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Biomedical subjects

V Gross-Tsur

Publications and source records attributed to V Gross-Tsur.

At least 37 records · Page 2Linked to original sources
PubMed1995

Familial microcephaly with severe neurological deficits: a description of five affected siblings.

Autosomal recessive microcephaly is usually characterized by normal developmental milestones and minor neurological deficits. In this report, we describe five siblings in one family with marked microcephaly, intractable seizures, quadriplegia and profound mental retardation. The recurrence risk of microcephaly when associated with devastating neurological deficits, as exemplified by this family, may be high and in such cases, the role of appropriate genetic counseling is of utmost importance.

Child↗
PubMed1995

Developmental dyscalculia behavioral and attentional aspects: a research note.

Behavioral characteristics of 140 children with developmental dyscalculia (DC) were evaluated using the Child Behavior Checklist. DC children demonstrated more behavior problems than normal children but significantly fewer problems than children psychiatrically referred. DC children had significantly more attentional problems although they had normal levels of anxiety/depression. Significantly higher scores on all syndrome scales were found for DC children who had attentional problems in the clinical range. When associated with dyslexia and a low verbal IQ, DC children had more attentional problems and externalizing syndromes. The implications of the behavioral characteristics of DC are discussed.

Anxiety↗
PubMed1995

Sustained attention, activation and MPH in ADHD: a research note.

Sustained attention was studied in children with attention deficit hyperactivity disorder (ADHD) and normal controls using a continuous performance task with slow presentation of stimuli and carried out with an experimenter-present and absent condition. Children with ADHD were slower than controls, with performance deteriorating over time, particularly in the experimenter-absent condition. Both the slowness and deterioration normalized when the children received MPH. Hence the rate of stimuli presentation and the presence or absence of experimenter are both crucial factors in the performance of children with ADHD. MPH is able to ameliorate the performance decrement seen under these conditions.

Attention↗
PubMed1995

Developmental right-hemisphere syndrome: clinical spectrum of the nonverbal learning disability.

We report the clinical characteristics of the developmental right-hemisphere syndrome (DRHS), a nonverbal learning disability, in 20 children (9 girls and 11 boys; mean age = 9.5 years) who also manifested attention-deficit/hyperactivity disorder (ADHD), severe graphomotor problems, and marked slowness of performance. Diagnostic criteria for this study included (a) emotional and interpersonal difficulties; (b) paralinguistic communication problems; (c) impaired visuospatial skills, verbal IQ > performance IQ, and verbal IQ > or = 85; and either (d) dyscalculia or (e) neurological signs on the left side of the body. In this group, verbal IQ was significantly higher than performance IQ (106.6 +/- 13.0 vs. 85.1 +/- 13.1, respectively, p < .01). Arithmetic was the lowest score among the verbal subtests (7.8 +/- 3.5, p < .01) and Geometrical Design was the lowest score among the performance subtests (5.8 +/- 1.7). Thirteen children had soft neurological signs on the left side of the body. ADHD was seen in all 20 children, marked slowness of performance in 16, and severe graphomotor problems in 18. The latter two features have not been previously described as part of DRHS.

Adolescent↗
PubMed1993

Protracted clinical course for patients with Canavan disease.

Before the establishment of N-acetylaspartic aciduria due to aspartoacylase deficiency as the cause of Canavan disease, diagnosis was based on the characteristic clinical features and spongiform encephalopathy, a pathological response shared by a number of other unrelated conditions. Thus confusion exists in the literature about the phenotype of spongiform encephalopathy (Canavan disease), with reports of 'juvenile' and 'congenital' forms, as well as the classical infantile type. In this report, six of 22 patients with infantile-onset Canavan disease survived beyond six years of age. This phenotypical pattern might be the result of better medical management and care, rather than evidence of genetic heterogeneity.

Adolescent↗
PubMed1993

The acquisition of arithmetic in normal children: assessment by a cognitive model of dyscalculia.

Developmental dyscalculia (DC) is a learning disability affecting the acquisition of arithmetic skills. The authors studied the normal developmental of arithmetic function of 200 normal children in grades 3 to 6. Number comprehension and production were well established by grade 3. With age and experience, children became more adept at addition, subtraction, multiplication and division for both number facts and complex exercises. The scores did not display a Gaussian distribution, but were negatively skewed. Low scores on the battery correlated well with the teacher's rating of the child's arithmetic knowledge, but not with reading ability. An estimate of the fifth centile was calculated. On the basis of these results, the authors conclude that this battery assesses number knowledge. Use of the fifth centile as a cut-off point will aid in identification of children with DC.

Child↗
PubMed1993

Convulsive status epilepticus in children.

