dementia for Dementia

In the early stages, memory impairment usually affects In the early stages, memory impairment usually affects registration, storage, and retrieval of registration, storage, and retrieval of newnew information. information.

In the late stages, In the late stages, olderolder material (e.g., birthplace, names of material (e.g., birthplace, names of siblings) may be lost. siblings) may be lost.

Short-term memory tests :Short-term memory tests : Registering 3 objects and recalling them after 5 minutesRegistering 3 objects and recalling them after 5 minutes List names of objects within categories (animals, foods, furniture)List names of objects within categories (animals, foods, furniture)

Screening tests may include the MMSE, Cognistat, RBANS, Screening tests may include the MMSE, Cognistat, RBANS, and DRS.and DRS.

The Basics of The Basics of ScreeningScreening for Dementia for Dementia

Diagnosis requires deficits in at least one of the following areas: Diagnosis requires deficits in at least one of the following areas: Impaired ability to plan, organize, and sequenceImpaired ability to plan, organize, and sequence Issues with thinking abstractlyIssues with thinking abstractly Agnosia: Inability to identify objects despite intact sensesAgnosia: Inability to identify objects despite intact senses Apraxia: Problems with learned activities despite intact motor Apraxia: Problems with learned activities despite intact motor

functions functions Aphasia: Impairment in comprehending or expressing Aphasia: Impairment in comprehending or expressing

language language

Each cognitive deficit must substantially impair functioning and Each cognitive deficit must substantially impair functioning and represent a significant decline from the previous ability level. represent a significant decline from the previous ability level.

Differential DiagnosisDifferential DiagnosisFalse-PositivesFalse-Positives

DeliriumDelirium is a reversible condition. The features are usually is a reversible condition. The features are usually inattentivenessinattentiveness and poor awareness. The symptoms have a and poor awareness. The symptoms have a short durationshort duration. It can be . It can be superimposed on dementia.superimposed on dementia.

Side-effects to certain medications Side-effects to certain medications may mimic or worsen symptoms of may mimic or worsen symptoms of dementia : dementia : Antihistamines Antihistamines Benzodiazepines and anticholinergics Benzodiazepines and anticholinergics Tricyclic antidepressants and antipsychoticsTricyclic antidepressants and antipsychotics

Other Important Medical Considerations:Other Important Medical Considerations: Substances (Intoxication or Withdrawal States)Substances (Intoxication or Withdrawal States) Mixed level of activityMixed level of activity Urinary tract infectionsUrinary tract infections Renal or liver failure causing toxicityRenal or liver failure causing toxicity

Is the condition better accounted for by another medical condition or Is the condition better accounted for by another medical condition or mental disorder?mental disorder?

PseudodementiaDepressive DisorderDepressive Disorder

Depression may be the first sign of early stage dementia. Depression may be the first sign of early stage dementia.

Prevalence of major depressive disorder in people with Prevalence of major depressive disorder in people with dementia is falls between 6% to 20%. Dementia can cause dementia is falls between 6% to 20%. Dementia can cause brain changes that lead to depression.brain changes that lead to depression.

Those with only depression rarely forget important current Those with only depression rarely forget important current events or personal matters.events or personal matters.

Neurologic examinations are normal except low motivation or Neurologic examinations are normal except low motivation or psychomotor slowing. psychomotor slowing.

Those with depression make little effort to respond, while Those with depression make little effort to respond, while those with dementia often try hard, but respond incorrectly. those with dementia often try hard, but respond incorrectly.

When depression and dementia coexist, treating depression When depression and dementia coexist, treating depression does not fully restore cognition. does not fully restore cognition.

Psychiatric Symptoms Associated with Dementia

Psychosis —hallucinations, delusions, or paranoia—occur in 10% of individuals with dementia, although a higher percentage may experience these symptoms temporarily.

Anger and Aggression –Dementia causes individuals to lose their impulse control and become disinhibited.

Anxiety—the diagnosis of dementia itself can cause anxiety. The person may fear the effects of the disease in the future, worry about making mistakes and forgetting things, get anxious when separated from caregivers, or become confused when schedules are changed.

