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Understanding and Treating Dementia (Neurocognitive Disorders) in Intellectual Disability


Understanding and Treating Dementia (Neurocognitive Disorders) ... such as Down syndrome who have four times the risk of developing Alzheimer s disease. – PowerPoint PPT presentation

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Title: Understanding and Treating Dementia (Neurocognitive Disorders) in Intellectual Disability

Understanding and Treating Dementia
(Neurocognitive Disorders) in Intellectual
Demographics of Dementia
  • In the general population, dementia affects 5-10
    of those aged 65 to 74, and 40 of those over 85.
  • It accounts for more than 50 of nursing home
  • At least 5 million people in the United States
    are diagnosed with dementia. The term is retained
    with the DSM-5 for continuity.
  • Neurocognitive disorder is now the preferred
    terminology, especially with impairments
    secondary to other conditions that affect younger
    individuals (e.g., TBI).

Relationship between Dementia and Intellectual
  • 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 Alzheimers
  • 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 individuals
    previous or baseline level of functioning.
  • This underscores the importance of establishing
    an individuals premorbid capabilities prior to
    the onset of perceived changes.

Brain Regions
General Signs and Symptoms of Dementia
  • Appreciable disturbance in higher cortical
  • - Memory - Calculation -
  • - Orientation - Language -
  • - 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 Screening for Dementia
  • In the early stages, memory impairment usually
    affects registration, storage, and retrieval of
    new information.
  • In the late stages, older material (e.g.,
    birthplace, names of siblings) may be lost.
  • Short-term memory tests
  • Registering 3 objects and recalling them after 5
  • List names of objects within categories (animals,
    foods, furniture)
  • Screening tests may include the MMSE, Cognistat,
    RBANS, and DRS.

The Basics of Screening for Dementia
  • Diagnosis requires deficits in at least one of
    the following areas
  • Impaired ability to plan, organize, and sequence
  • Issues with thinking abstractly
  • Agnosia Inability to identify objects despite
    intact senses
  • Apraxia Problems with learned activities despite
    intact motor functions
  • Aphasia Impairment in comprehending or
    expressing language
  • Each cognitive deficit must substantially impair
    functioning and represent a significant decline
    from the previous ability level.

Differential Diagnosis False-Positives
  • Delirium is a reversible condition. The features
    are usually inattentiveness and poor awareness.
    The symptoms have a short duration. It can be
    superimposed on dementia.
  • Side-effects to certain medications may mimic or
    worsen symptoms of dementia
  • Antihistamines
  • Benzodiazepines and anticholinergics
  • Tricyclic antidepressants and antipsychotics
  • Other Important Medical Considerations
  • Substances (Intoxication or Withdrawal States)
  • Mixed level of activity
  • Urinary tract infections
  • Renal or liver failure causing toxicity
  • Is the condition better accounted for by another
    medical condition or mental disorder?

Pseudodementia Depressive Disorder
  • Depression may be the first sign of early stage
  • Prevalence of major depressive disorder in people
    with dementia is falls between 6 to 20.
    Dementia can cause brain changes that lead to
  • Those with only depression rarely forget
    important current events or personal matters.
  • Neurologic examinations are normal except low
    motivation or psychomotor slowing.
  • Those with depression make little effort to
    respond, while those with dementia often try
    hard, but respond incorrectly.
  • When depression and dementia coexist, treating
    depression does not fully restore cognition.

Psychiatric Symptoms Associated with Dementia
  • Psychosis hallucinations, delusions, or
    paranoiaoccur 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
  • Anxietythe 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 Dementia Poor judgment and decision making Losing track of the date or the season Difficulty having a conversation Misplacing things and being unable to retrace steps to find them Up to 50 of individuals with mild cognitive impairment develop dementia usually within 3 years Confabulation (i.e., filling in memory gaps with false information) Typical Age-Related Changes Making a bad decision once in a while Forgetting which day it is and then remembering later Sometimes forgetting which word to use Losing things from time to time Slower recall Performance is adequate when given enough time
Major Neurocognitve Disorder
  • Significant decline from previous level of
    cognitive functioning
  • Complex Attention, Executive Functions, Memory,
    Language, Motor Abilities or Social Skills
  • Based on collateral information including
    self-report and standardized neuropsychological
    testing or quantified clinical assessment.
  • Cognitive deficits interfere with everyday
  • For example, requires assistance in areas that
    were previously independent.

