Understanding and Treating Dementia (Neurocognitive Disorders) in Intellectual Disability

1. Understanding and Treating Dementia (Neurocognitive Disorders)in Intellectual Disability Tolisano DDS December 2013

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 Alzheimer’s 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 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.

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.

11. Differential DiagnosisFalse-Positives • Deliriumis 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?

12. 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 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.

14. Telling the Difference between Dementia and Age-Related Cognitive Decline

15. 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 andstandardized 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 • Modestdecline from previous level of cognitive functioning: • Complex Attention, Executive Functions, Memory, Language, Motor Abilities or Social Skills • Based on collateral information including self-report andstandardized neuropsychological testing or quantified clinical assessment. • Cognitive deficits do not interfere with the capacity for independence in everyday activities.

17. Dementia Classifications • DSM-5: Major or Minor Neurocognitive Disorder due to… • Types: • Alzheimer’s vs. Non-Alzheimer’s • Vascular • Lewy Body • Frontotemporal • Hydrocephalus • Traumatic Brain Injury • Substance/Medication-Induced • Prion (Transmittable Disease) • Parkinson’s and Huntington’s • Multiple Etiologies • Unspecified • Cortical or Subcortical • Common or Rare

18. Dementia of the Alzheimer’s Type • Biochemical problems inside brain cells from abnormal proteins called amyloid plaques and neurofibrillary tangles. • Most common cause of dementia. Accounts for > 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.

20. 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.

21. FRONTOTEMPORAL DEMENTIAFormerly Pick’s 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.

23. 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.

24. Early Stage Dementia2-4 yearsThis 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.

25. Intermediate Stage Dementia2-10 yearsProblems 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.

26. 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.

27. Interventions Two Types of Anti-Dementia Medications • Actelycholinesterase inhibitors are intended to preservefunctioning (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 Alzheimer’s, 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.

28. 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.

29. 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.