Sensory profile in infants and toddlers with behavioral insomnia and/or feeding disorders.

BACKGROUND: Sleep and feeding difficulties are two common disorders in early childhood. It has been shown that feeding difficulties are more common among children with sleep disorders and vice versa. Since a child's characteristics play a substantial role in these two conditions, we aimed to investigate the sensory profile of infants and toddlers with behavioral insomnia (BI) or feeding disorders (FDs) in comparison with healthy age-matched controls. METHODS: Children aged 7-36 months with BI or FD were recruited from the sleep and feeding disorders clinics. Healthy controls were recruited from well-baby clinics. Parents completed a questionnaire which included demographics and socioeconomic status, as well as a sensory profile evaluation using the Infant/Toddler Sensory Profile (ITSP). RESULTS: Twenty-five children with BI, 28 with FDs and 32 controls were recruited. Oral processing scores were significantly lower in both BI and FD groups vs the controls (p = 0.015 and 0.001, respectively). Auditory processing scores were lower in the FD group vs the controls (p = 0.028). The scores of three out of the four ITSP sensory quadrants (Low Registration, Sensory Sensitivity, and Sensation Avoiding) were significantly lower in the FD group vs the controls (p = 0.027, 0.025, and 0.001, respectively), and in one quadrant (Sensation Avoiding) in the BI group vs the controls (p = 0.037). CONCLUSIONS: There were considerable differences in sensory processing, as reported by parents between children with BI and those with FDs compared to healthy controls, most often in the direction of the 'hypersensitive' profile. These differences may underlie the development and partially explain the coexistence of the two disorders. Sensory profile may be a target of intervention as part of the management of sleep and feeding disorders in early childhood.

Isolated mild white matter signal changes in preterm infants: a regional approach for comparison of cranial ultrasound and MRI findings.

OBJECTIVE: To compare echogenicity detected using cranial ultrasound (cUS) and diffuse excessive high signal intensity (DEHSI) detected using magnetic resonance imaging (MRI) by identical region-based scoring criteria in preterm infants. To explore the association between these white matter (WM) signal changes with early neurobehavior. STUDY DESIGN: Forty-nine pre-selected premature infants with only echogenicity on a first routine cUS1 underwent MRI and a repeated cUS2 at term equivalent age. Echogenicity and DEHSI were graded in various brain areas and diffusivity values were calculated. Neurobehavior was assessed using the Rapid Neonatal Neurobehavioral Assessment Procedure. RESULT: WM signal changes were significantly higher on cUS1 than cUS2; and higher in MRI than cUS2 in posterior regions. Infants with DEHSI demonstrated reduced tissue integrity. Imaging findings were not correlated with early neurobehavior. CONCLUSION: Echogenicity and DEHSI likely represent the same phenomenon. Reduction of over-interpretation of WM signal changes may help define criteria for the judicious use of imaging in routine follow-up of premature infants.

The neurocognitive outcome of IUGR children born to mothers with and without preeclampsia.

OBJECTIVE: To examine long-term behavioral and neurodevelopmental outcome of children born growth restricted and exposed to hypertension in utero at 9 years of age. METHODS: Somatic growth and neurocognitive outcomes were evaluated at age 9-10 years of age in 42 children born with intra uterine growth restriction (IUGR) after pregnancies complicated by hypertensive disorder (17 with maternal preeclampsia and 25 after gestational hypertension). This study group was compared to a control group of 78 IUGR children born after normotensive pregnancy. RESULTS: Only weight was found to be significantly lower in the hypertensive-IUGR group, versus the normotensive IUGR children. No significant differences were found in any of the neurocognitive parameters including IQ, school achievements, and neurodevelopmental score at age 9-10 years. CONCLUSION: IUGR is a well known risk factor for later cognitive difficulties but maternal hypertensive disorder does not seem to add significantly to this risk.

Personal and environmental pathways to participation in young children with and without mild motor disabilities.

