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1
Understanding and Treating Dementia
(Neurocognitive Disorders)in Intellectual
Disability
2
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
  admissions.
• 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).

3
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 Alzheimers
  disease.
•  
• Overall, the age-related prevalence of dementia
  in persons with intellectual disability is
  similar to the general population.

4
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.

5
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.

6
Brain Regions
7
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.

8
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.

9
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
  minutes
• List names of objects within categories (animals,
  foods, furniture)
• Screening tests may include the MMSE, Cognistat,
  RBANS, and DRS.

10
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.

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Differential DiagnosisFalse-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?

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PseudodementiaDepressive Disorder

• Depression may be the first sign of early stage
  dementia.
• Prevalence of major depressive disorder in people
  with dementia is falls between 6 to 20.
  Dementia can cause brain changes that lead to
  depression.
• 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.

13
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
  disinhibited.
• 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.

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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
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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
  activities
• For example, requires assistance in areas that
  were previously independent.

16
Mild Neurocognitve Disorder

• Modest 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 do not interfere with the
  capacity for independence in everyday activities.

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Dementia Classifications

• DSM-5 Major or Minor Neurocognitive Disorder due
  to
• 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

18
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.

19
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.

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LEWY BODY DEMENTIA

• 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
  Alzheimers
• 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.

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FRONTOTEMPORAL DEMENTIAFormerly Picks Disease

• 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.

22
HYDROCEPHALUS

• 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.

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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.

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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.
• 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.

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Intermediate Stage Dementia2-10 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
  surroundings.
• Risk of falls and accidents increase
  substantially.
• Become easily distressed when frustrated.
• Restlessness and aggression may occur due to
  confusion, particularly at night (Sundowning
  Effect).
• Sleep patterns are often disorganized.

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Late Stage Dementia1-3 yearsRequires total care

• Unable to remember information, even for a few
  minutes.
• 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.

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Interventions

• 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.

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Interventions

• Individuals with Dementia are Highly Sensitive to
  their Environment
• Provide clear, calm, and comforting structure and
  routine.
• 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.

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Prevention

• 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.