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From the American Academy of PediatricsClinical Report

Motor Delays: Early Identification and Evaluation

Garey H. Noritz, Nancy A. Murphy and NEUROMOTOR SCREENING EXPERT PANEL
Pediatrics June 2013, 131 (6) e2016-e2027; DOI: https://doi.org/10.1542/peds.2013-1056
Garey H. Noritz
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Nancy A. Murphy
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  • FIGURE 1
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    FIGURE 1

    Identifying children with motor delays: an algorithm for surveillance and screening.

  • FIGURE 2
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    FIGURE 2

    Examples of physical examination maneuvers. Adapted from Nercuri E, Haataja L, Dubowitz L. Neurological assessment in normal young infants. In: Cioni C, Mercuri E, eds. Neurological Assessment in the First Two Years of Life. London, UK: Mac Keith Press; 2007:30–31. a In term infants, these findings do not change significantly after 3 months of age.

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    TABLE 1

    Motor Milestones for Developmental Surveillance at Preventive Care Visitsa

    AgeGross Motor MilestonesFine Motor Milestones
    2 moLifts head and chest in prone
    4 moRolls over prone to supine; supports on elbows and wrists in proneHands unfisted; plays with fingers in midline; grasps object
    6 moRolls over supine to prone; sits without supportReaches for cubes and transfers; rakes small object with 4 fingers
    9 mobPulls to stand; comes to sit from lying; crawlsPicks up small object with 3 fingers
    1 yWalks independently; standsPuts 1 block in a cup; bangs 2 objects together; picks up small object with 2-finger pincer grasp
    15 moWalks backward; runsScribbles in imitation; dumps small object from bottle, with demonstration
    18 mobWalks up steps with hand heldDumps small object from bottle spontaneously; tower of 2 cubes; scribbles spontaneously; puts 10 blocks in a cup
    2 yRides on toy without pedals; jumps upBuilds tower and horizontal train with 3 blocks
    2.5 ybBegins to walk up steps alternating feetImitates horizontal and vertical lines; builds a train with a chimney with 4 blocks
    3 yPedals; climbs on and off furnitureCopies a circle drawing; draws a person with head and one other body part; builds a bridge with 3 blocks
    4 yClimbs stairs without support; skips on 1 footDraw a person with 6 parts, simple cross; buttons medium-sized buttons
    • Adapted from Capute AJ, Shapiro BK, Palmer FB, Ross A, Wachtel RC. Normal gross motor development: the influences of race, sex and socioeconomic status. Dev Med Child Neurol. 1985;27(5):635–643; Accardo PJ, Capute AJ. The Capute Scales: Cognitive Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS). Baltimore, MD: Paul H. Brooks; 2005; and Beery KE, Beery NA. The Beery-Buktenica Developmental Test of Visual-Motor Integration (Beery VMI) Administration, Scoring and Teaching Manual. Minneapolis, MN: NCS Pearson Inc; 2004.

    • ↵a These milestones generally represent mean age of performance of these skills.

    • ↵b It is recommended that a standardized developmental test be performed at these visits.

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    TABLE 2

    Key Elements of the Motor History

    Key Elements of Motor HistoryExample
    Delayed acquisition of skillIs there anything your child is not doing that you think he or she should be able to do?
    Involuntary movements or coordination impairmentsIs there anything your child is doing that you are concerned about?
    Regression of skillIs there anything your child used to be able to do that he or she can no longer do?
    Strength, coordination, and endurance issuesIs there anything other children your child’s age can do that are difficult for your child?
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    TABLE 3

    “Red Flags” in the Evaluation of a Child With Neuromotor Delay

    Red Flags: Indications for Prompt ReferralImplications
    Elevated CK to greater than 3× normal values (boys and girls)Muscle destruction, such as in DMD, Becker muscular dystrophy, other disorders of muscles
    Fasciculations (most often but not exclusively seen in the tongue)Lower motor neuron disorders (spinal muscular atrophy; risk of rapid deterioration in acute illness)
    Facial dysmorphism, organomegaly, signs of heart failure, and early joint contracturesGlycogen storage diseases (mucopolysaccharidosis, Pompe disease may improve with early enzyme therapy)
    Abnormalities on brain MRINeurosurgical consultation if hydrocephalus or another surgical condition is suspected
    Respiratory insufficiency with generalized weaknessNeuromuscular disorders with high risk of respiratory failure during acute illness (consider inpatient evaluation)
    Loss of motor milestonesSuggestive of neurodegenerative process
    Motor delays present during minor acute illnessMitochondrial myopathies often present during metabolic stress
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    TABLE 4

