Introduction Questions and Answers

1. IntroductionQuestions and Answers Dr Janice Bothwell Consultant Paediatrician Belfast HSCT

2. Objectives • Role of Paediatrician in management of children with hemiplegia • Multidisciplinary team • Some questions parents have when told their child has a hemiplegia • Other questions?

3. What is the role of the doctor ? • A Paediatrician is a doctor who specialises in the care of children. • Neurodevelopmental Paediatricians specialise in the care of children with developmental disorders and/or medical conditions such as hemiplegia which can affect the development of skills in a growing child • Neurologists specialise in children who present with neurological conditions such as hemiplegia

4. Assessment process • Unidisciplinary • Multidisciplinary

5. Medical Assessment • History of pregnancy, birth, family history, significant illnesses, any injuries sustained, medications taken, developmental progress, educational history • Physical examination - examination of heart, lungs, abdomen, central and peripheral nervous system, hearing and vision - height and weight

6. Developmental Assessment • Gross Motor Skills • Fine Motor Skills • Speech and Language – ability to both speak and understand language • Personal and social skills • Cognitive ability

7. Child development team Speech & Language therapist Dentist Doctor Child Physiotherapist Occupational therapist Social Worker

8. Investigations • Neuroimaging • Blood investigations • EEG • Hearing tests • Vision tests

9. Diagnosis - Hemiplegia • Congential • Acquired

10. What questions do parents ask when their child is diagnosed with hemiplegia?

11. What does hemiplegia mean? Paralysis of one side of the body resulting from disease or injury to the motor centres of the brain.

12. My child has hemiplegia does this mean he/she has cerebral palsy? • Cerebral palsy is an umbrella term covering a group of non-progressive but often changing motor-impairment syndromes secondary to anomalies or lesions in the brain • These anomalies or lesions can be prenatal/perinatal/postnatal in origin • Hemiplegic CP is classified as a unilateral motor disability

13. What is a Congenital Hemiplegia? • A congenital hemiplegia is one which occurs as a result of a prenatal/perinatal or early postnatal problem (<28 days after birth).

14. What went wrong? • Three main processes can occur, each occur at different developmental stages during pregnancy/birth - cerebral malformations originating early in the pregnancy - periventricular lesions mainly arising during 24 – 34 weeks (either during pregnancy or after a preterm birth) - cortical infarctions occurring just before or around the time of a term birth

15. What does a normal brain look like?

16. What are cerebral malformations? • All malformations originate from early fetal life • Neuronal migration disorder - agyria or lissencephaly at early stage - schizencephaly and pachygyria at later stage - heterotopias and polymicrogyria at later stages (up to 30 weeks gestation)

17. What do these conditions look like on MRI? lissencephaly schizencephaly polymicrogyria

18. What are periventricular lesions? • During the period between 24th and 34th week of gestation the brain is very vulnerable to bleeding • As a result periventricular leukomalacia or periventricular haemorrhagic infarctions can develop which may result in a congenital hemiplegia

19. What does this look like on a scan?

20. How can this cause hemiplegia? A simplified diagram to show how the corticspinal tracts can be affected by PVL and hydrocephalus

21. What are cortical infarctions? • Cortical infarctions occur around term and are caused by ischaemia (lack of oxygen and/or blood flow to part/parts of brain tissue). • This is actually the least common type

22. What does this look like?

23. My child has a congenital hemiplegia – why were there no signs when he/she was born? • Clinical signs of a hemiplegia may not be evident until the child is old enough to use the affected limb. • The child may show signs such as being slower to crawl, pull to stand, walk or may develop hand dominance at a very early age.

24. What else can you tell me about congenital hemiplegia? • This affects about 4 babies per 10,000 • Its more common in males 1.4:1 • R sided hemiplegia is more common

25. What is an acquired hemiplegia? • An acquired hemiplegia is a weakness which develops in a child who has previously been well • Acute • Chronic progressive

26. What is an acute hemiplegia? • This is a sudden onset of one sided weakness which can be associated with seizures

27. What causes an acute hemiplegia? • An acute hemiplegia can have a number of causes. • A brief outline of some of the causes will be given

28. What is an alternating hemiplegia? • This is a hemiplegia which appears and any time from birth to 54 months. • The initial signs are mild delay in your child’s development • This may be followed by abnormal positioning of your child’s limbs or weakness of one side and can be associated with jerking movements of the eyes

29. How long does the hemiplegia last for? • In alternating hemiplegia the duration varies from minutes to days and the seriousness of symptoms can vary. • The side affected with weakness can alternate and the arm is usually more affected than the leg. • Hemiplegia is also noted to disappear with sleep and reappear on wakening

30. Will a child recover from alternating hemiplegia? • After each episode the child may recover to a degree, but after multiple episodes the child may have learning difficulties and possibly lose some skills • Medications can be used with various degrees of success

31. My child has been diagnosed as having a stroke why? • There are many reasons why a child will have a stroke which results in a hemiplegia. • These reasons can include clotting problems, heart disease, trauma, infections, vascular problems, tumours

32. What investigations will doctors do to identify why? • Investigations include multiple blood investigations, urine sampling, heart scans, brain scans

33. What do strokes look like on a scan?

34. Can infection cause hemiplegia? • An acute hemiplegia can be caused by brain infections such as meningitis or encephalitis • Infection which causes hemiplegia is also usually associated with seizures

35. What causes progressive hemiplegias? • Progressive hemiplegias can be caused by • AV malformations • Brain abscesses • Tumours • Demyelinating disease • Sturge Weber syndrome

36. What is Sturge Weber syndrome?

37. What are the signs of Sturge Weber syndrome? • Port wine stain on face (cutaneous angioma) • Epilepsy in 80% • Hemiplegia evident in 50% affecting limbs on side opposite to facial signs

38. We have a diagnosis of hemiplegia what other problems may be present? • Children with hemiplegia may also have • Learning disability (1 in 5 children) • Epilepsy present in 25 to 33% of children • Vision: significant problems rare but squints and refraction errors are common • Hearing most often normal • Speech is well preserved

39. Who should be involved with my child after diagnosis? Voluntary agencies Paediatrician Orthopaedic surgeon Neurologist Child Occupational therapist Physiotherapist Educational Psychologist

40. How do we treat hemiplegia? • Main treatment aims are • Child will walk and aims of treatment are to maximise efficiency and look of walking • The arm can be very often ignored by the child so early awareness of its use and encourage use of this limb is important. This should be combined with improvement of efficiency and look • Monitor spine

41. Is early treatment important? • Yes to ensure • Greater symmetry of posture and movement • Develop an awareness of affected side • Prevention of fixed deformity • Provide support for families

42. Are there any surgical or medical treatment available? • Yes surgical treatments are offered by the orthopaedic surgeons to correct deformities or to improve limb function • Medical treatment includes treatment for spasticity (tight muscles) this includes baclofen, diazepam • Botulinum toxin now used as injection form to help spastic limbs

43. What other therapeutic options are there? • Numerous – need to look at evidence base for effectiveness

44. Any other questions ????