Reliability of the peabody developmental gross motor scale in children with cerebral palsy.

The test/retest, intrarater, and interrater reliability of the Peabody Development Gross Motor Scale (PDGMS) was assessed in 12 children with mild or moderate cerebral palsy. A baseline test was administered, scored, and videotaped by one rater and rescored from the videotape by a second independent rater. In order to minimize the effect of developmental maturation, test/retest correlation coefficients of the tests were performed two weeks apart. The intraclass correlation coefficients ranged from 0.82 to 0.98. For interrater reliability, testing following the same protocol was repeated at 2 weeks, 3 and 6 months. Interrater correlation coefficients (r) ranged from 0.89 to 0.98. Interrater correlation coefficients (ICC) from scoring and later rescoring ten videotapes with the closest and furthest interrater agreement ranged from 0.88 to 0.99. The balance and locomotor skill categories were most responsive for assessing gross motor function in this population. These data support the use of the PDGMS as an assessment tool for children with cerebral palsy and the reliability of videotaping assessments.

Continence following electrical stimulation and EMG biofeedback in a teenager with imperforate anus.

A teenage boy with repaired high imperforate anus relied on daily enemas for social continence. After treatment with low intensity transcutaneous electrical stimulation and electromyographic biofeedback home programs, he achieved improved fecal continence requiring only one enema per month.

Neuromuscular approach to the motor deficits of cerebral palsy: a pilot study.

Six children with mild cerebral palsy (CP) entered a study of overnight low-intensity transcutaneous electrical stimulation (ES) to the leg muscles. After 6 months, statistically significant improvement was noted on the Peabody Developmental Motor Scales scores in gross motor, locomotor, and receipt/propulsion skills. When ES was withdrawn for 6 months, there was uniform loss in scores. Reinstitution of ES resulted in further significant improvements in total gross motor, balance, locomotor, and receipt/propulsion skills. In selected cases, overnight ES may be a useful addition to standard rehabilitation services.

The slump test.

The Slump Test is an inexpensive, easily administered assessment tool that can help record qualitative changes in sitting in an objective manner. Objective data are useful to document treatment efficacy over time. Over the past 2 years, the Slump Test has been administered at our clinic to a varied population with respect to age, neuromuscular condition, and functional abilities. At the time of this writing, more than 200 children have had the Slump Test administered as part of their assessment protocol. Most of these children have been between the ages of 3 and 8 years; have cerebral palsy, decreased trunk control, and limb spasticity; and are dependent on a wheelchair or aide for ambulation. In conclusion, the picture representation and objective data obtained from the Slump Test along with a qualitative description of sitting have provided us with a useful means of assessing change over time.

Necrotizing enterocolitis and volvulus in the premature neonate.

Premature infants presenting to the neonatal intensive care unit at the Hospital for Sick Children with a surgical abdomen over a 5-year period were studied retrospectively to determine the factors leading to the diagnosis of malrotation with volvulus and necrotizing enterocolitis (NEC). Fifteen preterm infants (less than 37 weeks) were diagnosed as having volvulus, and 54 had surgically treated NEC. Those with NEC were more likely to be systematically ill with grossly bloody stools, abdominal tenderness, and thrombocytopenia (P less than .005). Bilious vomiting and bilious gastric residuals were the only hallmarks of volvulus (P less than .005). Although the radiographic findings of thickened bowel walls and intramural air were significantly related to NEC, the accuracy and interobserver reliability in diagnosing these features was variable as was the ability to distinguish NEC from volvulus or normal on plain abdominal radiographs. Volvulus is an important cause of surgical abdomen in the preterm infant and can be misdiagnosed as NEC. An unusual course or the presence of bilious vomiting in any patient thought to have NEC should alert the clinician to the possibility of this diagnosis.

Association of changes in bombesin immunoreactive neuroendocrine cells in lungs of newborn infants with persistent fetal circulation and brainstem damage due to birth asphyxia.

The pulmonary neuroendocrine (NE) cells, from 16 term infants dying at 1-4 days of age from birth asphyxia, were immuno stained for bombesin-like immunoreactivity by the immunoperoxidase method. The distribution and frequency of bombesin-immunoreactive NE cells were quantified morphometrically and correlated with the presence or absence of brainstem function and persistent fetal circulation (PFC). In infants with loss of brainstem function, the frequency of bombesin immunoreactive NE cells was significantly increased compared to infants with intact brainstem function, i.e. meconium aspiration with PFC. Infants with brainstem injury, with one exception, failed to develop PFC. Pathological changes in the tegmentum of the brainstem, i.e. containing the respiratory center, correlated in nine of 10 cases with loss of brainstem function. These data suggest an inverse relationship between brainstem function, release of bombesin-like peptide from the pulmonary NE cells and the functional state of the pulmonary vasculature. Intact brainstem function appears to be essential for both the release of bombesin-like peptide from the NE cells and for pulmonary vasoconstriction leading to PFC; absence of brainstem function is, on the other hand, associated with failure to release bombesin-like peptide and loss of PFC type reactivity in the pulmonary vasculature. However, it appears unlikely that bombesin itself is a direct mediator of pulmonary vasoconstriction.

Periventricular infarction diagnosed by ultrasound: a postmortem correlation.

Ultrasound brain scans sometimes demonstrate increased echogenicity or cysts, or both, in the periventricular white matter, superolateral to the ventricle, in the most common site of periventricular infarction. Over 33 months, 23 preterm infants dying after 20 or more days of life were entered into this study. Superolateral echogenicity or cysts were found in 13 (57%) cases. Periventricular infarction was present at autopsy in 12 (52%) cases. Ultrasound accurately diagnosed the size, site, and extent of periventricular infarction in 78% of scans. Interpretive errors were made with poor-quality scans and with early and late studies. We conclude that sector ultrasound brain scans accurately diagnose major periventricular infarction. Hemorrhage into the site of infarction is not a prerequisite for diagnosis of periventricular infarction by ultrasound.