OBJECTIVE: Participation in everyday activities has a positive influence upon health and well-being and is considered as an outcome measure. According to recent models child participation is the product of the dynamic interaction between health states and both individual and environmental factors. Children with mild developmental disabilities often present decreased participation in everyday activities. The purpose of this study was to explore the extent to which individual and environmental factors explain the participation of young children, with and without mild motor disabilities. METHODS: The study population included 58 kindergarten children together with their parents (29 children with mild motor disabilities who were referred to occupational therapy and 29 children without motor disabilities). Both groups of children were matched for: age; gender; age of parents; and socio-economic status (SES). We assessed participation using the Child Participation Questionnaire (intensity, diversity, independence, child enjoyment and parental satisfaction) and we assessed children's self-efficacy and motor abilities for individual factors. Parental self-efficacy and SES were collected by questionnaires. RESULTS: Participation diversity (number of activities) was predicted by child and mother self-efficacy and by SES. Child independence and enjoyment as well as parental satisfaction were predicted by child motor ability but mainly by maternal self-efficacy. Results suggest that the total explained variance is more than double when the environmental variables (parental self-efficacy and SES) are inserted to the participation model. CONCLUSIONS: Maternal self-efficacy and SES serve as facilitators to increased participation and well-being of children with mild motor disabilities.

Verbal short-term memory span in children: long-term modality dependent effects of intrauterine growth restriction.

BACKGROUND: Recent reports showed that children born with intrauterine growth restriction (IUGR) are at greater risk of experiencing verbal short-term memory span (STM) deficits that may impede their learning capacities at school. It is still unknown whether these deficits are modality dependent. METHODS: This long-term, prospective design study examined modality-dependent verbal STM functions in children who were diagnosed at birth with IUGR (n = 138) and a control group (n = 64). Their STM skills were evaluated individually at 9 years of age with four conditions of the Visual-Aural Digit Span Test (VADS; Koppitz, 1981): auditory-oral, auditory-written, visuospatial-oral and visuospatial-written. Cognitive competence was evaluated with the short form of the Wechsler Intelligence Scales for Children--revised (WISC-R95; Wechsler, 1998). RESULTS: We found IUGR-related specific auditory-oral STM deficits (p < .036) in conjunction with two double dissociations: an auditory-visuospatial (p < .014) and an input-output processing distinction (p < .014). Cognitive competence had a significant effect on all four conditions; however, the effect of IUGR on the auditory-oral condition was not overridden by the effect of intelligence quotient (IQ). CONCLUSIONS: Intrauterine growth restriction affects global competence and inter-modality processing, as well as distinct auditory input processing related to verbal STM functions. The findings support a long-term relationship between prenatal aberrant head growth and auditory verbal STM deficits by the end of the first decade of life. Empirical, clinical and educational implications are presented.

Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures.

Five young children developed slowly progressive hemiparesis as the initial manifestation of Rasmussen encephalitis (RE). Three have remained seizure free over an observational period of 1.3-1.9 years. In the remaining two patients, seizures occurred after 0.5 and 0.6 years respectively. We suggest that RE might be presently underdiagnosed and should be suspected in cases of new onset hemiparesis. In this series, three out of five patients showed oligoclonal bands on examination of cerebrospinal fluid (CSF) which represented additional diagnostic hints towards an immune-mediated condition. According to recently published formal diagnostic criteria, evidence of progressive cerebral hemiatrophy or bioptic identification of RE-typical inflammation confirms the diagnosis in such cases. Long-term immunotherapy is recommended in order to prevent further tissue loss and functional decline.

Failure of conservative treatment for lumbar spinal stenosis in elderly patients.