Status epilepticus (SE) occurs most commonly in infancy and childhood. Children with prior neurological abnormalities are most susceptible. More than 90% of cases are convulsive and the majority are generalized. SE may occur in the setting of an acute illness, in patients with established epilepsy or as a first unprovoked seizure. The etiology can be classified as idiopathic, remote symptomatic, febrile, acute symptomatic, or associated with a progressive encephalopathy. The morbidity and mortality of status have dramatically declined in recent years. Overall mortality in recent pediatric series was 3-10%, with almost all fatalities associated with acute central nervous system insults or progressive neurologic disorders. Neurological sequelae in children with idiopathic or febrile status are rare. Neurologically normal children with SE as their first unprovoked seizure have the same risk of experiencing subsequent seizures of any type as children who present with a brief first seizure. The risk of recurrent episodes of convulsive SE approaches 50% in neurologically abnormal children but is very low in neurologically normal children. The favorable outcome of SE in children may be related to advances in therapy and to the resistance of the immature brain to damage from seizures.

Adult↗
PubMed1993

Intravenous high-dose gammaglobulins for intractable childhood epilepsy.

Immunological mechanisms have been implicated in the pathogenesis of epileptic seizures in some patients and in experimental animal models of epilepsy. A beneficial effect of high dose intravenous gammaglobulin (IVIG) has been demonstrated for some children with intractable epilepsy. In this study we treated 9 children ages 1.1-9.2 years (mean 5.0 years) with intractable epilepsy not responsive to conventional antiepileptic drugs (AEDs) and steroid therapy. Eight children had Lennox-Gastaut syndrome and 1 had complex partial seizures with secondary generalization. Each child received 3 doses of IVIG (200 mg/kg of polyvalent immunoglobulin) on Days 1, 15 and 36. Concomitant AEDs were not changed. Four children had complete remission, 3 had partial response with a more than 50% reduction in seizure frequency and 2 had no response. Onset of response varied from immediate to 7 months after the last injection. No toxicity was noted. Duration of remission was 9 months in 1 case. The other 3 cases have remained in remission to date with a follow up period of 22-26 months. We conclude that IVIG is a safe therapy which appears to be effective in some children with intractable seizures. Children with shorter duration of their seizure disorder (< 1 year) and relatively preserved cognitive function (IQ > 70) appear to have a more favorable response. Larger scale controlled trials are needed to determine the optimal timing and dosage, as well as to identify specific subgroups which may benefit most from IVIG treatment.

Child↗
PubMed1993

Developmental dyscalculia and medical assessment.

Developmental dyscalculia (DC) is a primary cognitive disorder of childhood manifested by disturbance of arithmetic ability. As an isolated learning disability (LD), it is usually treated by remedial education and not referred for further medical evaluation. We examined a group of 7 third-grade children with DC attending a mainstream school who had not progressed academically in spite of specific special education intervention. We were able to identify in all 7 children neurological conditions that had direct bearing on the children's cognitive disabilities and remedial programs. One child had petit mal seizures, another developmental Gerstmann syndrome, a third had dyslexia for numbers, and 4 children had attention deficit disorders without hyperactivity. Based on this experience, we suggest that the indications for medical or neurological assessment be broadened to include children who are not improving academically in spite of appropriate professional intervention.

Attention Deficit Disorder with Hyperactivity↗
PubMed1992

A 2-year prospective study of very low birthweight infants.

A prospective 2-year follow-up study was carried out on 68 of the 69 surviving very low birthweight (VLBW) infants (< 1,501 g) born in Bikur Holim Hospital in the years 1985-87. The aims were a) to determine the incidence of major disability, and b) to compare the 2-year outcome of VLBW infants without major disability with that of a control group of full-term small-for-gestational-age infants, using the Mental Development Index (MDI) of the Bayley Scales. Mean birthweight of the VLBW infants was 1,234 +/- 216 g and mean gestational age was 30.7 +/- 2.4 weeks. Their neonatal mortality during the study period was 29.8%. Major disability was diagnosed in 11/68 infants (16%). At age 2 years there was no significant difference between the mean MDI of the VLBW infants without major disability (97.7 +/- 19.5) and that of the controls (99.7 +/- 17.0). These data, representing the outcome of VLBW infants from a community-based hospital with neonatal intensive care facilities, are comparable in incidence of major disability with data of large tertiary centers. Cognitive ability of VLBW infants without major disability at age 2 years was equivalent to that of their full-term peers.

Analysis of Variance↗
PubMed1990

Cardio-facio cutaneous syndrome: neurological manifestations.

Cardio-facio-cutaneous (CFC) syndrome is a not uncommon syndrome with a characteristic face, mental retardation, abnormal skin and hair and congenital heart disease. We report the 16th case of this syndrome and give details of the spectrum of neurological manifestations in the cases so far reported.

Abnormalities, Multiple↗