COMMUNITY NETWORKS OF SPECIALIZED CARE

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COMMUNITY NETWORKS OF SPECIALIZED CARE

• www.community-networks.ca

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AGING IN DEVELOPMENTAL DISABILITIES

• DR.JAY RAO
• M.B.B.,S. ,D.P.M. ,M.R.C.PSYCH(U.K.).,
  F.R.C.P.(C).
• ASSOCIATE PROFESSOR
• UNIVERSITY OF WESTERN ONTARIO

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LIFE EXPECTANCY AND AGING IN DEVELOPMENTAL
DISABILITIES

• LIFE EXPECTANCY WAS LOW IN THE 1920s.
• For Downs, it was in the early 20s.
• A large number were in institutions.
• Cause of death was usually Bronchopneumonia.
• TODAY, LIFE EXPECTANCY IS AROUND
• 67 YEARS OF AGE.

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Context of aging

• Developmentally Disabled

• General population

• there are declines in speed of processing,
  working memory, inhibitory functions, long term
  memory, decreases in brain structure and white
  matter integrity (Parks, Reuter-Lorenz)
• Medical morbidity, health and nutritional
  risks increase
• Psycho-social problems gather force
  

• There may be pre-existing cognitive problems
• Pre-existing Health and nutrition problems
• Pre-existing psycho-social problems

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Three Factors to be considered in aging

• Neuro-medical vulnerabilities
• Neuro-developmental issues
• Ex Scaffolding
• Neuro-Executive Issues

• Developmentally Disabled at higher risk for these
• DD at disadvantage due to developmental
  immaturity of brain architecture
• Pre-existing executive brain dysfunction

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Neuro-developmental issues--- Scaffolding

• In the younger brain
• specialization of circuitry
• Ex Remembering, working memory tasks,
  Novel tasks
• In response to challenges, initially, a wider
  set of neural circuits are recruited.
• These are Scaffolds
• As the task is over-learned, a specific,
  honed circuit is developed.
• This provides the ability for efficient
  cognitive operations

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In the older brain - Firstlygtgt

• Scaffolds are invoked even to perform familiar
  tasks and basic cognitive processes
• Ex (working memory tasks)
• Young
• focal, left Para-hippocampal
  activation
• Old
• Wider Right and left pre-frontal
  brain activation

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In the older secondlygtgtgt

• Scaffolds (wider net works) are recruited
• even for low levels of task demand (remembering
  where one put the car keys)

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In the
older thirdly gtgtgt

• Generating scaffolds and recruiting them is even
  more inefficient
• because of aging pathology

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In the older Developmentally
disabled
we propose

• Scaffolding, even in younger ages is
  inefficient
• There is impaired ability to recruit
• Pre-frontal networks, especially bilaterally

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• In older ages neurobiological decline is rapid or
  more profound in its impact resulting in poor
  scaffolding capacity
• Whatever scaffolding there is , is penetrated by
  neural pathology leading to collapse of the
  scaffolds
• (Parks, Reuter-Lorenz Burke and Barnes)

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Neural Connections in Autism

• Frontal and Temporal development is stunted at an
  early stage leading to lack of differentiation
• This lack of differentiation leads to
  hyper-connectivity
• Blocks coherence development with other critical
  brain regions

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Connectivity problems

• HYPO-connectivity
• Orbito-frontal
• Mixed sensory-motor
• Occipital/Parietal-Temporal
• Frontal-posterior
• Left Intra-hemisphere

• HYPER-connectivity
• Frontal-temporal
• Left Hemisphere intra-hemispheric

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EXECUTIVE FUNCTIONS
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Executive Functions
Inhibit Shift Emotional Control Monitor
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Working Memory Plan/ organize Organization
of Materials Task Completion
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Orbitofrontal

• Disinhibition
• Lability
• Irritability
• Impulsivity
• Sexual preoccupation
• Distractability
• May go unrecognized

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Lobes of the Brain
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Ventromedial PC

• Decreased verbal output
• Diminished motor initiation
• Withdrawal
• apathy

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Lobes of the Brain
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Dorsomedial PC

• Apathy
• Akineticmutism
• incontinence

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Lobes of the Brain
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Dorsolateral PC

• Working memory
• Spatial
• Object-faces
• Verbal
• Executive functions
• Language sequencing

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Caudate-putomen-orbitofrontal

• OCD
• Response bias toward stimuli related to
  socioterritorial concerns about danger, violence,
  hygiene, order, sex mediated by
  orbitofrontal-subcortical circuits
• Inadequate repression (filtering) in caudate of
  input from the orbital cortex (worry)
• Cortex (caudate) globus pallidus

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Frontal lobe

• Dysfunction results in
• Disinhibition
• Emotional lability
• Irritability
• Lack of drive, motivation
• Deficits in memory
• Attentional deficits
• Apathy akinesia Abulia
• Aphasia

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Temporal lobe

• Dominant
• Euphoria
• Auditory hallucinations, illusions
• Thought disorder
• Anterograde amnesia
• Receptive language deficits
• Memory impairment
• Non-dominant
• Dysphoria
• Disinhibition of sexual and aggressive behaviours
• Cognitive difficulties

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Parietal

• Dominant
• Alexia, agraphia, acalculia
• Agnosis, left-right disorientation
• Non-dominant
• Impaired spatial ability
• Anosognosia
• Autopagnosia
• Apraxia, etc.

