2. Introduction
• It is a static encephalopathy defined as a non-
progressive disorder of posture and
movement resulting from a lesion/defect of
the developing brain.
• It may be associated with epilepsy
abnormalities of speech, vision and intellect.
• Causes are commonly prenatal, natal and
postnatal.
8. GMFCS Levels
I: Walks indoors & outdoors without limitation. Some difficulties in complex
(running, jumping) gross motor activities. Difficulties with uneven surfaces.
II: Walks indoors without assistive mobility device. Climbs stairs holding railing.
Unable to run or jump.
III: Walks with an assistive mobility device on level surfaces, climbs stairs with
adult assistance. Frequently transported when traveling for long distances or
outdoors.
IV: May walk short distances with a walker and supervision, with difficulty turning
& maintaining balance on uneven surfaces. Transported in the community.
May achieve self-mobility using a power wheelchair.
V: Physical impairments restrict voluntary movement. All areas of motor function
limited. No means of independent mobility.
11. Clinical manifestations
• The symptoms vary significantly but in most
cases
• They are essentially those of upper motor
neuron lesions.
• Decreased spontaneous activities
• A child < lyr will demonstrate hand preference
• Delayed walking may be a feature
12. Clinical manifestations contd.
• Spastic hemiplegia may have have circumductive
gait
• Growth arrest in extremities
• Tip-toeing activity
• Commando crawls is common in spastic diplegia.
• The mother may experience difficulty applying
diaper
• When suspended, there is demonstration of
scissoring posture.
14. Clinical manifestations contd
• Spastic quadriplegia is the most severe form.
• It is often associated with mental
retardation, hypereflexia and hypertonia.
• Flexion contraction seen at knee in late
childhood.
• Speech abnormality is common.
15. Clinical manifestations contd
• In Athetoid CP; poor head control, feeding
difficulty, tongue thrust, drooling of saliva
and Slurred speech are features.
• There is impairment of voice modulation due
to involvement of oropharyngeal muscles.
17. Diagnosis
• A thorough history and physical examination.
• An MRI scan of the brain is indicated to
determine the location and extent of
structural lesions or associated congenital
malformations; an MRI scan of the spinal
cord is indicated if there is any question
about spinal cord pathology.
18. Diagnosis
• Additional studies may include tests of
hearing and visual function.
• Genetic evaluation should be considered in
patients with congenital malformations
(chromosomes) or evidence of metabolic
disorders.
19. Treatment
• Because CP is usually associated with a wide
spectrum of developmental disorders, a
multidisciplinary approach is most helpful in the
assessment and treatment of such children.
• Treatment is multidisciplinary, co-ordinated by
the Paediatrician. Other members of the team
include Occupational therapist, Speech
pathologist, Social worker, Special Educator,
Developmental Psychologists, Physical therapist,
Understanding and caring parents.
20. Drugs useful in Cerebral Palsy
• For those with spasticity, useful drugs include
benzodiazepine, dantrolin Na, Intrathecal
Baclofen.
• Currently being researched for severe
spasticity is bolitilium toxin. (BOTOX)
• Levodopa is useful in those incapacitating
athethosis.
• carbamazepine is useful in Dystonia.
23. GLOBAL DEVELOPMENTAL DELAY
• It is a subset of developmental disabilities
defined as significant delay in 2 or more of
the following developmental domains:
–Gross/fine motor
–Speech/language
–Social/personal
–Activities of daily living
24. • Significant delay is defined as performance
that is 2 standard deviation or more below the
mean.
• The term global developmental delay is
reserved for younger children (under 5 years)
while the term mental retardation is usually
applied to older children among whom IQ
testing is more valid and more reliable.
26. Introduction
• It is a condition of both medical and social
importance defined as significantly
subaverage general intellectual functioning
which is seen during developmental period,
characterized by inadequacy of adaptive of
behaviour.
27. • The diagnosis depends on
– IQ below 2 standard deviation of mean
– manifestation before 18 years of age
– presence of maladaptive behaviour
• IQ is calculated as Mental age x 100
Chronological age
28. Aetiology
• In general terms:
• There are two overlapping populations of
children with intellectual disability:
• Mild/moderate mental retardation (IQ >50),
which is more associated with environmental
influences; and Severe mental retardation (IQ
<50), which is more frequently linked to
biologic causes.
29. • Mild mental retardation is four times more likely
to be found in the offspring of women who have
not completed high school than in women who
have graduated.
• This is presumably a consequence of both
genetic (children may inherit an intellectual
impairment) and socioeconomic (poverty,
undernutrition) factors.
• The specific causes of mild mental retardation
are currently identifiable in <50% of affected
individuals.
30. • The most common biologic causes of mild
mental retardation include genetic
syndromes with multiple minor congenital
anomalies, fetal deprivation, prematurity,
perinatal insults, intrauterine exposure to
drugs of abuse, and sex chromosomal
abnormalities.
• Familial clustering is frequent.
