The Many Faces of Cerebral Palsy

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The Many Faces of Cerebral Palsy

• Dr Emeka Ejeliogu
• Lecturer/Consultant Paediatric Neurologist
• University of Jos/Jos University Teaching Hospital

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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.

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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

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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

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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

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During pregnancy

• Maternal

• Foetal

• Maternal infections
• Chorioamnionitis
• Antepartum haemorrhage
• Maternal drug abuse
• Trauma to the abdomen
• Abdominal irradiation

• Congenital malformations of the brain
• Other congenital anomalies esp cardiovascular
• Congenital infections
• Cord accidents

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During labour delivery

• maternal

• Infant

• Prolonged obstructed labour
• Precipitate labour
• Instrumental delivery

• Severe birth asphyxia
• Prematurity
• Multiple birth
• Intracranial haemorrhage
• Birth trauma
• Neonatal jaundice
• Hypoglycaemia
• Meningitis/Encephalitis

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After birth

• Meningitis
• Encephalitis
• Head injury
• Thromboembolism
• Severe shock
• Sickle cell stoke

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Causes...2

• In Nigeria the commonest causes are
• Severe birth asphyxia
• Severe jaundice
• Prematurity
• Meningitis

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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
• Spastic CP
• Athetoid CP
• Ataxic CP
• Mixed CP

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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

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Signs and Symptoms of Cerebral Palsy

• Muscle tone
• Movement coordination and control
• Reflexes
• Posture
• Balance
• Fine motor function
• Gross motor function
• Oral motor function

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Muscle tone

• Hypotonia
• Hypertonia
• Dystonia
• Mixed
• Muscle spasms
• Fixed joints
• Abnormal neck or truncal tone
• Clonus

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Movement coordination

• Spastic
• Athetoid or dyskinetic movements
• Ataxic movements
• Mixed movements
• Gait disturbances

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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

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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.

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Fine motor function

• Grasping small objects
• Holding objects between thumb and forefinger
• Setting objects down gently
• Using crayons
• Turning pages in a book

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Oral motor function

• Speaking
• Swallowing
• Feeding/chewing
• Drooling

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Associated Disabilities

• CP may be associated with a spectrum of
  developmental disabilities, including
• Intellectual disability
• Seizure disorder
• Visual defect
• Hearing impairment
• Speech defect
• Cognitive dysfunction
• Behavioural problems
• ADHD

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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 childrens 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

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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

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Treatment

• A multidisciplinary team provide important
  contributions to the treatment of children with
  CP. These include
• 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

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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
• Other associated disabilities
• Level of motivation of the family
• Family, community social support
• Type intensity of facilities available for
  rehabilitative intervention

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Prevention

• 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

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Other diagnostic considerations

• Developmental delay
• Physical disability
• Behavioural problem
• Developmental disability
• Neurodegenerative problem

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Developmental delay

• Constitutional delay in development
• Social smile 6-8 weeks
• Neck control 2-3 months
• Sit without support 4-6 months
• Crawl 7-9 months
• Walk 12-18 months

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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

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Behavioural problem

• Attention deficit hyperactivity disorder (ADHD)
• 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

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Developmental disability

• Down syndrome
• Autism spectrum disorder
• Autism
• Asperger syndrome
• Intellectual disability

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Down syndrome

• 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
• 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)

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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

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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
• 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

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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
• genetic syndromes
• chromosomal abnormalities
• foetal deprivation
• prematurity
• perinatal insults
• intrauterine exposure to drugs of abuse

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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

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Neurodegenerative problems

• Inborn errors of metabolism
• Rett syndrome

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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.

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Inborn errors of metabolism

• Common ones
• Galactosaemia
• Glycogen storage disease
• Phenylketonuria
• Features
• Developmental delay
• Seizures
• Muscle weakness
• Hypertonia
• Intellectual disability

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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.

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Rett syndrome

• Features similar to CP
• Hypotonia
• Delayed or absent ability to walk
• Gait/movement difficulties
• Ataxia
• Abnormally small head
• Spasticity
• Chorea
• Dystonia

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The way forward

• 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.

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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,
  dont 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.

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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.

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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.

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Thank You