An Evaluation of the Infant with Motor Delays: When and How Much? Sarah Winter, MD Neurodevelopmental Pediatrician Division of General Pediatrics University of Utah Terry Holden, PT CHSCN February 23, 2010 Objectives • Discuss hallmarks of motor delays in infants such as tone patterns, primitive reflexes, milestone delays, and deviances • Using cases to prompt discussion, compare differences in the approach to evaluation • Review brain MRI abnormalities with patterns of motor impairment • Review the purpose of the Neuromotor Team Developmental Disorders in Children Disorders of brain function Cognition : MR/LD Social skills: Autism Behavior : ADHD 11.03.03 Movement : Cerebral Palsy Sensation : Hearing / Vision Impairment Neuromotor Team Children with Special Health Care Needs • Specialty team within the “Child Developmental Clinic” • Types of patients seen – Typically birth to 36 months – Older children with motor impairment with case management needs • Team members: – Sarah Winter, MD, Terry Holden, PT, Scott Jerome, PT, and Alison Seppi, RN – Oversight: Jim Taliaferro, LCSW My developmental path to understanding gross motor delay • But how do you apply this disparate knowledge and be helpful to this child and family? Developmental Progress Move from chaotic pieces of knowledge To A systematic approach of identification, evaluation, and intervention A Systematic Approach Screen Examine Diagnose Treat References for Screening • 2001 AAP Policy Statement: Developmental Surveillance and Screening of Infants and Young Children • EPSDT: Early Periodic Screening, Diagnosis, and Treatment • 2006 AAP Policy Statement: Identifying Infants and Young Children with Developmental Disorders in the Medical Home: An algorithm for Developmental Surveillance and Screening References to Screening and Surveillance • Society for Development and Behavioral Pediatrics website: www.dbpeds.org • Grand Rounds by Paul Young and Charlie Ralston • Podcast produced by Dr. Paul Carbone www.utah.edu/podcast Features of a Good Developmental History • Parents describe gross motor skill delays well • People; both parents and medical care providers, don’t tend to pay attention to fine motor skills e.g. when are kids supposed to have a hand preference? • Ask about language, it is a clue to cognitive development The Pediatric Neurodevelopmental Exam • Gross Motor findings • Fine Motor findings • Language findings – Expressive – Receptive • Social/Behavioral findings Focused exam findings • Tone patterns – Low tone: mild or severe? – High tone: is it symmetric?, is it typical (LE>UE)? – “weird” tone : ataxia, tremor, fluctuating (dystonia) Gross Motor Exam Findings Focused exam • Reflexes; – High or absent – Primitive reflexes – see figure • Postural or protective responses – Lateral, anterior, posterior supports – parachute Examples of primitive reflexes • The Moro is normal in a newborn and should be gone by 4 months • The ATNR is normal in a newborn and should be gone at 6 months Postural (or protective) responses Lateral support: (appears at 6 months) Parachute response: (appears at 10 months) • Anterior (comes 1st) , lateral (2nd), and posterior (3rd) support responses • Parachute response Increased tone 11.03.03 gross motor examination • This child presents with delays in gross motor milestones. His tone is likely low • Differentiate between tone and strength Focused Exam • “Deviant” (atypical but not always delayed) motor patterns – “Bottom scooters” – Circling hands and feet while balanced on the bottom (hypotonia) Some confuse this for a sign of autism – Commando crawling (hemiplegia) Gross motor testing: standardized measures of gross motor function • Purpose • Frequently used tools – Further diagnostic information – Peabody Developmental Motor Scales – Qualifying children for therapeutic services – Bayley Scales of Infant Development – Developmental measure or mark in time – Gross Motor Function Scale – TIMP – Alberta Infant Motor Scales Motor Assessment Tools Test Age Range Description Sens/Spec Time frame/cost Test of Infant Motor Performance (2002) Preterm (>34 weeks) to 4 months adj age Early identification of infants with neuromotor dysfunction Sens 83-96% Spec 78 – 65% 30 minutes Alberta Infant Motor Scales (1994) 0 – 18 months Useful in high risk infants for screening gross motor function Sens 74 – 86% Spec 81 – 93% 20 minutes Peabody Birth – 6 years Developmental Motor Scale 2nd ed. (2000) Measures of gross and fine motor skills, has subtests, gives age equivalent At 4 mo: sens 20 -30 minutes for 25%, spec 73% each subtest At 8 mo: sens 86% spec 46 – 60% 1 – 42 months Frequently used by NICU followup programs and outcome studies Bayley Scales of Infant Development: (PDI) 15-20 minutes Narrowing the Differential Diagnosis • Patient A: in top graph 18 months with negative past medical history. 