Spinal stenosis syndrome affects mainly patients at their 5th-6th decades of life. There is a strong debate in the literature whether patients with spinal stenosis should be operated or treated conservatively. Our aim was to evaluate patients with lumbar spinal stenosis who were treated conservatively. All patients over 65 years of age who were handled conservatively in the private clinic of the senior author due to spinal stenosis syndrome were evaluated. There were 21 males and 15 females aged between 65 and 88 years. Intermittent claudication was the dominant clinical presentation. The radiological examination showed a frequent narrowing at the level L4-L5. All patients had a severe stenosis of less than 10mm diameter of the spinal canal. All patients underwent a conservative therapy which included physical therapy with ultrasound waves, short waves, and flexion exercises. All patients were offered to undergo an epidural or nerve root injection. Twenty-four patients agreed also to have one of these procedures to relieve their symptoms. All patients were followed between 6 and 10 months after the initial diagnosis. We used the Oswestry index category for evaluation. In all parameters the conservative treatment failed to improve the symptoms in the vast majority of patients. None of the patients was very satisfied with the results of conservative treatment while 33 patients (92%) were not so satisfied or unsatisfied with the results. We conclude that conservative treatment for lumbar spinal stenosis is not a success for elderly patients. These patients should be convinced to undergo operative treatment.

Deficits in spatial orientation of children with intrauterine growth retardation.

The spatial orientation of intrauterine growth retarded (IUGR) children versus age-matched controls was examined using two spatial tests. The first test was the radial arm maze (RAM), a navigational test frequently used in animal models. The second test was a subtest from the Kaufman assessment battery for children (K-ABC). The IUGR group comprised 28 children aged 6 years. The control group comprised 29 appropriate-for-gestational age children. The performance of the IUGR children was significantly inferior to controls in both tests. In the RAM test, the ratio between the correct entrances to the total entrances was significantly lower in the IUGR group than in the control group (P<0.001). In the K-ABC, the IUGR group could not perform as well as control children (P<0.001). These results suggest that spatial orientation in IUGR children is inferior to their age-matched controls, possibly contributing to their potential learning difficulties. The present results also suggest that the RAM can be potentially used to test spatial orientation of children at-risk.

Prenatal diagnosis and management of intrauterine growth restriction: A long-term prospective study on outcome and maternal stress.

This study examines long-term effects of antenatal management of intrauterine growth restriction (IUGR) on developmental outcome and on maternal coping using a prospective cross-sectional design. Sixty-nine families were evaluated using psychological testing and risk questionnaires. The effects of timing of diagnosis (prenatal/perinatal) and of pregnancy management [induction of labor (IL)/conservative management (CM)/none, i.e., diagnosed-at-birth (DaB)] on maternal stress were tested at 6 years' postbirth. In general, prenatal management protocols of IUGR were efficient in preventing major disabilities; however, 49% of the variance in maternal stress at 6 years' postbirth could be attributed to the child's presenting behavior and to pregnancy management of IUGR condition. Mothers who received CM treatment reported being more stressed by their child's poor emotional adjustment (ps < .01-.002) and distractibility (p < .029), and to have more difficulty in accepting them (p < .01). Prenatal psychological consultation to better handle stress for parents whose fetus is diagnosed with IUGR is recommended, particularly when pregnancy is managed conservatively and familial-educational resources are low.

The first nonsense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings.

Eight mutations in the ALS2 gene have been described as causing autosomal-recessive juvenile-onset forms of the motor neuron diseases amyotrophic lateral sclerosis, primary lateral sclerosis and hereditary spastic paraplegia. All mutations are small deletions that are predicted to result in a frameshift and premature truncation of the alsin protein. Here we describe a ninth ALS2 mutation, in two siblings affected by infantile-onset ascending spastic paraplegia with bulbar involvement. This mutation is predicted to result in the substitution of an amino acid by a stop codon, and thus is the first nonsense mutation detected in this gene. It is probable that full-length alsin is required for the proper development and/or functioning of upper motor neurons.

Harvesting bone graft from the posterior iliac crest by less traumatic, midline approach.

