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 CP Potpourri Edward J. Coll, COL, MC Chief/Director, Developmental Pediatrics Medical Director, WRAMC CP Potpourri Edward J. Coll, COL, MC Chief/Director, Developmental Pediatrics Medical Director, WRAMC EFMP

 Ward Admission Developmental History (Page 1 of 15) • • • • Poverty, Ward Admission Developmental History (Page 1 of 15) • • • • Poverty, homelessness, and neglect, which may also result in infrequency of clinical visits Genetic or physical abnormality Depression in the parent, linked to stress or environment Depression in the child Inexperience in the parent, which may result in the failure to recognise hunger signals from the infant, or inability to breast-feed coupled with inadequate medical and social support Overprotection by the parent, who may respond to a perceived reduction in feeding by using coercive feeding practices Underfeeding by the parent in the case of a child that frequently vomits, to prevent emesis Inattention from the parent, perhaps distracted by other children or events Infants with special needs Prematurity, which may result in difficulties in sucking and in keeping feeds down, and in secondary illness such as bronchopulmonary dysplasia or sepsis Autism leading to tactile sensitivity or sensory defensiveness with oral aversion Intrauterine growth retardation may result in infants who grow normally but never exhibit catch-up growth, thus remaining small for age Infants with difficult temperaments Irregular lifestyle in parents Mothers with eating disorders, such as anorexia nervosa, or body image issues Epidemiology… And furthermore… Yada, yada…

 Ward Admission Developmental History • Gettin’ better r worse • Walkin’ • Talkin’ Ward Admission Developmental History • Gettin’ better r worse • Walkin’ • Talkin’ • Comprehendin’

 ICF: • International Classifications of Functioning, Disability and Health: • Impairment- tissue level ICF: • International Classifications of Functioning, Disability and Health: • Impairment- tissue level • Activity- motor movement • Participation- functional motor event • Societal change-Child Health Q. , others

 Health and Social Outcomes of Children with CP J Ped Aug 2004 S Health and Social Outcomes of Children with CP J Ped Aug 2004 S 36 -S 40 World Health Organization model for International Classification of Functioning, Disability and Health.

 Health and Social Outcomes of Children with CP J Ped Aug 2004 S Health and Social Outcomes of Children with CP J Ped Aug 2004 S 36 -S 40

Developmental Pediatrics spanning the globe Developmental Pediatrics spanning the globe

 • • Pediatric Orthopedists Developmental Pediatricians Physiatrists Ped Physical Therapists Ped Occupational Therapists • • Pediatric Orthopedists Developmental Pediatricians Physiatrists Ped Physical Therapists Ped Occupational Therapists Speech Pathologists Educators Others

 The Big Picture • Research collaboration • Functional status • Quality of Life The Big Picture • Research collaboration • Functional status • Quality of Life • Management ‘controversies”

 The Little Picture • Pathophysiology • Genetic Factors • CNS/neural recovery The Little Picture • Pathophysiology • Genetic Factors • CNS/neural recovery

 Something for Everybody 2007 • • • Gen: Quality of Life!!! GI: G-tubes Something for Everybody 2007 • • • Gen: Quality of Life!!! GI: G-tubes are good, aren’t they? Endo: CP and bone mineral density Pulm: bronchiolitis Adolescent: transition issues Neonatology: outcome studies

FIGURE 3 Weight centiles for boys with CP versus healthy boys Stevenson, R. D. FIGURE 3 Weight centiles for boys with CP versus healthy boys Stevenson, R. D. et al. Pediatrics 2006; 118: 1010 -1018 Copyright © 2006 American Academy of Pediatrics

FIGURE 1 KH centiles for Boys with CP versus healthy boys Stevenson, R. D. FIGURE 1 KH centiles for Boys with CP versus healthy boys Stevenson, R. D. et al. Pediatrics 2006; 118: 1010 -1018 Copyright © 2006 American Academy of Pediatrics

 Du. Pont: CP & Fractures • retrospective review: 156 pts, 178 fractures • Du. Pont: CP & Fractures • retrospective review: 156 pts, 178 fractures • Q 105, D 32, 19 H; 66% non-ambulators • compared pre- vs post- 1992 • compared post-1992 vs age/sex CP controls • pattern, G-tube, seizure meds, ambulatory status, wt/age, wt/ht, osteopenia, surgery, proximity of contractures

 Du. Pont: CP & Fractures • • Post-1992 with greater # fractures 68% Du. Pont: CP & Fractures • • Post-1992 with greater # fractures 68% lower limbs, 62% showed osteopenia 48% delayed Risk Factors: seizure meds, spastic quad. , non-ambulatory, osteopenia • ? more severe patients surviving