Diagnostic accuracy of neonatal brain imaging: a postmortem correlation of computed tomography and ultrasound scans.

The aim of this study was to validate brain imaging techniques in the preterm infant. A homogeneous group of very immature (less than 32 week) neonates dying in the neonatal period were sequentially scanned with linear-array real-time ultrasound scans, and after death with compound B static sector ultrasound and high-resolution computed tomography (CT) scans. All three imaging techniques were correlated with the autopsy results. All germinal matrix bleeds greater than 5mm in size and intraventricular hemorrhages associated with ventricular dilation or distortion were accurately diagnosed. In the immature infant it was difficult to distinguish the normal highly vascular germinal matrix and choroid plexus from hemorrhage into the brain or ventricles, respectively. Further studies that address the questions of accurate timing and incidence of bleeds must consider the spatial resolution of the individual scanner, the maturity of the brain, the site and size of the lesion, and the evolution of the lesion. For the diagnosis of major hemorrhagic lesions in the preterm infant, either ultrasound or CT scans may be used with confidence.

Prediction of death and major handicap in very preterm infants by brain ultrasound.

A linear-array real-time ultrasound scanner was used to examine the brains of all 95 infants born at less than 33 weeks of gestation who were admitted to the neonatal unit of University College Hospital in 1979. Abnormalities were detected in 41 (43%). 36 infants had haemorrhages into the germinal layer (GLH) and/or ventricles (IVH). 8 infants had cerebral atrophy (together with GLH/IVH in 5 infants). 8 (13%) of 63 infants with normal scans or small (grade-I) GLH/IVHs died, whereas 19 (59%) of 32 infants with larger haemorrhages or other intracranial lesions died (p less than 0.0005). At follow-up, at a median corrected age of 45 weeks, only 2 (4%) of 53 infants with normal scans or grade-I haemorrhages had evidence of major neurodevelopmental handicaps, but 5 (38%) of 13 infants with more extensive haemorrhages or cerebral atrophy had major handicaps (p less than 0.005). Brain scanning with ultrasound in the first days of life identified most infants in the population studied who subsequently died or survived with handicaps severe enough to be detected within the first year.

Ultrasound detection of brain damage in preterm infants.

A linear-array real-time ultrasound scanner with a 5 MHz probe was used to examine the brains of 31 infants born at less than 33 weeks of gestation. The equipment was mounted on a small trolley and the infants could easily be scanned in their incubators. 7 of the 31 infants were shown to have cerebral lesions, including haemorrhages into the germinal layer and ventricles, hydrocephalus, and infarction of the periventricular region and cerebral cortex. The type and extent of the lesions were conformed by computerised tomography and at necropsy. Ultrasound scanning is a safe, simple, non-invasive technique that provides valuable information for the diagnosis, investigation, and treatment of lesions in the brains of newborn infants.

Peripheral median nerve damage secondary to brachial arterial blood gas sampling.

Examination at 18 months post-term of 139 infants of birth weight less than or equal to 1,500 gm revealed 18 instances (13%) of persistent median nerve damage. All affected infants had received frequent percutaneous brachial artery punctures as neonates. Block sections of the cubital fossa done at autopsy on 12 randomly selected very low-birth-weight infants showed perineural hemorrhage, and Wallerian degeneration or traumatic neuroma of the median nerve in eight patients. It is recommended that brachial artery punctures be avoided whenever possible in the neonatal period.

An integrated model for haemorrhagic and ischaemic lesions in the newborn brain.

The vascular anatomy of the developing brain changes from a predominantly basal ganglia orientated pattern at 24 wk to a cortically orientated pattern by 34 wk. This information, combined with other known data on cerebral vascular anatomy and physiology, allows us to develop a model for haemorrhagic and ischaemic lesions in the newborn brain based on two main series of reactions. One series involves the effects of hypoxia and hypercapnia in leading to breakdown of the blood--brain barrier with resultant oedema or haemorrhage. The other links a fall in perfusion pressure and factors causing vasoconstriction with the development of ischaemic lesions. Application of the model involves additional consideration of the state of development of the cerebral vessels at the gestational age concerned. The model helps to explain the observed findings in germinal layer haemorrhage/intraventricular haemorrhage, periventricular leukomalacia and venous infarction, in the preterm brain. Its use also suggests that there are three patterns of vulnerability in the term infant brain. The model carries several implications for the neonatal management of preterm infants. Routine continuous monitoring of blood pressure is of critical importance as cerebral blood flow may vary with blood pressure in the ill newborn. It is important to avoid head compression which may lead to impaired cerebral perfusion. Finally, control of the acid--base status is essential for maintenance of the blood--brain barrier. Correction of abnormal values must be carried out without provoking rapid swings in either the serum osmolarity or the blood pressure.

The status at two years of low-birth-weight infants born in 1974 with birth weights of less than 1,001 gm.

A two-year follow-up study of 43 infants of birth weight less than or equal to 1,000 gm born during 1974 revealed the following: average height at two years was between the tenth and twenty-fifth percentiles; average weight between the third and tenth percentiles. Fifteen (35%) developed lower respiratory tract infections during the first two years. Seven (16%) had retrolental fibroplasia. Major neurologic defects occurred in four (9%); severe developmental delay (mean developmental quotient less than 80) was found in nine others (21%). Defects of the central nervous system were closely associated with a neonatal history of intracranial hemorrhage or seizures or both.