Transcript The Many Faces of Cerebral Palsy - Benola

The Many Faces of Cerebral Palsy
Dr Emeka Ejeliogu
Lecturer/Consultant Paediatric Neurologist
University of Jos/Jos University Teaching Hospital
What is cerebral palsy
• Cerebral palsy (CP) is a chronic motor disorder involving
posture and/or movement that results from a non-progressive
injury to the developing brain.
• CP is not a progressive disorder but it usually evolves over
time, however it may appear to be progressive especially to
concerned and worried parents and other family members.
• CP is not curable because the brain neurones affected by the
injury cannot be regenerated.
• However if a child with CP is identified early and early
intervention instituted, the child could attain his/her full
potential and function at a high educational and vocational
level.
Prevalence in developed countries
Prevalence rate is 1-3 per 1000 children
In USA:
 About 764,000 children and adults currently have
cerebral palsy
 About 500,000 children under age of 18 currently have
cerebral palsy
 About 2-3 children out of every 1,000 have cerebral
palsy
 About 10,000 babies born each year will develop
cerebral palsy
Prevalence in developing countries
• No population based studies in Nigeria & most other
developing countries
• Couper 2002 in Kwazulu, S.A reported a prevalence
rate of 28 per 1000 children
• If we assume a prevalence rate of 28 per 1000 in
Nigeria, and a population of 160m then as many as
2m children may be living with CP in Nigeria
Causes
• CP is caused by a broad group of problems
that occur during:
• Pregnancy
• Labour and delivery
• After birth
• The problem could affect:
• The mother
• The foetus
• The child
During pregnancy
Maternal
• Maternal infections
• Chorioamnionitis
• Antepartum haemorrhage
• Maternal drug abuse
• Trauma to the abdomen
• Abdominal irradiation
Foetal
• Congenital malformations
of the brain
• Other congenital anomalies
esp cardiovascular
• Congenital infections
• Cord accidents
During labour & delivery
maternal
• Prolonged obstructed
labour
• Precipitate labour
• Instrumental delivery
Infant
• Severe birth asphyxia
• Prematurity
• Multiple birth
• Intracranial haemorrhage
• Birth trauma
• Neonatal jaundice
• Hypoglycaemia
• Meningitis/Encephalitis
After birth
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Meningitis
Encephalitis
Head injury
Thromboembolism
Severe shock
Sickle cell stoke
Causes...2
• In Nigeria the commonest causes are:
– Severe birth asphyxia
– Severe jaundice
– Prematurity
– Meningitis
Classifications
• CP may be classified in different ways.
• The most acceptable classifications are the
International and the functional classifications which
both classify CP into 4
• International classification
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Spastic CP
Athetoid CP
Ataxic CP
Mixed CP
Classification 2
• Functional classification
– Class I-no limitation of activity
– Class II-slight to moderate limitation
– Class III-moderate to great limitation
– Class IV-no useful physical activity
Signs and Symptoms of Cerebral Palsy
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Muscle tone
Movement coordination and control
Reflexes
Posture
Balance
Fine motor function
Gross motor function
Oral motor function
Muscle tone
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Hypotonia
Hypertonia
Dystonia
Mixed
Muscle spasms
Fixed joints
Abnormal neck or truncal tone
Clonus
Movement coordination
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Spastic
Athetoid or dyskinetic movements
Ataxic movements
Mixed movements
Gait disturbances
Balance
• Requiring both hands for support
• Having difficulty balancing when not using hands for
support
• Unable to sit without using hands for support
• Swaying when standing
• Unsteady when walking
• Difficulty making quick movement
• Walking with abnormal gait
Gross motor function
• Impaired gross motor functions – limited capability
of accomplishing common physical skills such as
crawling, walking, running, jumping, and maintaining
balance.
• Delayed gross motor functions – physical skills
developed later than expected.