 UVa/UNC: CP and Frx • • 306 pts: GMFCS III, IV, V 2 UVa/UNC: CP and Frx • • 306 pts: GMFCS III, IV, V 2 -18 y/o (mean age 9. 3 years) 15. 6% had fractures in past history 19% had fractures subsequently

 UVa/UNC: CP and Frx • • • Children with fractures were; Older (11. UVa/UNC: CP and Frx • • • Children with fractures were; Older (11. 5 vs 8. 9 years) Greater body fat (triceps measurement) Less lean body mass (arm muscle area) No relationship to severity, gender,

 UVa/UNC: CP and Frx • Increased risk – G-tube – prior fracture • UVa/UNC: CP and Frx • Increased risk – G-tube – prior fracture • 24 frx/560 person-years = 4. 3 % • If prior fracture = 9. 5% • G-tube = 6. 5%

 Gross Motor Functional Classification System • Level I – V • Standardized assessment Gross Motor Functional Classification System • Level I – V • Standardized assessment measure – Gross Motor Function Measure • Similar to “growth” curves

 Cerebral Palsy Gross Motor Function Classification System • I: Walk-without restriction – limited Cerebral Palsy Gross Motor Function Classification System • I: Walk-without restriction – limited in more advanced gross motor skills • II: Walks without devices – limited in outdoors and in community • III: Walks with mobility – limited in outdoors and in community

 Cerebral Palsy Gross Motor Function Classification System • IV: Self mobility with limitations Cerebral Palsy Gross Motor Function Classification System • IV: Self mobility with limitations – children are transported or – use power mobility outdoors and in community • V: Self mobility severely limited – even with aid of supporting technology – (don’t say “wheelchair-bound”)

 Gross Motor Functional Classification System • • • I - sitting by age Gross Motor Functional Classification System • • • I - sitting by age 2 independently II- sitting by age 2, +/- hand support III- sitting by age 2 with back support IV -sitting always requires trunk support V -poor antigravity control in sitting/prone

Gross Motor “Growth Chart” Motor Skills as Outcome Gross Motor “Growth Chart” Motor Skills as Outcome

 Apolipoprotein E and cerebral palsy severity K Donovan et al. • Brain injury Apolipoprotein E and cerebral palsy severity K Donovan et al. • Brain injury in adults – Poor outcome association • Brain injury in children – Potentially protective

 Apolipoprotein E and cerebral palsy severity K Donovan et al. • • • Apolipoprotein E and cerebral palsy severity K Donovan et al. • • • U Va 179 CP patients Mean 9 y/o 56% male GMFCS APO E 4 allelles compared per functional level

 Apolipoprotein E and cerebral palsy severity K Donovan et al. • 30% of Apolipoprotein E and cerebral palsy severity K Donovan et al. • 30% of patients had at least 1 allele • If 1 allele – 61% of patients with 1 were less severe • If 2 alleles – 80% of patients with 2 were less severe • Greatest protective effect – Injury in perinatal period – > 36 weeks gestation

 Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Low Birthweight With Cerebral Palsy J Child Neurol. 2006 Sep; 21(9): 743 -7. Bodensteiner JB, Johnsen SD • 1025 patients in CP rehab registry • 157 patients – < 1000 g BW – < 28 weeks gestation • 94 had MRI’s • 50 MRI’s available for review

 Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Low Birthweight With Cerebral Palsy J Child Neurol. 2006 Sep; 21(9): 743 -7. Bodensteiner JB, Johnsen SD • normal cerebrum 8 • decreased white-matter volume without gliosis (n = 36) • periventricular leukomalacia (n = 16) • thin corpus callosum (n = 18).

 Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Magnetic Resonance Imaging (MRI) Findings in Children Surviving Extremely Premature Delivery and Extremely Low Birthweight With Cerebral Palsy J Child Neurol. 2006 Sep; 21(9): 743 -7. Bodensteiner JB, Johnsen SD • Cerebellar abnormalities 32 – vs 18 normal • destruction of major portions of the cerebellum (usually the inferior vermis and hemispheres) (n = 23) • focal or unilateral loss of cerebellar tissue (n = 4). “_ _ _ Syndrome”

 Polymorphism in the neuregulin gene and brain damage in preterm newborns W Bueter Polymorphism in the neuregulin gene and brain damage in preterm newborns W Bueter et al. • • High risk follow-up 54 patients 2 y/o < 32 week gestation Periventricular echodensity PVE Cystic periventricular leukomalacia c. PVL Cerebral palsy CP Developmental delay DD

 Neuregulin gene and brain damage in preterm newborns W Bueter et al. PVE Neuregulin gene and brain damage in preterm newborns W Bueter et al. PVE

 Neuregulin gene and brain damage in preterm newborns W Bueter et al. PVE Neuregulin gene and brain damage in preterm newborns W Bueter et al. PVE c. PVL 41 19 9 17 CC 6 1 0 0 0 CT 36 50 19 6 14 TT 12 25 25 25 33 Total 54 CP DD