Complications of the donor site after the harvest of corticocancellous bone graft from the posterior iliac crest are very common. The most common are chronic donor site pain, tenderness, and sensory disturbances. This study investigates the results of the midline, lumbar fascia splitting approach for harvesting bone graft in lower lumbar spine fusion and compares them with the classic separate incision approach. A retrospective study of 107 patients compares two groups. The first group of 56 patients (35 males and 21 females with an average age of 41.8 years) had bone graft taken by splitting the two layers of the lumbar fascia down to their attachment to the iliac crest. The second group of 51 patients (29 males and 22 females with an average age of 43.7 years) had a separate incision over the iliac crest. In the first group, 82.1% had no tenderness, 8.9% mild, 7.1% moderate, and only 1.8% severe tenderness over the donor site. In the second group, 45.1% had no tenderness, 21.6% mild, 17.6% moderate, and 15.7% severe tenderness over the donor site. Five patients of the separate incision group (9.8%) had a lump in the donor site compared with none in the "same incision" group. Sensory disturbances over the donor site were found in 5.4% of the first group and in 21.6% of the second group. Harvesting bone graft from the posterior iliac crest for lower lumbar spine fusion through a midline, fascia splitting approach was found superior to the traditional, separate incision approach.

Surgical treatment of lumbar spinal stenosis in patients aged 65 years and older.

Spinal stenosis syndrome affects mainly patients at their 5th-6th decades of life. The main goals of surgical treatment in the elderly are to allow the individual to walk longer distances, maintain the activities of daily living (ADL) and social life. Our aim was to evaluate the results of surgical treatment for lumbar spinal stenosis in elderly patients. All patients over 65 years of age who underwent surgery due to spinal stenosis syndrome between 1990 and 1998 were evaluated. There were 29 males and 17 females aged between 65 and 90 years. The clinical presentation included low back pain (89%), intermittent claudication (100%) and neurological involvement (87%). The radiological examination showed a frequent narrowing at the level L4-L5 in 93.5% of the patients. The results of the surgery in a mean follow-up of 22 months were good to excellent in 80% of the patients, fair in 11%, and poor in 9%. An improvement in the intensity of pain and in walking distances was noted in 89 and 85% of the patients, respectively. Improvement was achieved in the level of daily activity and in social lives in 57 and 61%, respectively. Major and minor complication rates were 6.5 and 19.5%, respectively. No mortality was noted in this series. Eighty-seven percent of the patients were satisfied with the results of the surgery. We conclude that Surgery for spinal stenosis is a successful and relatively safe procedure, also for patients aged over 65, and should be considered as a treatment option for these patients.

Blood pressure values in children with intrauterine growth retardation.

BACKGROUND: Low birth weight has been shown to be strongly related to hypertension in adult life. OBJECTIVES: To determine whether blood pressure is higher in children with intrauterine growth retardation than in control subjects. METHODS: Blood pressure was measured in 58 children aged 4-6 years with IUGR and in 58 age-matched controls. The control children, whose birth weight was appropriate for gestational age, were also matched for gestational age. RESULTS: The children with IUGR had significantly higher mean values of systolic (P < 0.05) and diastolic blood pressures (P < 0.05) and mean arterial pressure (P < 0.05). Significant differences in blood pressure values were found between preterm IUGR (n = 21) and preterm controls (P < 0.05). CONCLUSIONS: These data indicate that children with IUGR may be at higher risk of hypertension already in childhood.

The effect of intrauterine growth restriction on the development and health of children.

The neurodevelopmental and cognitive outcome of long-term Intrauterine Growth Restriction (IUGR) has been followed up from pregnancy to school age at the Tel Aviv Child Development Centre.

Unprovoked seizures after complex febrile convulsions.

