Presentation on theme: "The Many Faces of Cerebral Palsy"— Presentation transcript:
1 The Many Faces of Cerebral Palsy Dr Emeka EjelioguLecturer/Consultant Paediatric NeurologistUniversity of Jos/Jos University Teaching Hospital
2 What is cerebral palsyCerebral 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.
3 Prevalence in developed countries Prevalence rate is 1-3 per 1000 childrenIn USA:About 764,000 children and adults currently have cerebral palsyAbout 500,000 children under age of 18 currently have cerebral palsyAbout 2-3 children out of every 1,000 have cerebral palsyAbout 10,000 babies born each year will develop cerebral palsy
4 Prevalence in developing countries No population based studies in Nigeria & most other developing countriesCouper 2002 in Kwazulu, S.A reported a prevalence rate of 28 per 1000 childrenIf 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
5 Causes CP is caused by a broad group of problems that occur during: PregnancyLabour and deliveryAfter birthThe problem could affect:The motherThe foetusThe child
6 During pregnancy Maternal Foetal Maternal infections Chorioamnionitis Antepartum haemorrhageMaternal drug abuseTrauma to the abdomenAbdominal irradiationCongenital malformations of the brainOther congenital anomalies esp cardiovascularCongenital infectionsCord accidents
7 During labour & delivery maternalInfantProlonged obstructed labourPrecipitate labourInstrumental deliverySevere birth asphyxiaPrematurityMultiple birthIntracranial haemorrhageBirth traumaNeonatal jaundiceHypoglycaemiaMeningitis/Encephalitis
8 After birth Meningitis Encephalitis Head injury Thromboembolism Severe shockSickle cell stoke
9 Causes...2 In Nigeria the commonest causes are: Severe birth asphyxia Severe jaundicePrematurityMeningitis
10 Classifications CP may be classified in different ways. The most acceptable classifications are the International and the functional classifications which both classify CP into 4International classificationSpastic CPAthetoid CPAtaxic CPMixed CP
11 Classification 2 Functional classification Class I-no limitation of activityClass II-slight to moderate limitationClass III-moderate to great limitationClass IV-no useful physical activity
12 Signs and Symptoms of Cerebral Palsy Muscle toneMovement coordination and controlReflexesPostureBalanceFine motor functionGross motor functionOral motor function
13 Muscle tone Hypotonia Hypertonia Dystonia Mixed Muscle spasms Fixed jointsAbnormal neck or truncal toneClonus
14 Movement coordination SpasticAthetoid or dyskinetic movementsAtaxic movementsMixed movementsGait disturbances
15 Balance Requiring both hands for support Having difficulty balancing when not using hands for supportUnable to sit without using hands for supportSwaying when standingUnsteady when walkingDifficulty making quick movementWalking with abnormal gait
