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
Approximately 80,500 individuals age five and over in
Ontario have a developmental disability (2009)
16,000 individuals above 45 years of age.
“the number and proportion of older people with intellectual
disability is increasing and will continue to do so until after the baby
boom generation moves into later life in 2012.”
Uncertain Prevalence of Developmental Disabilities Among Older Adults in the CRD,
2007 and 2018
Projected
Prevalence
0.4%
Prevalence
Difference
Age
Range
Year
65+
2007
63,922
255
319
64
2018
89,240
357
446
89
2007
226, 838 907
1,134
227
2018
241,267
1,206
241
45-65
Population
0.5%
965
The aging and disability service systems have historically
developed in parallel but separate tracks ...
… despite often overlapping concerns about issues such as
affordable housing, public transportation, access to
healthcare, long-term care needs, and economic stability.
As a result, the geriatric care system lacks the disabilities-specific skills
and experience needed to care for older adults with developmental
disabilities.
A recurrent theme in the literature is that while the disabilities care
system and the geriatric care system share many common concerns,
neither is well equipped to provide services to older adults with
developmental disabilities.
The former lacks skills and experience with geriatric care; the latter lacks
skills and experience with disabilities.
LIFE EXPECTANCY AND AGING IN
DEVELOPMENTAL DISABILITIES
1.
LIFE EXPECTANCY WAS LOW IN THE 1920s.
2.
For Down’s, it was in the early 20s.
3.
A large number were in institutions.
4.
Cause of death was usually Bronchopneumonia.
TODAY, LIFE EXPECTANCY IS AROUND
67 YEARS OF AGE.
5.
U.S. data suggest that the mean age at which people with developmental disabilities die was
 66 years in 1993
 59 years in the 1970s
 19 years in the 1930s
 Among people with less severe disabilities who do not have Down’s syndrome, life
expectancy is now approaching that of the general population.
On the basis of changes in life expectancy and the
aging of the baby boom generation, U.S. studies
suggest that …
…the total population of people with
developmental disabilities aged 55 and
over will double by 2030.
Some conditions occur in people with developmental disabilities more often than in the general
population, for example,
 thyroid disorders,
 heart disorders,
 sensory impairments.
 the rate of psychiatric problems among the older people with developmental disabilities is two to four
times the rate in other older people.
Other age-related health issues are more frequent in people with particular syndromes.
The best known are :
 the increased risk of precocious aging, dementia, and increased sensory loss in people
with Down’s syndrome.
 adults with Down syndrome have a higher prevalence (15% to 40%) of early-onset Alzheimer’s disease
occurring 15-20 years earlier compared to the general population,
 may experience hypothyroidism and sleep apnea more frequently (McCarron, Gill, McCallion, & Begley,
2005).
 osteoporosis in women with cerebral palsy due to
long-term inactivity
 osteoporosis in people with epilepsy due to their
use of medications
 bladder and swallowing problems in people with
cerebral palsy
 mitral valve prolapse and musculoskeletal
disorders in people with Fragile X syndrome
 Adults with Fragile X may have more issues with
heart problems (mitral valve prolapse),
musculoskeletal disorder, earlier menopause,
epilepsy, and visual problems.
 diabetes, cardiovascular disease, and obesity in
people with Prader-Willis syndrome
As persons with cerebral palsy age, they have >>>







