Bruce K. Shapiro

The effects of physical therapy on cerebral palsy. A controlled trial in infants with spastic diplegia

Legislatively mandated programs for early intervention on behalf of handicapped infants often stipulate the inclusion of physical therapy as a major component of treatment for cerebral palsy. To evaluate the effects of physical therapy, we randomly assigned 48 infants (12 to 19 months of age) with mild to severe spastic diplegia to receive either 12 months of physical therapy (Group A) or 6 months of physical therapy preceded by 6 months of infant stimulation (Group B). The infant-stimulation program included motor, sensory, language, and cognitive activities of increasing complexity. Masked outcome assessment was performed after both 6 and 12 months of therapy to evaluate motor quotient, motor ability, and mental quotient. After six months, the infants in Group A had a lower mean motor quotient than those in Group B (49.1 vs. 58.1, P = 0.02) and were less likely to walk (12 vs. 35 percent, P = 0.07). These differences persisted after 12 months of therapy (47.9 vs. 63.3, P less than 0.01, and 36 vs. 73 percent, P = 0.01, respectively). We noted no significant differences between the groups in the incidence of contractures or the need for bracing or orthopedic surgery. Group A also had a lower mean mental quotient than Group B after six months of therapy (65.6 vs. 75.5, P = 0.05). The routine use of physical therapy in infants with spastic diplegia offered no short-term advantage over infant stimulation. Because of the limited scope of the trial, our conclusions favoring infant stimulation are preliminary. The results suggest that further study of the effects of both physical therapy and infant stimulation is indicated.

GROWTH OF SEVERELY IMPAIRED CHILDREN: NEUROLOGICAL VERSUS NUTRITIONAL FACTORS

Nineteen children with cerebral palsy who have had gastrostomies since 1981 and who have been followed for at least six months postoperatively were reviewed to assess the effects of gastrostomy feeding on their growth and to determine whether growth failure was due to neurological or nutritional dysfunction. The childrens ages ranged from five to 168 months (mean 60·4 months) and follow‐up extended from six to 41 months (mean 23·1 months). All were profoundly handicapped, with involvement of all four limbs. 11 also had seizures and all 19 were severely to profoundly retarded. Heights and weights were recorded during the visit immediately before surgery and at most recent follow‐up. Data were recorded as a standard deviation score (Z‐score).

NORMAL GROSS MOTOR DEVELOPMENT: THE INFLUENCES OF RACE, SEX AND SOCIO-ECONOMIC STATUS

The ages at attainment of 12 gross motor milestones were obtained prospectively during well‐baby visits in the first two years of life for 381 children. All had been born at term and were judged to be normal at one year. A longitudinal analysis, using an index summarising each chilďs progress for eight selected milestones, is reported for the 284 children for whom data were complete. A high percentage of parents were able to report the age at attaining milestones with an acceptable degree of variability. The children attained milestones at earlier ages than traditionally reported. There were only minor sex differences in age at attainment, but black children attained milestones earlier than white children.

Clinical linguistic and auditory milestone scale: prediction of cognition in infancy.

At each well‐child examination between birth and two years, parents of 448 infants were questioned about their childs age at attainment of 25 linguistic and auditory milestones. Parental reports were compared with the results of independently administered Bayley Mental Developmental Index (MDI) at one year of age. Parental recall of information was high, ranging from 70 to 99 per cent for 21 of the 25 milestones. The milestone performance of infants with normal MDI scores showed an orderly, sequential progression of expressive and receptive language. Across the entire population studied, the correlation between milestone attainment and MDI was statistically significant for 24 of the 25 milestones, and later attainment of milestones correlated with lower MDI. As a group, ‘delayed’ infants (MDI <68) attained milestones significantly later than ‘average’ infants (MDI 85 to 116) for 20 of 25 items. Attention to linguistic and auditory milestones early in infancy can contribute to the early detection and diagnosis of mental retardation and disorders of communication.

A PROPOSED FORMULA FOR CALCULATING ENERGY NEEDS OF CHILDREN WITH CEREBRAL PALSY

This study compared two methods of calculating the energy needs of children with CP: the traditional method, using RDA for chronological age; and the Krick method, which calculates the BMR and includes factors for muscle tone, movement or level of activity, and energy requirements for normal and catch‐up growth. 30 tube‐fed children, aged between nine months and 18 years, who were inpatients for longer than one week at the Kennedy Institute, were reviewed. 14 were female. They were weighed at admission and discharge to evaluate the rate of growth; calorie prescriptions at discharge were based on the clinical course. The Krick method was found to be a more potent predictor of the discharge prescription than the RDA method.