31. • In children with severe mental retardation, a
biologic cause (most commonly prenatal) can
be identified in >75% of cases.
• Causes include chromosomal (Down
syndrome) and other genetic syndromes
(fragile X syndrome), abnormalities of brain
development (lissencephaly), and inborn
errors of metabolism/neurodegenerative
disorders (mucopolysaccharidoses).
32. Causes of Mental Retardation
• Chromosomal abnormalities such as
klinefelter syndrome, Praeder Willi, Fragile x
syndrome.
• Developmental brain abnormalities such as
hydrocephalus
• Inborn errors of metabolism
• Congenital infections
• Hypoxic Ischaemic Encephalopathy
• Trauma, meningitis, metabolic disorders such
as hypothyroidism amongst others.
33. Clinical Manifestations
• Most children with intellectual disability 1st
come to the Paediatrician's attention in
infancy because of dysmorphisms, associated
dysfunctions, or failure to meet age-
appropriate developmental milestones.
• There are no specific physical characteristics
of intellectual disability, but dysmorphisms
are the earliest signs that bring children to
the attention of the pediatrician.
34. • They may comprise a genetic syndrome such
as Down syndrome or be isolated, as in
microcephaly.
• Associated dysfunctions are neurologic
disorders (seizures, cerebral palsy, autism)
that are seen more frequently in conjunction
with mental retardation than in the general
population.
35. • Most children with intellectual disability do
not keep up with their peers and fail to meet
age-expected norms.
• In early infancy, failure to meet age-
appropriate expectations may include a lack
of visual or auditory responsiveness, unusual
muscle tone (hypo- or hypertonia) or
posture, and feeding difficulties.
36. • Between 6 and 18 months of age, motor
delay (lack of sitting, crawling, walking) is the
most common complaint.
• Language delay and behaviour problems are
common concerns after 18 month.
• Earlier identification of atypical development
is likely to occur with more severe
impairments; mental retardation is usually
identifiable by age 3 yr.
37. AGE vs AREA OF CONCERN
• Newborn …… Dysmorphic syndromes,
microcephaly, Major organ system
dysfunction (e.g., feeding and breathing)
• Early infancy (2–4 month)………..Failure to
interact with the environment, Concerns
about vision and hearing impairments
• Later infancy (6–18 month)………..Gross
motor delay
38. AGE vs AREA OF CONCERN
• Toddlers (2–3 yr)………….Language delays or
difficulties
• Preschool (3–5 yr)…………Language difficulties or
delays, behavior difficulties, including play,
delays in fine motor skills: cutting, coloring,
drawing
• School age (>5 yr)……… Academic
underachievement, behavior difficulties
(attention, anxiety, mood, conduct, and so on)
39. Classification
IQ Educational
Terminology
Psychosocial
Terminology
Usual Time of
Diagnosis
Academic
Potential
70-84 Slow learner Borderline Increasing
academic
difficulties
Eventually
complete
school with
help
55-69 Educable Mild MR Nursery school Primary school
40-54 Trainable Moderate MR Pre-school ,
when there is
delayed
developmental
milestone
Can be taught
survival words,
self help, social
and vocational
skills.
25-39, <25 Sub-trainable Severe MR /
Profound
Infancy and
Early
childhood
Needs
assistance even
in self help.
40. Evaluation of a child with mental
retardation
• Indepth history
• Detailed physical examination
• Basic tests to include visual and hearing
evaluation
• Neuromaging maybe necessary.
• Chromosomal studies and hormonal assays
especially the thyroids assay.
41. • The diagnosis of mental retardation is however
clinical involving the determination of Intelligence
Quotient using specified tools such as:
• Bayley Scales of Infant Development ,
• Wechsler Scales,
• Vineland Adaptive Behavior Scale (VABS),
• Woodcock-Johnson Scales of Independent Behavior
• American Association on Mental Retardation
Adaptive Behavior Scale (ABS),
• “Draw –a-man test”
42. • Ziler ‘draw a man’ test has been validated for
use in Nigerian children by Izuora and Ebigbo.
• It requires pencil and paper with a simple
instruction to the child to draw a person.
44. Severity of mental retardation and
adult-age functioning.
• Mild……………………………………………… 9-11yrs
• Moderate ……………………….…………….6-8yrs
• Severe………………………………………….. 3-5yrs
• Profound……………………………………… <3yrs
45. PREVENTION
• Increasing public awareness of the adverse
effect of drugs of abuse in pregnancy.
• Adequate antenatal care
• Prevention of early teenage pregnancy
• Keeping medicines and potential poisons far
from reach of children
• Promote early prenatal care
• Promote immunization
46. COMPLICATIONS
• Children with mental retardation have higher
rates of vision, hearing, orthopedic, and
behavioral/emotional disorders than do
typically developing children.
• These and some other problems are
identified later in children with mental
retardation.
47. • If untreated, the associated impairments can
potentially adversely affect the individual's
outcome more than the intellectual disability
does.