 Neuregulins • Ligand of tyrosine kinase • Cell communication model • Differentiation in Neuregulins • Ligand of tyrosine kinase • Cell communication model • Differentiation in lung, heart, breast, and nervous system • Secretion by one cell type acted on by neighboring different cell type with receptor

Molecular and Cellular Neuroscience 7, 247– 262 (1996) Article No. 0019 MCN REVIEW Neuregulins Molecular and Cellular Neuroscience 7, 247– 262 (1996) Article No. 0019 MCN REVIEW Neuregulins in Development Greg Lemke

 Neuregulins • • Radial glial formation Neurotransmitter receptor regulation Oligodendrites development Onset of Neuregulins • • Radial glial formation Neurotransmitter receptor regulation Oligodendrites development Onset of puberty

B R A I N R E S E A R C H R B R A I N R E S E A R C H R E V I E W S 5 1 ( 2 0 0 6 ) 1 6 1 – 1 7 5; Neuregulins: Versatile growth and differentiation factors in nervous system development and human disease R. M. Esper et al.

Cerebral Palsy- definition • • Descriptive, not etiologic diagnosis Two parts: anatomy and physiology Cerebral Palsy- definition • • Descriptive, not etiologic diagnosis Two parts: anatomy and physiology Disorder of posture and movement Secondary to static lesion of brain

Non-orthopedic Impairments • CNS related: MR, seizure, vision, hearing • HCM: feeding, nutrition, growth, Non-orthopedic Impairments • CNS related: MR, seizure, vision, hearing • HCM: feeding, nutrition, growth, behavior • Orifices: dental, drooling, bowel, bladder

Classification • Spastic – Hemiparesis – Diplegia – Quadriparetic 70 -80 Classification • Spastic – Hemiparesis – Diplegia – Quadriparetic 70 -80

Classification • Dyskinetic – Athetoid – Dystonic – Chorea – Ballismus – Tremor 10 Classification • Dyskinetic – Athetoid – Dystonic – Chorea – Ballismus – Tremor 10 -15

Classification • Rigid 5% • Ataxic 1% • Mixed 10 -15% Classification • Rigid 5% • Ataxic 1% • Mixed 10 -15%

Mental Retardation • 60% of all patients • IQ < 50 – 2/3 in Mental Retardation • 60% of all patients • IQ < 50 – 2/3 in quadriplegics – 1/2 in all other types • Subtypes – mild – moderate – severe/profound 15% 35% 50%

Predictors of Mental Retardation • Hemiplegia – size, not location of lesion • Diplegia Predictors of Mental Retardation • Hemiplegia – size, not location of lesion • Diplegia – worse with term than preterm in general • Dyskinesia – 1/2 MR – choreoathetoid majority with normal IQ

Seizures • • Incidence 35 -45% Proportionally with increasing MR Spastic type > dyskinetic Seizures • • Incidence 35 -45% Proportionally with increasing MR Spastic type > dyskinetic type Quadriplegia > spastic diplegia, dystonic

Hearing • Incidence of SNL 1/8 - 1/3 of all • Highest with athetoid Hearing • Incidence of SNL 1/8 - 1/3 of all • Highest with athetoid due to kernicterus

Vision • Strabismus – 1/2 of all spastic CP • Hemianopsia – > 1/4 Vision • Strabismus – 1/2 of all spastic CP • Hemianopsia – > 1/4 of all hemiplegics • Higher incidence of refractive errors

Orifices • • Dental Drooling Bowel Bladder Orifices • • Dental Drooling Bowel Bladder

Dental • Gum disease • Caries – extrinsic factor: hygeine difficult – ? Inherent Dental • Gum disease • Caries – extrinsic factor: hygeine difficult – ? Inherent enamel problems

Drooling • Oromotor dysfunction NOT hypersalivation • Why a problem? – Social rejection – Drooling • Oromotor dysfunction NOT hypersalivation • Why a problem? – Social rejection – wet/foul odor, bibs – maceration, skin breakdown – fluid loss

Drooling: Therapy • Behavior Modification • Oro-motor therapy • Surgery – removal of gland, Drooling: Therapy • Behavior Modification • Oro-motor therapy • Surgery – removal of gland, reposition duct • Medication – anticholinergics – glycopyrrolate

Bowel • Constipation tendency • Toilet training – parental frustration – remember the developmental Bowel • Constipation tendency • Toilet training – parental frustration – remember the developmental level – problem if loss of motor level also

Bladder • Not as well recognized • Neurogenic bladder can occur • think/ask about Bladder • Not as well recognized • Neurogenic bladder can occur • think/ask about it