Telling the Difference between Dementia and Age-Related Cognitive Decline

Signs of DementiaSigns of DementiaPoor judgment and decision makingPoor judgment and decision making

Losing track of the date or the Losing track of the date or the seasonseason

Difficulty having a conversationDifficulty having a conversation

Misplacing things and being unable Misplacing things and being unable to retrace steps to find themto retrace steps to find them

Up to 50% of individuals with mild Up to 50% of individuals with mild cognitive impairment develop cognitive impairment develop dementia usually within 3 yearsdementia usually within 3 years

Confabulation (i.e., filling in memory Confabulation (i.e., filling in memory gaps with false information)gaps with false information)

Typical Age-Related ChangesTypical Age-Related ChangesMaking a bad decision once in a Making a bad decision once in a whilewhile

Forgetting which day it is and then Forgetting which day it is and then remembering laterremembering later

Sometimes forgetting which word Sometimes forgetting which word to useto use

Losing things from time to timeLosing things from time to time

Slower recallSlower recall

Performance is adequate when Performance is adequate when given enough timegiven enough time

Major Neurocognitve DisorderMajor Neurocognitve Disorder

SignificantSignificant decline from previous level of cognitive decline from previous level of cognitive functioning: functioning: Complex Attention, Executive Functions, Memory, Language, Complex Attention, Executive Functions, Memory, Language,

Motor Abilities or Social SkillsMotor Abilities or Social Skills Based on Based on collateral information collateral information including self-report including self-report andand

standardized neuropsychological tesstandardized neuropsychological testing or ting or quantified quantified clinical assessmentclinical assessment..

Cognitive deficits interfere with everyday activities:Cognitive deficits interfere with everyday activities: For example, requires assistance in areas that were For example, requires assistance in areas that were

previously independent.previously independent.

Mild Neurocognitve DisorderMild Neurocognitve Disorder

ModestModest decline from previous level of cognitive decline from previous level of cognitive functioning: functioning: Complex Attention, Executive Functions, Memory, Language, Complex Attention, Executive Functions, Memory, Language,

Motor Abilities or Social SkillsMotor Abilities or Social Skills Based on Based on collateral information collateral information including self-report including self-report andand

standardized neuropsychological tesstandardized neuropsychological testing or ting or quantified clinical quantified clinical assessmentassessment..

Cognitive deficits Cognitive deficits do not do not interfere with the capacity for interfere with the capacity for independence in everyday activities.independence in everyday activities.

Dementia Classifications Dementia Classifications DSM-5: Major or Minor Neurocognitive Disorder due to…

Types:o Alzheimer’s vs. Non-Alzheimer’s o Vascularo Lewy Bodyo Frontotemporalo Hydrocephaluso Traumatic Brain Injuryo Substance/Medication-Inducedo Prion (Transmittable Disease)o Parkinson’s and Huntington’s o Multiple Etiologieso Unspecified

Cortical or Subcortical

Common or Rare

Dementia of the Alzheimer’s Type

Biochemical problems inside brain cells from abnormal Biochemical problems inside brain cells from abnormal proteins called proteins called amyloid plaques amyloid plaques and and neurofibrillary neurofibrillary tanglestangles..

Most common cause of dementia. Accounts for > 65% of Most common cause of dementia. Accounts for > 65% of dementias in the elderly.dementias in the elderly.

Twice as common in women because they have a longer Twice as common in women because they have a longer life expectancy. life expectancy.

In the early stage, individuals with Alzheimer's disease are In the early stage, individuals with Alzheimer's disease are often better groomed and neater than those with other often better groomed and neater than those with other dementias.dementias.

VASCULAR DEMENTIA

Cognitive deterioration related to cerebrovascular disease.

Second most common cause of dementia among the elderly. Common in men after age 70.

Risk factors include strokes, TIA, hypertension, diabetes mellitus, hyperlipidemia, and smoking.

Both vascular dementia and Alzheimer's disease can exist.

Decline appears gradual because small ischemic changes. The “patchy” course can be frustrating to caregivers.

Cognitive loss may be focal and there may be greater awareness of deficits.

LEWY BODY DEMENTIA

Cognitive deterioration due to changes in cortical neurons.

Third most common dementia. Age of onset is typically > 60.

Lewy body dementia, Parkinson's disease, and Alzheimer's disease overlap considerably.

Lewy Body Dementia is differentiated from Alzheimer’s: Fluctuating cognition. Alertness and coherence alternate

with unresponsiveness and confusion.

Hallucinations and delusions are common.

Short-term memory may be preserved.

Rigidity occurs early and tremors occur later.

FRONTOTEMPORAL DEMENTIAFormerly Pick’s DiseaseFormerly Pick’s Disease

Hereditary disorders that affect the frontal and temporal lobes. Hereditary disorders that affect the frontal and temporal lobes.

Accounts for up to 10% of dementias.Accounts for up to 10% of dementias.

Age at onset is typically younger (age 55 to 65).Age at onset is typically younger (age 55 to 65).

Mainly affects personality and languageMainly affects personality and language

Behavior becomes disinhibited and repetitive. Behavior becomes disinhibited and repetitive.

HYDROCEPHALUS

Characterized by gait disturbance (unsteady balance), Characterized by gait disturbance (unsteady balance), urinary incontinence, and enlarged brain ventricles.urinary incontinence, and enlarged brain ventricles.

This disorder accounts for up to 6% of dementias. This disorder accounts for up to 6% of dementias.