100 50 0 GM FM Lang Social/Beh GM FM Lang 100 • Patient B: 12 month old with history of failure to thrive 75 50 25 0 Soc/Beh Medical Diagnostic Considerations Case # 1 • 10 month old not sitting or rolling • Pregnancy/Labor/birth HX: Uncomplicated pregnancy, NSVD, BW 8# 3 oz. Apgars normal • FHx: noncontributory #1 • Physical exam: cute blonde, fair skinned, growth parameters: weight >>95%, hgt 75%, OFC, 75% rest of exam normal • Neuro exam: low trunk tone, high extremity tone upper extremities more involved than lower extremities. Hyperreflexia throughout • Neurodevelopmental exam: GM: no sitting balance, prominent extensor thrust , FM: fisted hands, language: smiling, babbling What is an appropriate work-up? • Imaging? – CT vs. MRI • Metabolic Studies? • Genetic Studies? Case # 2 • 24 month old with language delay, not using left arm as well as right arm • Pregnancy.labor /delivery: uncomplicated • Family History: negative • Physical exam: • Neuro exam: reflexes, tone, strength intact • Neurodevelopmental exam: walking, asymmetric use of hands L<R, language delay How helpful is imaging? • Practice Parameter: Diagnostic assessment of the child with cerebral palsy…” Ashwal and Russman et al, Neurology (2004) – Yield of abnormal brain CT in children with CP: 77% – Yield of abnormal brain MRI in kids with CP: 89% and it is helpful in determining timing of injury – Depended on type (n=264) • (dyskinetic CP 100%, quadriplegia 98%, hemi 96%, diplegia 94% ataxic 75%) How helpful are metabolic and genetic studies? • In children with dx of CP – 0 – 4% of children have a metabolic or genetic cause (Ashwal, Russman) – In almost all cases there were atypical features • Hx suggestive of regression • Neuroimaging suggesting genetic or metabolic injury • Family history of childhood neurologic disorder • “If clinical history or findings on neuroimaging do not determine a specific structural abnormality or if there are atypical or additional features on the history or clinical exam, metabolic and genetic testing should be considered.” What if the child doesn’t have CP but a broader presentation of global developmental delay? • Moeschler J, Shevell M and the Committee on Genetics Pediatrics, 2006 – Describes what pediatricians can anticipate as an optimal clinical genetics evaluation • Karyotype, FISH for subtelomere abnormalities, Frag X, molecular genetic testing, imaging, metabolic testing – Report on the usefulness of high resolution chromosome studies (9 – 36%) in patients evaluated for DD/MR – Routine metabolic screening of all patients with DD/MR is not required Gross motor delay and its relationship to other brain functions • Cognition function and CP, depends of the type • For children with spastic quadriplegic CP (Strauss, DMCN, 2005) – 95% with MR • If had dyskinetic CP only 40% with profound MR and 20% no MR Gross motor delay and its relationship to other brain functions • Cognitive Function and Developmental Coordination Disorder or mild motor delays – No good epidemiologic data Using CP as a paradigm… Children Who Outgrew CP Nelson, Ellenberg Pediatrics, 1982 • 229 infants age 12 months with diagnosis of CP • Examined again at 7 y. o. • 118 free of motor handicap • 13% of white children and 25% of black children with MR • Nonfebrile sz, abnormalities of speech, behavior, and extraocular movements were more frequent than controls When do you not need a brain MRI when evaluating a child with motor delays? • Mild delay • Looking for an inutero infection that would leave calcifications (CT is better) • Ultrasound in the NICU showed cystic encephalomalacia and development is consistent with this pattern of CP (generally SQ CP but can have mixed tone) Treatment/Intervention • Cure vs. maximize functional abilities • Only one “cure” in my clinical years National Center for Medical Rehabilitation Research (1995) • Model to assist in the direction of research • Paradigm for chronic disorders • Good fit for persons with motor disorders such as CP, muscular dystropy, spinal cord injury or birth defect (SB) Neuromotor Team Evaluations • Medical Evaluation and Diagnosis • Therapy: PT performs PDMS for evaluation • Educational Concerns: frequent referrals to EI • Technology: assistive devices • Social Supports: referrals to programs as needed 11.03.03 Questions?? Sarah Winter, MD Phone : 801-581-7877