Fine motor function
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Grasping small objects
Holding objects between thumb and forefinger
Setting objects down gently
Using crayons
Turning pages in a book
Oral motor function
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Speaking
Swallowing
Feeding/chewing
Drooling
Associated Disabilities
CP may be associated with a spectrum of developmental
disabilities, including
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Intellectual disability
Seizure disorder
Visual defect
Hearing impairment
Speech defect
Cognitive dysfunction
Behavioural problems
ADHD
Early identification
• Appropriate follow-up of children with known risk factors like
Neonatal jaundice, Severe Birth Asphyxia, Prematurity, and
Meningitis.
• Newborn examination with emphasis on general activity
(floppiness, hyperalert), poor feeding, and seizure.
• Physical examination with the following in mind:
– Persistence of primitive reflexes
– Movement of the limbs: limited movement of one side of the
body/hand dominance before 1year
• Educating mothers on children’s developmental milestones and
asking them to seek medical care whenever they notice any
delay.
• Routine assessment of developmental milestones during
immunization and well-infant clinic visits.
• Training of healthcare worker on early identification of signs and
symptoms of CP
Goal of treatment
Optimize mobility
Manage primary conditions
Control pain
Prevent and manage complications, and
associated conditions
Maximize independence
Enhance social and peer interactions
Foster self-care
Maximize communication
Maximize learning potential
Enhance quality-of-life
Treatment
A multidisciplinary team provide important
contributions to the treatment of children with
CP. These include:
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Physicians from various specialties
Occupational and physical therapists
Speech therapists
Social workers/counsellors
Special educators
Developmental and clinical psychologists
Counselling is very important as soon as the
diagnosis is made
Family support is crucial
Prognosis
• Long term outcome depends on the severity of the
motor disability; and the presence & severity of
cognitive dysfunction.
• Other factors that determine the prognosis include:
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Other associated disabilities
Level of motivation of the family
Family, community & social support
Type & intensity of facilities available for rehabilitative
intervention
Prevention
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Well supervised pregnancy and safe delivery
Appropriate investigations during pregnancy
Early identification of high risk pregnancies
Well trained birth attendants
Efficient resuscitation of asphyxiated babies
Good referral system
Newborn examination and screening
Good newborn care including intensive care
Prompt diagnosis and appropriate management of NNJ
Prompt diagnosis and appropriate treatment of meningitis
Other diagnostic considerations
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Developmental delay
Physical disability
Behavioural problem
Developmental disability
Neurodegenerative problem
Developmental delay
• Constitutional delay in development
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Social smile
Neck control
Sit without support
Crawl
Walk
6-8 weeks
2-3 months
4-6 months
7-9 months
12-18 months
Physical disabilities
• Muscular dystrophies: Duchenne/Becker
– Inherited as an X-linked recessive trait-boys usually
affected
– Absence of dystrophin, a cytoskeletal protein encoded on
X chromosome
– Hypertrophy of the calves
– Progressive muscle weakness
– Proliferation of connective tissue in muscle.
– Intellectual impairment
– Loss of ambulation by 7-10 years
– Contractures and scoliosis are common
Behavioural problem
• Attention deficit hyperactivity disorder (ADHD)
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Inattention and difficulty sustaining attention
increased distractibility
poor impulse control and decreased self-inhibitory capacity
motor overactivity
academic underachievement
Poor interpersonal relationships with family members and
peers
– Evidence suggest that genetic and environmental factors
play significant role during foetal and postnatal
development in the evolution of ADHD
Developmental disability
• Down syndrome
• Autism spectrum disorder
– Autism
– Asperger syndrome
• Intellectual disability
Down syndrome
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Presence of 3 chromosome 21 instead of 2
Occurs as a result of non-dysjunction in one parent
Incidence increases with increasing maternal age
Presents with
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Hypotonia
Flat face, low-set ears
Upward and outward slanted palpebral fissures
Varying degrees of intellectual disability and growth
retardation
– Cardiac malformations
– Single transverse palmer crease (simian crease)
Autism spectrum disorder (ASD)
• A neurodevelopmental disorder characterized by:
• Impaired social interaction.
• poor eye contact,
• little symbolic play,
• limited joint attention
• Impaired verbal and non-verbal communication
• reliance on non-verbal communication with delay in use of words
• echolalia
• Restricted, repetitive or stereotyped behavior.