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Occipital

• Disturbed spatial orientation (metamorphopsia)
• Visual illusions
• Visual hallucinations, etc.

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DOWN SYNDROME AND AGING

• Predilection to early Alzheimers
• However, many questions still not satisfactorily
  answered.
• A) there has been no methodologically
  satisfactory population based study of Downs
• B) No Neuro-pathological confirmation on a
  large enough sample.
• Therefore calculation of the size of the
  problem skewed.

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DOWN SYNDROME AND ALZHEIMERS

• Brains of Downs adults shows Alzheimers like
  organization.
• In most of these, there is no clinical evidence
  of cognitive decline.
• Other conditions mimic Dementia (Depression)
• No comparison of similar IQ bearing syndromal
  groups with Downs.

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Continued

• However, the Incidence and Prevalence of Dementia
  may be higher in Downs.
• But we have no population based data on Incidence
  and prevalence in other Developmentally disabled
  for specific comparison.
• Alzheimers-like brain pathology alone does not
  indicate Alzheimers in Downs.
• Downs, even in their 20s may have such brain
  configuration without actually manifesting any
  clinical decline.

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CASE HISTORY - I

• Depression as Dementia
• 38 yr. old female, admitted with two months
  history of poor memory, disinhibition, emotional
  dyscontrol, incontinence of urine and bowels.
• Worked as a cashier in a store for 12 years
  previously ( job shadowing)
• All investigations normal.
• Mental status exam unproductive

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CASE HISTORY - II

• DEMENTIA AS DEPRESSION
• 67 year old man in a group home, previously well
  functioning, gradually became more withdrawn,
  irritable, forgetful, paranoid, impulsive.
• Did not enjoy activities, became very quiet.
• Treated with anti-psychotics, anti-depressants.
• Became more irritable, rages, Parkinsonian
• Neuro psychological assessment revealed serious
  deficits.
• MRI indicated degenerative changes

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AGING AND Developmental Disability

• As in the general population, aging brings
  the following problems
• PHYSICAL PROBLEMS
• Cardiovascular disease
• Musculo-skeletal disease
• Gastro-intestinal problems
• Sensory problems

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Psychiatric problems

• ( HIGHER INCIDENCE AS ONE GETS OLDER)
• Depression
• Anxiety disorders
• Mood disorders
• Psychosis

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COGNITIVE PROBLEMS

• Slower ability to process information
• Memory problems
• Attention Difficulties
• Executive function deficits (impulsivity, poor
  problem solving ability, difficulty in shifting,
  mood dysregulation)
• Communicational difficulties

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What is the BASE LINE?

• Developmentally disabled may already have
• Epilepsy
• Brain tumors (Tuberous sclerosis)
• Immature, miswired cortex.
• Eye (cataracts) and hearing problems
• Poor articulation, expressive and Receptive
  language problems

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What is the base line?

1. Thyroid problems (ex Downs)
2. Cardiac defects (ex Downs, VCF, Tuberous
   Sclerosis)
3. GI malformations/ Swallowing difficulties
4. Kidney problems (tuberous sclerosis)
5. Skeletal Deformities
6. Lung/Immune deficiencies

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WHAT IS THE BASE LINE?

• Anxiety disorders.
• Mood instability
• Executive function deficits
• Memory and Attention difficulties
• Given such pre-existing conditions, the
  developmentally disabled are more likely to
  decline faster, with aging.
• Often, these are not known because of inadequate
  health
• evaluation.

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Older developmentally disabled experience

• MORE LOSSES AND INCONSISTENCIES WHILE IN CARE
• POORER ACCESS TO MEDICAL FACILITIES
• FINANCIAL HARDSHIPS
• POORER NUTRITION
• LESS ACCESS TO RECREATION AND APPROPRIATE JOB/
  OCCUPATIONAL INVOLVEMENT

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EVALUATION

• MULTIFACTOR EVALUATION is essential
• Careful researching of past medical history and
  family history.
• Multidisciplinary involvement
• Use of structured inventories/rating scales
• BUT REMEMBER
• THESE SCALES ARE NOT DIAGNOSTIC INSTRUMENTS but
  tools to enable management

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INVESTIGTIONS

• CT, EEG,MRI,ULTRA SOUND,X-RAY
• BLOOD WORK THE USUAL
• Neuro-cognitive assessments
• Skills assessments (OT)

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Treatment

• Assessment is the cornerstone
• Treat physical as well as psychiatric issues
• Dementia forms a small proportion of the problems
  in this population
• Physical decline, cognitive difficulties,
  isolation, loneliness, losses, poor nutrition,
  neglected health issues, mood instability are
  more pressing problems in this population

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Aging is a more challenging problem than
dementia

• This is true in the developmentally disabled
  because of the neuro-bio-psycho-social decline.
• As more of the developmentally disabled get
  older, we may need to develop strategies for
  support ,and anticipate the resource implications