    Common Genetic Disorders for Which Neuromotor Delays May Be a Presenting Feature

    ConditionInheritanceClinical TestingClinical Caveats
    Angelman syndromeSporadicMethylation testing for Prader-Willi/Angelman syndrome critical region, gene sequencing of UBE3A geneInfantile hypotonia and delayed motor milestones, usually present with global delays; dysmorphic features are subtle in infancy.
    Chromosome disordersMany sporadic; high recurrence risk for unbalanced translocations if 1 parent has a balanced translocationChromosome analysis, single nucleotide polymorphism microarraySome patients will have multiple anomalies and will have global developmental delays. Some may present in infancy or early childhood with delayed motor and/or speech milestones.
    Down syndromeChromosome mosaicism also seen.
    Klinefelter syndrome
    Rare deletions and duplications
    Deletion 22q11 syndrome (velocardiofacial syndrome)Autosomal dominant (most cases new mutations)Fluorescence in situ hybridization (FISH) for deletion 22q11.290% of cases new mutations. Feeding and speech disorders and cognitive impairment also seen. >50% will have a congenital heart defect.
    DMDX-linked recessiveCK, sequencing of dystrophin geneBecker and Duchenne muscular dystrophies are caused by mutations in different regions of the dystrophin gene. Becker muscular dystrophy has a later onset of symptoms with a less severe course; 67% of cases are inherited, 33% are new mutations.
    Becker muscular dystrophy
    Fragile X syndromeX-linkedGene sequencing and methylation analysis of FMR1 geneUsually have global delays and cognitive impairment but may present in infancy or early childhood with predominantly motor delays. Males affected primarily, but females with FMR1 expansions may also be affected.
    Mitochondrial myopathiesAutosomal recessive;Constitutional and mitochondrial genetic testing, lactate/pyruvate levels and ratio, serum amino acidsGenetic heterogeneity. May not present in infancy. Also at risk for cardiomyopathy, vision loss, hearing loss, cognitive disabilities.
    X-linked recessive
    mitochondrial inheritance
    Myotonic muscular dystrophyAutosomal dominantGene sequencing for DMPK geneMay see anticipation with progression of phenotype in subsequent generations.
    Neurofibromatosis type 1 (NF1)Autosomal dominantUsually a clinical diagnosis, gene sequencing NF1 gene50% new mutations. Hypotonia most evident in infancy and early childhood. Suspect NF1 if hypotonia seen with multiple café au lait spots.
    Noonan syndromeAutosomal dominantGene sequencing for PTPN11 gene, genetically heterogeneous and multiple gene sequencing panels are availableGenetic heterogeneity. Commonly associated with short stature, ptosis, learning and developmental delays, hypotonia, pulmonary stenosis, cryptorchidism, cardiomyopathy.
    Prader-Willi syndromeSporadicDNA methylation testing for Prader-Willi/Angelman syndrome critical regionHypogonadism, especially in boys. Hypotonia most evident in infancy and may be profound.
    Spinal muscular atrophy, including congenital axonal neuropathy, Werdnig-Hoffmann disease, Kugelberg-Welander diseaseAutosomal recessiveGene deletion or truncation studies for SMN1 gene (95% to 98% of cases)Usually presents in early infancy with severe hypotonia. Milder forms identified at later ages.
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    TABLE 5

    CPT Codes for Developmental Screening

    Services/Step in AlgorithmNotesCPT CodeComments
    Pediatric preventive care visitAll preventive care visits should include developmental surveillance; screening is performed as needed or at periodic intervals99381–99394 (EPSDT)
    Developmental/medical evaluation: Office or Other Outpatient Services Codes; New PatientIf performed by the physician as a new patient outpatient office visit99201-9920599204 is used for evaluations performed by the physician that are detailed and moderately complex or take at least 45 min (with more than half spent counseling); 99205 is used for evaluations that are comprehensive and highly complex or take 60 min (with more than half spent counseling)
    Developmental/medical evaluation: Office or Other Outpatient Services Codes; Established PatientIf performed by the physician as an outpatient office visit99210–9921599214 is used for evaluations performed by the physician that are detailed and moderately complex or take at least 25 min (with more than half spent counseling); 99215 is used for evaluations that are comprehensive and highly complex or take 40 min (with more than half spent counseling)
    Developmental/medical evaluation/Office or Other Outpatient Consultations CodesIf performed by the physician as an outpatient office visit99241–9924599244 is used for “moderate activities” of up to 60 min (with more than half spent counseling); 99245 is used for “high” activity of up to 80 min (with more than half spent counseling)
    Developmental screeningDoes not require any physician work; rather, clinical staff can score results and provide to physician for interpretation as part of E/M96110Reported in addition to E/M services provided on the same date
    Developmental testingUsed for extended developmental testing typically provided by the medical provider (often up to 1 h), including the evaluation interpretation and report96111Reported in addition to E/M services provided on the same date
    Identify as a child with special health care needs, and initiate chronic condition managementChildren with special health care needs are likely to require expanded time and a higher level of medical decision-making found in these “higher-level” outpatient codes; these codes are appropriate for services in the office and for outpatient facility services for established patients; these codes may be reported using time alone as the factor if more than half of the reported time is spent in counseling99211–99215As above
    Prolonged servicesAt any point during the algorithm when outpatient office or consultation codes are used, prolonged physician service codes may be reported in addition when visits require considerably more time than typical for the base code alone; both face-to-face and non–face-to-face codes are available in CPT9935499354 for first 30–74 min of outpatient face-to-face prolonged services
    9935599355 for each additional 30 min
    9935899358 for first 30–74 min of non–face-to-face prolonged services
    9935999359 for each additional 30 min
    • E/M, evaluation and management; EPSDT, Early and Periodic Screening, Diagnostic and Treatment program.

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1 Jun 2013
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Motor Delays: Early Identification and Evaluation
Garey H. Noritz, Nancy A. Murphy, NEUROMOTOR SCREENING EXPERT PANEL
Pediatrics Jun 2013, 131 (6) e2016-e2027; DOI: 10.1542/peds.2013-1056

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Motor Delays: Early Identification and Evaluation
Garey H. Noritz, Nancy A. Murphy, NEUROMOTOR SCREENING EXPERT PANEL
Pediatrics Jun 2013, 131 (6) e2016-e2027; DOI: 10.1542/peds.2013-1056
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    • Introduction
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    • The Algorithm: Identifying Children With Motor Delays: An Algorithm for Surveillance and Screening
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  • Noritz GH, Murphy NA, NEUROMOTOR SCREENING EXPERT PANEL. Motor Delays: Early Identification and Evaluation. Pediatrics. 2013;131(6). Available at: www.pediatrics.org/cgi/content/full/131/6/e2016
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