Mild Neurocognitve Disorder
  • Modest decline from previous level of cognitive
  • Complex Attention, Executive Functions, Memory,
    Language, Motor Abilities or Social Skills
  • Based on collateral information including
    self-report and standardized neuropsychological
    testing or quantified clinical assessment.
  • Cognitive deficits do not interfere with the
    capacity for independence in everyday activities.

Dementia Classifications
  • DSM-5 Major or Minor Neurocognitive Disorder due
  • Types
  • Alzheimers vs. Non-Alzheimers
  • Vascular
  • Lewy Body
  • Frontotemporal
  • Hydrocephalus
  • Traumatic Brain Injury
  • Substance/Medication-Induced
  • Prion (Transmittable Disease)
  • Parkinsons and Huntingtons
  • Multiple Etiologies
  • Unspecified
  • Cortical or Subcortical
  • Common or Rare

Dementia of the Alzheimers Type
  • Biochemical problems inside brain cells from
    abnormal proteins called amyloid plaques and
    neurofibrillary tangles.
  • Most common cause of dementia. Accounts for gt 65
    of dementias in the elderly.
  • Twice as common in women because they have a
    longer life expectancy.
  • In the early stage, individuals with Alzheimer's
    disease are often better groomed and neater than
    those with other dementias.

  • 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.

  • Cognitive deterioration due to changes in
    cortical neurons.
  • Third most common dementia. Age of onset is
    typically gt 60.
  • Lewy body dementia, Parkinson's disease, and
    Alzheimer's disease overlap considerably.
  • Lewy Body Dementia is differentiated from
  • 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.

  • Hereditary disorders that affect the frontal and
    temporal lobes.
  • Accounts for up to 10 of dementias.
  • Age at onset is typically younger (age 55 to 65).
  • Mainly affects personality and language
  • Behavior becomes disinhibited and repetitive.

  • Characterized by gait disturbance (unsteady
    balance), urinary incontinence, and enlarged
    brain ventricles.
  • This disorder accounts for up to 6 of dementias.
  • Improvements after removal of CSF, may predict
    the response to shunting.

Phases of Dementia
  • People differ in the speed in which their
    abilities deteriorate. Some may change from day
    to day, while others may decline slowly over a
    number of years.
  • It is important to remember that not all features
    will be present in every person, nor will every
    individual go through every stage.

Early Stage Dementia 2-4 years This stage often
becomes apparent in hindsight. It may be
impossible to identify the exact time it began.
  • Appear more apathetic.
  • Problems with word finding
  • Lose interest in hobbies or activities.
  • Unwilling to try new things.
  • Difficulty adapting to changes.
  • Indecisive
  • Take longer with routine jobs.
  • Forgetful about details of recent events.
  • Likely to repeat themselves.
  • May respond to loss of independence with
    irritability, hostility, and agitation.

Intermediate Stage Dementia 2-10 years
Problems are more apparent and disabling
  • Very forgetful about recent events.
  • Confuse one family member with another.
  • Forget names of friends.
  • Neglectful of hygiene, eating, or attire.
  • Easily disoriented as they miss social and
    environmental cues.
  • Tend to get lost if away from familiar
  • Risk of falls and accidents increase
  • Become easily distressed when frustrated.
  • Restlessness and aggression may occur due to
    confusion, particularly at night (Sundowning
  • Sleep patterns are often disorganized.

Late Stage Dementia 1-3 years Requires total care
  • Unable to remember information, even for a few
  • Lose their ability to understand and use speech.
  • Become immobile and incontinent.
  • Show no recognition of friends and family.
  • Fail to recognize everyday objects.
  • End-stage dementia results in coma and death,
    usually due to immune system compromise.

  • Two Types of Anti-Dementia Medications
  • Actelycholinesterase inhibitors are intended to
    preserve functioning (i.e., delay worsening) and
    usually prescribed for mild to moderate symptoms.
  • These include Cognex, Aricept, and Exelon.
  • 2. Other medications regulate glutamate to treat
    moderate to severe symptoms of Alzheimers, such
    as problems performing simple tasks.
  • These include Namenda.
  • There is evidence that some individuals taking an
    acetylcholinesterase inhibitor might also benefit
    from being prescribed a glutamate regulator.

  • Individuals with Dementia are Highly Sensitive to
    their Environment
  • Provide clear, calm, and comforting structure and
  • Changes in surroundings and people should be
    explained simply to avoid distressing reactions.
  • Rooms should be reasonably bright and contain
    sensory stimuli to reinforce orientation.
  • Regularly engage in low-stress activities.
  • Redirect with distractions and substitutions.
  • Be flexible.
  • Always use soothing and reassurance.

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