• Stereotypical body movements
• a marked need for sameness
• a very narrow range of interests
Autism spectrum disorder...2
• The diagnostic criteria require that symptoms become
apparent before a child is three years old.
• Cause is multifactorial- combination of genetic and
environmental factors
• Indication to proceed with evaluation for ASD
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No babbling by 12 months.
No gesturing (pointing, waving, etc.) by 12 months.
No single words by 16 months.
No two-word phrases by 24 months.
Any loss of any language or social skills, at any age.
• Autism is the most severe type
• Asperger syndrome is a milder type with relative preservation
of language and intellectual function
Intellectual disability
• Intellectual capacity that is so impaired that there is need for
special care or special education
• IQ test is used to determine if a child has intellectual disability
• Common causes of intellectual disability include
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genetic syndromes
chromosomal abnormalities
foetal deprivation
prematurity
perinatal insults
intrauterine exposure to drugs of abuse
Intellectual disability...2
• Mild
– can function as an independent adult if well adjusted, he can be
educated but most will require special education.
• Moderate
– is trainable, he can learn to read and write but needs close
supervision. The well adjusted ones can function semi-independently
in a sheltered workshop.
• Severe
– may be able to protect himself against physical danger, however he is
not trainable
• Profound
– unable to protect himself against physical danger, cannot speak more
than a few words, may require care similar to that needed for a baby
Neurodegenerative problems
• Inborn errors of metabolism
• Rett syndrome
Inborn errors of metabolism
• A large group of genetic diseases involving disorders
of metabolism.
• The majority are due to defects of single genes that
code for enzymes that facilitate conversion of various
substances (substrates) into others (products).
• In most of the disorders, problems arise due to
accumulation of substances which are toxic or
interfere with normal function, or to the effects of
reduced ability to synthesize essential compounds.
Inborn errors of metabolism
• Common ones:
– Galactosaemia
– Glycogen storage disease
– Phenylketonuria
• Features
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Developmental delay
Seizures
Muscle weakness
Hypertonia
Intellectual disability
Rett syndrome
• A rare genetic postnatal neurological disorder of the
grey matter of the brain that almost exclusively
affects females.
• Caused by mutations in the gene MECP2 located on
the X chromosome.
• Initial development is normal.
• Onset occurs between 6 and 18 months of age.
• A period of developmental stagnation is followed by
developmental regression involving language and
motor milestones.
Rett syndrome
• Features similar to CP
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Hypotonia
Delayed or absent ability to walk
Gait/movement difficulties
Ataxia
Abnormally small head
Spasticity
Chorea
Dystonia
The way forward
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Get a diagnosis from appropriate professionals.
Get informed (separate fact from fiction and myths).
Get counselling.
Identify local interventions.
Get involved or start a support group.
Determine severity, do needs assessment.
Determine clear indications and goals of alternative
therapies.
• Include parents in any intervention which they should be
able to incorporate into their everyday lives.
Coping tips
• Love your child--Focus first on the fact that this is your child
and second on their special need.
• Allow time for grief--It is a normal process, don’t bottle-up
emotions.
• Play with your child by getting down to his level so that he
can really interact with you.
• Get therapy -- speech, occupational, physical, and educational
-- as soon as possible.
• When professionals work with your child, learn as much as
you can from them and how you can implement some of the
techniques they use to challenge your child.
Take care of yourselves
• The stress of balancing career and family -- and balancing the
needs of a child with disabilities with the needs of other
children -- can also take a toll.
• Maintain your friendships and social activities.
• Plan fun activities with the whole family.
• Schedule special “alone time” with your partner/spouse or a
close friend.
• Hire a babysitter or respite caregiver to give yourself a break.
• Read uplifting books written by other caregivers of children
with special needs.
• Focus on the present instead of fretting about the future.
Conclusion
• Cerebral palsy may have many faces and could be
confused with different clinical conditions.
• For children with CP to achieve their maximum
potential and have an independent good quality life,
we need to have appropriate diagnosis, early
identification, and early intervention.
• We all are stakeholders in their care and we have to
be advocates for the needs of children with CP and
their families.
Thank You
Courtsey of noahsmiracle.blogspot.com