16 Gross motor functionImpaired 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.
17 Fine motor function Grasping small objects Holding objects between thumb and forefingerSetting objects down gentlyUsing crayonsTurning pages in a book
18 Oral motor functionSpeakingSwallowingFeeding/chewingDrooling
19 Associated Disabilities CP may be associated with a spectrum of developmental disabilities, includingIntellectual disabilitySeizure disorderVisual defectHearing impairmentSpeech defectCognitive dysfunctionBehavioural problemsADHD
20 Early identificationAppropriate 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 reflexesMovement of the limbs: limited movement of one side of the body/hand dominance before 1yearEducating 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
21 Goal of treatment Optimize mobility Manage primary conditions Control painPrevent and manage complications, and associated conditionsMaximize independenceEnhance social and peer interactionsFoster self-careMaximize communicationMaximize learning potentialEnhance quality-of-life
22 TreatmentA multidisciplinary team provide important contributions to the treatment of children with CP. These include:Physicians from various specialtiesOccupational and physical therapistsSpeech therapistsSocial workers/counsellorsSpecial educatorsDevelopmental and clinical psychologistsCounselling is very important as soon as the diagnosis is madeFamily support is crucial
23 PrognosisLong 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 disabilitiesLevel of motivation of the familyFamily, community & social supportType & intensity of facilities available for rehabilitative intervention
24 Prevention Well supervised pregnancy and safe delivery Appropriate investigations during pregnancyEarly identification of high risk pregnanciesWell trained birth attendantsEfficient resuscitation of asphyxiated babiesGood referral systemNewborn examination and screeningGood newborn care including intensive carePrompt diagnosis and appropriate management of NNJPrompt diagnosis and appropriate treatment of meningitis
25 Other diagnostic considerations Developmental delayPhysical disabilityBehavioural problemDevelopmental disabilityNeurodegenerative problem
26 Developmental delay Constitutional delay in development Social smile 6-8 weeksNeck control 2-3 monthsSit without support 4-6 monthsCrawl monthsWalk months
27 Physical disabilities Muscular dystrophies: Duchenne/BeckerInherited as an X-linked recessive trait-boys usually affectedAbsence of dystrophin, a cytoskeletal protein encoded on X chromosomeHypertrophy of the calvesProgressive muscle weaknessProliferation of connective tissue in muscle.Intellectual impairmentLoss of ambulation by 7-10 yearsContractures and scoliosis are common
28 Behavioural problem Attention deficit hyperactivity disorder (ADHD) Inattention and difficulty sustaining attentionincreased distractibilitypoor impulse control and decreased self-inhibitory capacitymotor overactivityacademic underachievementPoor interpersonal relationships with family members and peersEvidence suggest that genetic and environmental factors play significant role during foetal and postnatal development in the evolution of ADHD
29 Developmental disability Down syndromeAutism spectrum disorderAutismAsperger syndromeIntellectual disability
30 Down syndrome Presence of 3 chromosome 21 instead of 2 Occurs as a result of non-dysjunction in one parentIncidence increases with increasing maternal agePresents withHypotoniaFlat face, low-set earsUpward and outward slanted palpebral fissuresVarying degrees of intellectual disability and growth retardationCardiac malformationsSingle transverse palmer crease (simian crease)
31 Autism spectrum disorder (ASD) A neurodevelopmental disorder characterized by:Impaired social interaction.poor eye contact,little symbolic play,limited joint attentionImpaired verbal and non-verbal communicationreliance on non-verbal communication with delay in use of wordsecholaliaRestricted, repetitive or stereotyped behavior.Stereotypical body movementsa marked need for samenessa very narrow range of interests
32 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 factorsIndication to proceed with evaluation for ASDNo 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 typeAsperger syndrome is a milder type with relative preservation of language and intellectual function
33 Intellectual disability Intellectual capacity that is so impaired that there is need for special care or special educationIQ test is used to determine if a child has intellectual disabilityCommon causes of intellectual disability includegenetic syndromeschromosomal abnormalitiesfoetal deprivationprematurityperinatal insultsintrauterine exposure to drugs of abuse
34 Intellectual disability...2 Mildcan function as an independent adult if well adjusted, he can be educated but most will require special education.Moderateis trainable, he can learn to read and write but needs close supervision. The well adjusted ones can function semi-independently in a sheltered workshop.Severemay be able to protect himself against physical danger, however he is not trainableProfoundunable to protect himself against physical danger, cannot speak more than a few words, may require care similar to that needed for a baby
35 Neurodegenerative problems Inborn errors of metabolismRett syndrome
36 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.
37 Inborn errors of metabolism Common ones:GalactosaemiaGlycogen storage diseasePhenylketonuriaFeaturesDevelopmental delaySeizuresMuscle weaknessHypertoniaIntellectual disability
38 Rett syndromeA 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.
39 Rett syndrome Features similar to CP Hypotonia Delayed or absent ability to walkGait/movement difficultiesAtaxiaAbnormally small headSpasticityChoreaDystonia
40 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.
41 Coping tipsLove 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.
42 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.
43 ConclusionCerebral 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.
44 Courtsey of noahsmiracle.blogspot.com Thank YouCourtsey of noahsmiracle.blogspot.com