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SPECIALIZED CARE
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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
LIFE EXPECTANCY AND AGING IN DEVELOPMENTAL
DISABILITIES
1.
2.
3.
4.
5.
LIFE EXPECTANCY WAS LOW IN THE 1920s.
For Down’s, 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.
Context of aging
 General population
 Developmentally Disabled
 there are declines in speed of

There may be pre-existing
cognitive problems

Pre-existing Health and nutrition
problems

Pre-existing psycho-social
problems
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
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
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
In the older brain - Firstly>>
 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
In the older: secondly>>>
 Scaffolds (wider net works) are
recruited
even for low levels of task demand
(remembering where one put the car
keys)
In the older: thirdly >>>
 Generating scaffolds and recruiting them is even
more inefficient
because of aging pathology
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
 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;)
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
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
EXECUTIVE FUNCTIONS
Executive Functions
Inhibit
Shift
Emotional Control
Monitor
Working Memory
Plan/ organize
Organization of Materials
Task Completion
Orbitofrontal:

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Disinhibition
Lability
Irritability
Impulsivity
Sexual preoccupation
Distractability
– May go unrecognized
Lobes of the Brain
Ventromedial PC:

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

Decreased verbal output
Diminished motor initiation
Withdrawal
apathy
Lobes of the Brain
Dorsomedial PC:



Apathy
Akineticmutism
incontinence
Lobes of the Brain
Dorsolateral PC:

Working memory
Spatial
 Object-faces
 Verbal



Executive functions
Language sequencing
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
Thalamus
takes over
Frontal lobe:
Dysfunction results in:
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Disinhibition
Emotional lability
Irritability
Lack of drive, motivation
Deficits in memory
Attentional deficits
Apathy – akinesia – Abulia
Aphasia
Temporal lobe:

Dominant:
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Euphoria
Auditory hallucinations, illusions
Thought disorder
Anterograde amnesia
Receptive language deficits
Memory impairment
Non-dominant:

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Dysphoria
Disinhibition of sexual and aggressive behaviours
Cognitive difficulties
Parietal:

Dominant:
Alexia, agraphia, acalculia
 Agnosis, left-right disorientation


Non-dominant:
Impaired spatial ability
 Anosognosia
 Autopagnosia
 Apraxia, etc.

Occipital:

Disturbed spatial orientation
(metamorphopsia)

Visual illusions

Visual hallucinations, etc.
DOWN SYNDROME AND AGING
1.
Predilection to early Alzheimer’s
2.
However, many questions still not satisfactorily
answered.
A) there has been no methodologically satisfactory
population based study of Down’s
B) No Neuro-pathological confirmation on a large
enough sample.
Therefore calculation of the size of the problem
skewed.
DOWN SYNDROME AND ALZHEIMER’S
 Brains of Down’s adults shows Alzheimer’s 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 Down’s.
Continued:
 However, the Incidence and Prevalence of Dementia may
be higher in Down’s.
 But we have no population based data on Incidence
and prevalence in other Developmentally disabled for
specific comparison.
 Alzheimer’s-like brain pathology alone does not
indicate Alzheimer’s in Down’s.
 Down’s, even in their 20s may have such brain
configuration without actually manifesting any
clinical decline.
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
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
AGING AND Developmental Disability
As in the general population, aging brings the following
problems:
1.
2.
3.
4.
PHYSICAL PROBLEMS
Cardiovascular disease
Musculo-skeletal disease
Gastro-intestinal problems
Sensory problems
Psychiatric problems
( HIGHER INCIDENCE AS ONE GETS OLDER)
1. Depression
2. Anxiety disorders
3. Mood disorders
4. Psychosis
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
What is the BASE LINE?

1.
2.
3.
4.
5.
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
What is the base line?
Thyroid problems (ex: Down’s)
8. Cardiac defects (ex: Down’s, VCF, Tuberous
Sclerosis)
9. GI malformations/ Swallowing difficulties
10. Kidney problems (tuberous sclerosis)
11. Skeletal Deformities
12. Lung/Immune deficiencies
7.
WHAT IS THE BASE LINE?
13
14
15
16
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.
Older developmentally disabled experience:
 MORE LOSSES AND INCONSISTENCIES WHILE IN
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CARE
POORER ACCESS TO MEDICAL FACILITIES
FINANCIAL HARDSHIPS
POORER NUTRITION
LESS ACCESS TO RECREATION AND
APPROPRIATE JOB/ OCCUPATIONAL
INVOLVEMENT
EVALUATION
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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
INVESTIGTIONS
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CT, EEG,MRI,ULTRA SOUND,X-RAY
BLOOD WORK – THE USUAL
Neuro-cognitive assessments
Skills assessments (OT)
Treatment

Assessment is the cornerstone

Treat physical as well as psychiatric issues

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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
Aging is a more challenging problem than
dementia


This is true in the developmentally
disabled because of the neuro-biopsycho-social decline.
As more of the developmentally
disabled get older, we may need to
develop strategies for support ,and
anticipate the resource implications