dr valerie orr consultant in paediatric neurodisability rhsc, yorkhill
TRANSCRIPT
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Dr Valerie OrrConsultant in Paediatric Neurodisability
RHSC, Yorkhill
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Objectives
To be able to identify abnormal motor development
To develop understanding of common motor disorders
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Does early detection matter?Parents value early diagnosisImproved outcomeImproved quality of life for child and familyAccess to educational and social services
‘Early detection is of little value unless parents subsequently experience a well-organised service with a clear referral pathway to definitive diagnosis and management’
Ref: Health for all Children 4th Edition (Hall 4)
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How do we identify children with abnormal development?
Follow-up of ‘high risk’ infants
ScreeningListening to parentsOpportunistic
recognition
Ref. Hall 4
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Gross motor milestones – when to refer
Head control 4 monthsSits unsupported 9 monthsStands independently 12 monthsWalks independently 18 months
*Remember to adjust for prematurity until 2yrs
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Features that may suggest underlying motor disorder
Delayed motor milestonesAsymmetrical movement patternsAbnormalities of muscle tonePersisting primitive reflexesOther difficulties
e.g. feeding difficulties
unexplained irritabilityrespiratory problems
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Worrying signs / Red flags
Not reaching & grasping objects by 6 months
Hand preference before 1 year Hypertonicity
e.g. closed hand posture, extensor posturing, scissoring
HypotoniaLoss of previously acquired motor skills at
any age
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Floppy infant – assessment
HistoryPregnancy and birthFeedingDevelopment
ExaminationWeight & OFC ?thrivingFacial features ?
dysmorphismMovement ?antigravity
mvts
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Floppy infant - causes
Prematurity, illness & drugs - transient hypotonia‘Benign congenital hypotonia’Global developmental delayEvolving cerebral palsyGenetic syndromes
e.g. Downs, Prader-Willi syndrome
Neuromuscular problems (rare!)e.g. congenital myotonic dystrophy,
spinal muscular atrophy
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Clinical scenario
A mother brings her 18mth old son to thesurgery with a minor illness. She mentions
thatshe is concerned that he is not yet walking.
What particular points would you look for in the history and examination?
What action might you take?
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Delayed walking (>18mths)
Normal variantsAssociated with bottom shufflingCerebral palsy or minor neurological
problems <10%Consider CK in boys
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Toe walking
CausesIdiopathic toe walkingMuscle spasticity
e.g. cerebral palsy, spinal cord lesion
Muscle disease e.g. Duchenne muscular dystrophy, Charcot Marie Tooth
Treatments depend on causee.g. physiotherapy, casting, orthotics, surgery
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Cerebral Palsy: a multi-system disorderDescription not diagnosis!
Primarily a motor disorder
Other impairments often associatedVision CognitionHearing FeedingManual dexterity SeizuresSpeech & language Behaviour problems
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Aetiology of cerebral palsy
Incidence ~2 per 1000 live birthsAntenatal ~70%
e.g. prematurity, fetal & neonatal stroke, brain malformations, maternal infection
Perinatal 10-15%e.g. neonatal encephalopathy
Postneonatal events ~15%e.g. trauma, meningoencephalitis, stroke
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Cerebral Palsy: InterventionsAimsMaximise potentialPrevention of secondary
dysfunctionPromotion of improved function
and participation in society
Examples of tone managementOrthoticsBotulinum toxinOral medications e.g. Baclofen Intrathecal baclofenOrthopaedic surgery
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Duchenne Muscular DystrophyX-linked disorderHigh rate of new mutationsIncidence 1 in 3500 male live birthsMutation of dystrophin gene Xp218-10% of female carriers have some
manifestations of diseaseLoss of ambulation at mean age of 9yrsAverage life expectancy now mid-20s
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Duchenne muscular dystrophy: Diagnosis
Check CK in boys:not walking by 18 months4-6 months behind in general
development at 2 yearsawkward or clumsy gait under 4 yearsunable to run or jump by 4 yearspainful hips or legs under 4 years
Ref. Mohamed K et al. Delayed diagnosis of Duchenne muscular dystrophy.
Eur J Pediatr Neurol 2000
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Duchenne muscular dystrophy: ManagementMultidisciplinary team approachUse of steroids
Prolongation of ambulationReduction in complications e.g. scoliosisImprovement in respiratory function
Increased use of non-invasive ventilationCardiac surveillance every 2yrsSpinal surgery
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Developmental Coordination DisorderMale : Female 3 : 1
Impaired motor control & planningdifficulties with dressing and toiletingmessy feedingpoor handwriting and drawing skillspoor ball skills
Can become socially isolated
Poor self esteem and schooling difficulties
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Developmental Coordination Disorder:DSM-IV diagnostic criteriaMarked impairment of the development of motor
co-ordination
Impairment significantly interferes with academic achievement and activities of daily living
Problem not due to a recognised medical condition
Not a pervasive developmental delay
NHS QIS publication: ‘I still can’t tie my shoelaces...’Quick Reference Guide to Identification and Diagnosis of DCD
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Developmental Coordination Disorder: Management
Reassure the child that there is no medical disease process or refer to Paediatrician for assessment
Referral to Occupational Therapist
Classroom support
Group interventions to promote motor skills and self-esteem e.g. Rainbow Gym
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Summary‘Limit ages’ can guide need for referral
Neurological examination should identify worrying signs
Be alert to motor disorders that might present later in childhood
Listen and respond to parents concerns!
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Useful sources of information
From Birth to Five Years. Mary Sheridan.Health for All Children 4th Edition. Hall D. Developmental delay: Identification and
management. Aust Fam Phys 2005, Vol 34; 9:739-742
Voluntary organisations e.g. Hemihelp, SCOPE, Contact a Family
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Developmental Coordination Disorder: useful references
‘I still can’t tie my shoelaces...’Quick Reference Guide to Identification and Diagnosis of DCDwww.healthcareimprovementscotland.org/our_work/reproductive,_maternal__child/programme_resources/dcd_review_response.aspx
Why every office needs a tennis ball: a new approach to assessing the clumsy child Cheryl Missiuna et al. CMAJ August 29, 2006; 175 (5)
www.cmaj.ca/content/175/5/471.full