Primitive reflex profile: a quantitation of primitive reflexes in infancy.

This report describes quantitative standardization data on nine primitive reflexes for a cohort of 381 normal infants evaluated longitudinally at each visit between birth and two years of age. Normality was confirmed by the use of the Bayley Scales of Infant Development at one year of age. The standardization of this new examination technique complements the traditional infant neurological examination and may allow primitive reflexes to become a useful adjunct to the prediction of motor disability in early infancy.

CAT/CLAMS A Tool for the Pediatric Evaluation of Infants and Young Children With Developmental Delay

The American Academy of Pediatrics recommends regular developmental screening as a part of routine child health supervision. However, the pediatrician has a limited number of tools available to further evaluate a child who is found to be suspect or abnormal on a developmental screening test. The Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS) was therefore developed to provide pediatricians with a technique to assess infants and toddlers with suspected developmental delay. The CAT/CLAMS demonstrated strong psychometric properties. Concurrent validity with the Bayley Scales of Infant Development (BSID) was demonstrated in 43 children ages 12 to 19 months who were tested on three occasions with both instruments (correlation coefficient ranging between 0.63 and 0.87; P<.001). Predictive validity 6 and 12 months later was also demonstrated in this population with correlation coefficients ranging between 0.73 and .077, significant at the P=.001 level. Utilizing the CAT/CLAMS as part of the pediatricians evaluation of children with developmental concerns would allow the pediatrician to compare language and nonlanguage problem-solving abilities and, therefore, aid in diagnosis and appropriate referral.

Motor Functions: Associated Primitive Reflex Profiles

The results of reflex/motor activity interactions in 177 normal infants are evaluated. The asymmetrical tonic neck reflex, tonic labyrinthine reflex‐supine, and Moro reflexes were assessed for each child at birth and at intervals up to 12 months. Ages of rolling prone to supine, rolling supine to prone, and sitting alone were elicited from parents. The effects of the primitive reflexes on early motor activity were assessed, and statistically significant correlations were demonstrated between decreased reflex activity and the emergence of motor milestones. The distinctive association of reflex activity with motor function suggests the interaction of several reflexes (a primitive reflex profile) rather than the influence of isolated reflex activity. Such patterns support the hypothesis that decreasing primitive reflex activity is associated with the onset of volitional motor activity in normal infants.

Age of presentation in developmental disability.

ABSTRACT. It has not been determined whether severity of handicap or other associated factors are more important in determining the age of presentation for developmental disabilities. The relationship between age at presentation and referral source, presenting complaint, diagnosis, and associated factors (medical illness, motor signs, or behavioral disturbances) was examined in 738 consecutive children referred for developmental evaluation during 1982–1983. The nature of the complaint or diagnosis (motor, language, behavioral, or educational) was a far better predictor of age of presentation than the severity of the disorder. The degree of mental retardation did not affect age of presentation. Behavior problems did not affect the age of presentation for school failure or learning disability, but were associated with later presentation for motor delay, language delay, communication disorder, and within all IQ groups. The association of topography of handicap rather than severity with age of presentation should be considered when establishing or evaluating efforts at early identification of developmental disability.

The prevalence of the FMR1 and FMR2 mutations among preschool children with language delay

We examined the prevalence of the fragile X mental retardation (FMR1) full mutation and fragile X E mutation (FMR2) among preschoolers evaluated for language delay. A total of 534 preschoolers recruited from a Developmental Pediatric or Speech and Language Disorders clinic were tested with Southern blot and polymerase chain reaction DNA analyses; 3 were found to have the FMR1 full mutation. None of the 534 children tested positive for the FMR2 full mutation; however, 3 children had unusually small FMR2 alleles suggestive of FMR2 deletions. Screening for fragile X among language-delayed preschoolers is warranted, particularly when there is a family history of mental retardation, but regardless of sex or the presence of behavioral or physical features associated with the fragile X phenotype. The potential benefit of screening for FMR2 alterations is an unexpected implication of the study and is worthy of continued exploration.