an increased likelihood of having reduced mobility,
bone demineralization,
fractures,
decreased muscle tone and
increased pain,
difficulty eating or swallowing,
and bowel and bladder concerns (White-Scott, 2007).
 People aging with cerebral palsy and epilepsy who use psychotropic and anti-seizure
medications on a long-term basis also have a higher risk of developing osteoporosis
(brittle bone disease) and tardive dyskinesia (repetitive, involuntary, purposeless
movements caused by the long-term use of certain drugs).
This risk is often compounded by limited physical activity and diets
low in calcium and vitamin D.
Anti-epileptic medications are also frequently given long-term to
individuals with developmental disabilities.
Studies suggest that osteoporosis and osteomalacia (softening of the
bones) are potential side effects of certain antiepileptic medication,
and vitamin D may be reduced, leading to possible loss of bone
mass.
AGING
in Developmental Disability
1.
2.
3.
4.
As in the general population, aging brings the following
problems:
PHYSICAL PROBLEMS
Cardiovascular disease
Musculo-skeletal disease
Gastro-intestinal problems
Sensory problems
Caution:
 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.
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 Anxiety disorders.
14 Mood instability
15 Executive function deficits
13 Memory and Attention difficulties
Contd:
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.
Psycho-Social Aspects: :
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
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.
In the older brain
Scaffolds are invoked
To perform
even
familiar tasks and basic cognitive processes
In the older
 Scaffolds (wider net works) are
recruited
even for low levels of task demand
(Ex: remembering where one put the car
keys)
In the older
 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;)
Executive Functions
Inhibit
Shift
Emotional Control
Monitor
Working Memory
Plan/ organize
Organization of Materials
Task Completion
EXECUTIVE FUNCTIONS

B
R
I
E
F
 (1) the brain shrinks in volume and the ventricular system expands in healthy aging.
However, the pattern of changes is highly heterogeneous, with the largest changes
seen in the frontal and temporal cortex, and in the putamen, thalamus, and
accumbens.

(2) The volumetric brain reductions in healthy aging are likely only to a minor extent
related to neuronal loss. Rather, shrinkage of neurons, reductions of synaptic spines, and
lower numbers of synapses probably account for the reductions in grey matter. In
addition, the length of myelinated axons is greatly reduced, up to almost 50%.

(3) Reductions in specific cognitive abilities--for instance processing speed,
executive functions, and episodic memory--are seen in healthy aging. Such
reductions are to a substantial degree mediated by neuroanatomical changes, meaning
that between 25% and 100% of the differences between young and old participants in
selected cognitive functions can be explained by group differences in structural brain
characteristics.

Several studies have suggested that
cognitive deficits in normal aging arise from alterations in the
functional integration of these coordinated brain systems

Altered fronto-occipital connectivity has also been observed with increasing
age (Moeller et al., 1996),
 Fronto-parietal connectivity has been identified as
important in memory function
 alteration in patterns of functional connectivity
during memory tasks in older adults.
Notably, many long-range
connections between

fronto-temporal,

fronto-occipital,

fronto-parietal

temporal–parietal regions
were found to be impaired.
Aging & Neural Connections in
Autism
 Frontal and Temporal development are 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
Lobes of the Brain
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
Neural Changes with aging
Orbitofrontal:






Disinhibition
Lability
Irritability
Impulsivity
Sexual preoccupation
Distractability
 May go unrecognized
Lobes of the Brain
Neural Changes with aging
Ventromedial PC:
 Decreased verbal output
 Diminished motor initiation
 Withdrawal
 apathy
Lobes of the Brain
Neural Changes with Aging
Dorsomedial PC:
 Apathy
 Akineticmutism
 incontinence
Lobes of the Brain
Neural Changes with Aging
Dorsolateral PC:
 Working memory



Spatial
Object-faces
Verbal
 Executive functions
 Language sequencing
Neural Changes with Aging Frontal
lobe:
Dysfunction results in:








Disinhibition
Emotional lability
Irritability
Lack of drive, motivation
Deficits in memory
Attentional deficits
Apathy – akinesia – Abulia
Aphasia
Neural Changes with Aging
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
Neural Changes with Aging
Parietal:
 Dominant:


Alexia, agraphia, acalculia
Agnosis, left-right disorientation
 Non-dominant:




Impaired spatial ability
Anosognosia
Autopagnosia
Apraxia, etc.
Neural Changes with Aging
Occipital:
 Disturbed spatial orientation (metamorphopsia)
 Visual illusions
 Visual hallucinations, etc.
CASE HISTORY - I
 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
 DEPRESSION AS DEMENTIA
CASE HISTORY - II
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
 DEMENTIA AS DEPRESSION
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
MULTIFACTOR
CHALLENGES
I) Bio-Medical Conditions
II) Psychiatric Conditions
III) Developmental Disorders
IV) Emotional Issues
V) Sensory Processing Issues
VI) Environmental Factors
VII ) Communication Difficulties
VII) Learned Behaviour
. Bio-Medical Conditions
Check
A)
B)
C)
D)
E)
F)
G)
H)
Tonic/Clonic, generalized
Tonic/Clonic,
Focal generalized
Focal
Absence
Complex partial
Lennaux-Gestaut
Other

A)
B)
C)
D)
E)
F)
G)
H)
I)
J)
K)
Heart disease
Lung disorders (asthma etc.)
GI Disorders (GERD, hernias)
Allergies
Endocrinal disorders
Food sensitivities
Constipation
Arthritis
Chronic/acute pain
Ear infections
Diabetes

A)
B)
C)
D)
E)
F)
G)
Head injury
Meningitis
Encephalitis
Migraine
Stroke
Movement disorders
other

A)
B)
C)
D)
E)
F)
cataracts
retinal damage
Glaucoma
Blindness
Poor vision
Depth vision problem

A)
B)
C)
D)
A)
B)
C)
D)
E)
F)
Loss of hearing
Tinnitus
Hyperacusis
deafness
initiating and maintaining
hypersomnias
Sleep-Wake
Apnea
Narcolepsy
Other
A)
B)
C)
D)
E)
F)
G)
Akathesia
Abnormal involuntary movement disorders
Constipation
Drooling
Dystoni
Dry mouth
other
Epilepsy
Medical Disorders
Neurological problems
Vision problems
Hearing Problems

Sleep Disorders

Medication related
Specify

V.
SENSORY PROCESSING ISSUES
Check

28
A)
B)
C)
Oversensitive to touch
Under sensitive to touch
Difficulty in tactile discrimination

A)
B)
Sensory seeking
Difficulties in grading movements

A)
B)
Oversensitive to sounds
Under sensitive to sounds

A)
B)
C)
D)
Poor co-ordination
Poor muscle tone
Oversensitive to movement
Under sensitive to movement

A)
B)
Oversensitive to visual input
Under sensitive to visual input

A)
B)
Oversensitive to Oral input
Under sensitive to Oral input

A)
B)
Oversensitivity to smells
Under sensitivity to smells
Tactile function problems
29
Proprioceptive Dysfunction
30
Auditory function problems
31
Vestibular Dysfunction
32
Visual Function problems
33
Oral Function Problems
34
Olfactory function problems.
Rating*
Specify
1
never
2
3
some significant
1
2
3
1
2
3
1
2
3
1
2
3
1
2
3
1
2
3
Auditory-Language Processing Difficulties

Internal Regulation Difficulties.

Self-Regulation Difficulties

Impaired Social interactions

Emotional Regulation difficulties

VII
47
48
COMMUNICATION
DIFFICULTIES
Specify

Speech problems
a) Dysfluency
b) Articulation problems
c) Voice disorders

Language disorders
a) Aphasia (expressive,
receptive, anomic,
global)
b) Receptive language
deficit
c) Expressive language
deficit
d) Improper use of words,
meaning
e) Inappropriate
grammatical use
f) Reduced vocabulary
Rating*
1
never
1
2
3
some significant
2
3

Auditory Processing
Disorders
a) Difficulty with multi step
instructions
b) Poor listening skills
c) Difficulty attending to
auditory information
d) Difficulty reading,
comprehension
ENVIRONMENTAL FACTORS
Changes in environment
Stressful work/living environment
Care provider skill/competency deficit
Conflictual Home environment
Legal, Financial difficulties
INVESTIGTIONS
 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
 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-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
 End of slide show.
 Extra material follows leading to neurobiology of aging
presentation (70 + slides)
0
3m
2y
ADULT
30 + yrs
 Specifically, more differences were observed in dorsal
frontal and parietal regions with relatively few
differences observed in cortices of the temporal and
occipital lobes.