The Many Faces of Cerebral Palsy

Dr Emeka EjelioguLecturer/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 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

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

After birth

• 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• 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

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

Muscle tone

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

Movement coordination

• 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

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

Oral motor function

• Speaking• Swallowing• Feeding/chewing• Drooling

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

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:Physicians from various specialties Occupational and physical therapists Speech therapistsSocial 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:– Other associated disabilities– Level of motivation of the family– Family, community & social support– Type & intensity of facilities available for rehabilitative

intervention

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

Other diagnostic considerations

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

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

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)– 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• 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)

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

– 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

– 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– 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– Hypotonia– Delayed or absent ability to walk – Gait/movement difficulties – Ataxia– Abnormally small head – Spasticity– Chorea – Dystonia

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.

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