Children with complex febrile convulsions bear a higher risk of developing epilepsy than children with simple febrile convulsions. Complex febrile convulsions are defined by the presence of prolonged seizures, partial seizures and multiple seizures occurring during the same day. The aim of this study is to delineate the relative significance of each of the three criteria defining complex febrile convulsions. Fifty-seven out of 477 children (12%) admitted for febrile convulsions had complex febrile convulsions and normal neurological examination. Follow-up was available for 48 (84%) of them. Thirteen of these 48 (27%) had epilepsy at follow-up. The mean age of seizure onset among the patients with subsequent afebrile seizures was significantly lower than the rest (10.8 months versus 16.8 months). The patients with partial febrile convulsions showed a trend toward a higher risk (45%) of developing epilepsy than the patients with multiple febrile convulsions (21%).

Mental retardation subsequent to refractory partial seizures in infancy.

Whether seizures are the direct cause of cognitive deterioration in epileptic children is undetermined. This retrospective study aimed to delineate a subgroup of pediatric patients with cognitive deterioration and refractory seizures in the absence of recognized causes for mental retardation. Of the 80 children identified as having mental retardation and refractory seizure disorder, seven (8.7%) had normal cognitive development until at least 1 year of age. Their metabolic status was normal. Five of them suffered repeated frequent partial seizures with onset in the first year of life and two had repeated episodes of status epilepticus. All seven had similar characteristics of early onset partial seizures, six of them had partial seizures secondarily generalized and one had complex partial seizures. The time of peak cognitive deterioration correlated with increases in seizure frequency during that period. Evaluation revealed a well-defined epileptic focus in the absence of neuroimaging abnormality except for hippocampal atrophy in the two children with complex partial seizures and a small vascular malformation in one child. Uncontrolled partial seizures in the first months of life may result in cognitive deterioration.

Six-year follow-up of children with intrauterine growth retardation: long-term, prospective study.

This prospective study was designed to characterize the neurodevelopmental and cognitive difficulties specific to children with intrauterine growth retardation and to detect early clinical predictors of these difficulties. Eighty-one children with intrauterine growth retardation were monitored up to 6 to 7 years of age using biometric parameters, perinatal risk questionnaires, and detailed neurodevelopmental and cognitive assessments. Forty-one children served as age-matched, appropriate for gestational age controls. A significant difference in growth parameters (P < .001), neurodevelopmental score (P < .05), and IQ (P < .05) was found between the children with intrauterine growth retardation and controls. A specific profile of difficulties in coordination, lateralization, spatial and graphomotor skills, and abundance of associated movements is typical of the children with intrauterine growth retardation and hints at possible later learning disabilities. The clinical parameters best predicting neurodevelopmental outcome were the neonatal risk score (P < .05) and the weight and height at 6 years of age (P < .05). The children with intrauterine growth retardation with neonatal complications had lower neurodevelopmental scores than the controls but no difference in IQ. Intrauterine growth retardation children diagnosed prenatally had the same neurodevelopmental and IQ scores as those diagnosed at birth, probably due to the careful perinatal and obstetric care provided. Children with intrauterine growth retardation demonstrate a specific profile of neurodevelopmental disabilities at preschool age. Early diagnosis and intervention could probably reduce these difficulties to a minimum.

Neurodevelopmental outcome in children with intrauterine growth retardation: a 3-year follow-up.

The study was designed to detect early clinical predictors of developmental outcome in children with intrauterine growth retardation. Eighty-five children with intrauterine growth retardation were followed up prospectively to 3 years of age, using biometric parameters, perinatal risk questionnaires, and neurodevelopmental evaluations. Forty-two children served as controls. A significant difference in neurodevelopmental score at 3 years of age was noted between the intrauterine growth retardation and control groups (P < .001). In the intrauterine growth retardation group, the clinical parameters that most significantly correlated with outcome were cephalization index (head circumference:birthweight ratio), neonatal risk score, and birthweight. The best predictor of 3-year outcome was the cephalization index (P < .01). The children with intrauterine growth retardation with neonatal complications had significantly lower IQ scores (P < .05) and a poorer neurodevelopmental outcome (P < .01) than those without complications. Children with intrauterine growth retardation are at higher risk for developmental disabilities than are controls, especially in the presence of neonatal complications and a high cephalization index.