Improvements after removal of CSF, may predict the Improvements after removal of CSF, may predict the response to shunting. response to shunting.

Phases of Dementia

People differ in the speed in which their abilities People differ in the speed in which their abilities deteriorate. Some may change from day to day, deteriorate. Some may change from day to day, while others may decline slowly over a number of while others may decline slowly over a number of years.years.

It is important to remember that not all features will It is important to remember that not all features will be present in every person, nor will every individual be present in every person, nor will every individual go through every stage. go through every stage.

Early Stage Dementia2-4 years

This stage often becomes apparent in hindsight. It may be impossible to identify the exact time it began.

Appear more apathetic.Appear more apathetic. Problems with word findingProblems with word finding Lose interest in hobbies or activities.Lose interest in hobbies or activities. Unwilling to try new things.Unwilling to try new things.

Difficulty adapting to changes.Difficulty adapting to changes. IndecisiveIndecisive Take longer with routine jobs.Take longer with routine jobs.

Forgetful about details of recent events.Forgetful about details of recent events. Likely to repeat themselves.Likely to repeat themselves. May respond to loss of independence with irritability, hostility, May respond to loss of independence with irritability, hostility,

and agitation.and agitation.

Intermediate Stage Dementia2-10 years

Problems are more apparent and disabling

Very forgetful about recent events. Very forgetful about recent events. Confuse one family member with another.Confuse one family member with another. Forget names of friends.Forget names of friends. Neglectful of hygiene, eating, or attire.Neglectful of hygiene, eating, or attire.

Easily disoriented as they miss social and environmental cues.Easily disoriented as they miss social and environmental cues. Tend to get lost if away from familiar surroundings. Tend to get lost if away from familiar surroundings. Risk of falls and accidents increase substantially.Risk of falls and accidents increase substantially.

Become easily distressed when frustrated. Become easily distressed when frustrated. Restlessness and aggression may occur due to confusion, Restlessness and aggression may occur due to confusion,

particularly at night (particularly at night (Sundowning EffectSundowning Effect). ). Sleep patterns are often disorganized.Sleep patterns are often disorganized.

Late Stage Dementia1-3 years1-3 years

Requires total careRequires total care

Unable to remember information, even for a few minutes.Unable to remember information, even for a few minutes. Lose their ability to understand and use speech.Lose their ability to understand and use speech. Become immobile and incontinent. Become immobile and incontinent.

Show no recognition of friends and family. Show no recognition of friends and family. Fail to recognize everyday objects. Fail to recognize everyday objects.

End-stage dementia results in coma and death, usually due to End-stage dementia results in coma and death, usually due to immune system compromise.immune system compromise.

InterventionsInterventions

Two Types of Anti-Dementia Medications Two Types of Anti-Dementia Medications

1.1.Actelycholinesterase inhibitors are intended to Actelycholinesterase inhibitors are intended to preserve functioning (i.e., delay worsening) and usually prescribed functioning (i.e., delay worsening) and usually prescribed for mild for mild to moderate symptomsto moderate symptoms..

These include Cognex, Aricept, and Exelon.These include Cognex, Aricept, and Exelon.

2.2. Other medications regulate glutamate Other medications regulate glutamate to treat moderate to severe to treat moderate to severe symptoms of Alzheimer’s, such as problems performing simple symptoms of Alzheimer’s, such as problems performing simple tasks.tasks.

These include Namenda.These include Namenda.

There is evidence that some individuals taking an There is evidence that some individuals taking an acetylcholinesterase inhibitor might also benefit from being acetylcholinesterase inhibitor might also benefit from being

prescribed a glutamate regulator. prescribed a glutamate regulator.

InterventionsIndividuals with Dementia are Highly Sensitive to their Environment Individuals with Dementia are Highly Sensitive to their Environment

Provide clear, calm, and comforting structure and routine. Provide clear, calm, and comforting structure and routine.

Changes in surroundings and people should be explained simply to Changes in surroundings and people should be explained simply to avoid distressing reactions. avoid distressing reactions.

Rooms should be reasonably bright and contain sensory stimuli to Rooms should be reasonably bright and contain sensory stimuli to reinforce orientation.reinforce orientation.

Regularly engage in low-stress activities.Regularly engage in low-stress activities.

Redirect with distractions and substitutions. Redirect with distractions and substitutions. Be flexible. Be flexible.

Always use soothing and reassurance.Always use soothing and reassurance.

PreventionPrevention

It is impossible to stop aging. But, there are many things that improve health as one ages. For instance:

Eating well: Meet with a dietitian and use the Food Guide “Plate” to choose healthy food.

Exercising: Have a doctor or therapist create a special exercise program.

Keeping the mind active: Participate in activities that encourage thinking.

Seeing the physician for regular check-ups and for special screenings and examinations.