Abnormal Motor Development

1. Abnormal Motor Development Dr Valerie Orr Consultant in Paediatric Neurodisability RHSC, Yorkhill

2. Objectives • To be able to identify abnormal motor development • To develop understanding of common motor disorders

3. Does early detection matter? • Parents value early diagnosis • Improved outcome • Improved quality of life for child and family • Access 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)

4. How do we identify children with abnormal development? • Follow-up of ‘high risk’ infants • Screening • Listening to parents • Opportunistic recognition Ref. Hall 4

5. Gross motor milestones – when to refer • Head control 4 months • Sits unsupported 9 months • Stands independently 12 months • Walks independently 18 months *Remember to adjust for prematurity until 2yrs

6. Features that may suggest underlying motor disorder • Delayed motor milestones • Asymmetrical movement patterns • Abnormalities of muscle tone • Persisting primitive reflexes • Other difficulties e.g. feeding difficulties unexplained irritability respiratory problems

7. 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 • Hypotonia • Loss of previously acquired motor skills at any age

8. Floppy infant – assessment History • Pregnancy and birth • Feeding • Development Examination • Weight & OFC ?thriving • Facial features ?dysmorphism • Movement ?antigravity mvts

9. Floppy infant - causes • Prematurity, illness & drugs - transient hypotonia • ‘Benign congenital hypotonia’ • Global developmental delay • Evolving cerebral palsy • Genetic syndromes e.g. Downs, Prader-Willi syndrome • Neuromuscular problems (rare!) e.g. congenital myotonic dystrophy, spinal muscular atrophy

10. Clinical scenario A mother brings her 18mth old son to the surgery with a minor illness. She mentions that she 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?

11. Delayed walking (>18mths) • Normal variants • Associated with bottom shuffling • Cerebral palsy or minor neurological problems <10% • Consider CK in boys

12. Toe walking Causes • Idiopathic toe walking • Muscle spasticity e.g. cerebral palsy, spinal cord lesion • Muscle disease e.g. Duchenne muscular dystrophy, Charcot Marie Tooth Treatments depend on cause e.g. physiotherapy, casting, orthotics, surgery

13. Cerebral Palsy: a multi-system disorder • Description not diagnosis! • Primarily a motor disorder • Other impairments often associated Vision Cognition Hearing Feeding Manual dexterity Seizures Speech & language Behaviour problems

14. Aetiology of cerebral palsy Incidence ~2 per 1000 live births • Antenatal ~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

15. Cerebral Palsy: Interventions Aims • Maximise potential • Prevention of secondary dysfunction • Promotion of improved function and participation in society Examples of tone management • Orthotics • Botulinum toxin • Oral medications e.g. Baclofen • Intrathecal baclofen • Orthopaedic surgery

16. Duchenne Muscular Dystrophy • X-linked disorder • High rate of new mutations • Incidence 1 in 3500 male live births • Mutation of dystrophin gene Xp21 • 8-10% of female carriers have some manifestations of disease • Loss of ambulation at mean age of 9yrs • Average life expectancy now mid-20s

17. Duchenne muscular dystrophy: Diagnosis Check CK in boys: • not walking by 18 months • 4-6 months behind in general development at 2 years • awkward or clumsy gait under 4 years • unable to run or jump by 4 years • painful hips or legs under 4 years Ref. Mohamed K et al. Delayed diagnosis of Duchenne muscular dystrophy. Eur J Pediatr Neurol 2000

18. Duchenne muscular dystrophy: Management • Multidisciplinary team approach • Use of steroids • Prolongation of ambulation • Reduction in complications e.g. scoliosis • Improvement in respiratory function • Increased use of non-invasive ventilation • Cardiac surveillance every 2yrs • Spinal surgery

19. Developmental Coordination Disorder • Male : Female 3 : 1 • Impaired motor control & planning • difficulties with dressing and toileting • messy feeding • poor handwriting and drawing skills • poor ball skills • Can become socially isolated • Poor self esteem and schooling difficulties

20. Developmental Coordination Disorder:DSM-IV diagnostic criteria • Marked 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

21. 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

22. 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!

23. Useful sources of information • From Birth to Five Years. Mary Sheridan. • Health for All Children 4th Edition. Hall D. • Developmental delay: Identification and management. AustFam Phys 2005, Vol 34; 9:739-742 • Voluntary organisations e.g. Hemihelp, SCOPE, Contact a Family

24. Developmental Coordination Disorder: useful references • ‘I still can’t tie my shoelaces...’ Quick Reference Guide to Identification and Diagnosis of DCD www.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