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Biomedical subjects

D Benjamins

Publications and source records attributed to D Benjamins.

10 recordsLinked to original sources
PubMed2001

Myopathy with muscle spindle excess: A new congenital neuromuscular syndrome?

An infant presented with congenital weakness, hypotonia, arthrogryposis, atrial tachycardia, and a left intra-abdominal neuroblastoma. Muscle biopsy revealed marked excess of muscle spindles with atrophy of extrafusal fibers. The patient expired at age 14 months from progressive cardiorespiratory failure. Postmortem examination demonstrated muscle-spindle excess in other muscles, along with hypertrophic obstructive cardiomyopathy and organomegaly. Muscle spindle excess has previously been reported in two patients with Noonan syndrome and progressive hypertrophic cardiomyopathy. Muscle spindle excess with hypertrophic cardiomyopathy, organomegaly, and, possibly, congenital neuroblastoma suggests a syndromic association and may represent an unusual form of congenital myopathy.

Abnormalities, Multiple↗
PubMed1993

The influence of disabling condition visibility on family functioning.

Examined whether the level of family functioning and the components contributing to adaptive family functioning differed in families of visibly handicapped children (cerebral palsy) when compared to families of nonvisibly disabled children (diabetes). Other factors included effect of disability severity on family functioning, comparison of families of disabled children to families of able-bodied children, and comparison of mothers' and fathers' perceptions of family functioning. The sample comprised 139 two-parent families of children with cerebral palsy (n = 48), diabetes (n = 46), and able-bodied children (n = 45) (all 5-11 years old). Results showed that neither visibility nor severity of disability differentially impacted family functioning. Furthermore, families of the disabled children exhibited high levels of family functioning which were similar to control families. Differences were not found between the ways mothers and fathers perceived family functioning.

Attitude to Health↗
PubMed1991

Five cases of DeMyer sequence: an interophthalmic dysplasia.

Five cases with vomero-septal-prolabium agenesis, four with premaxillary agenesis, and one with hemipremaxillary agenesis are presented. All five patients had complete clefts of the secondary palate. No known family history for craniofacial dysmorphia was reported. Height and weight ranged from 2nd to 10th centile. Psychological testing showed intelligent quotients of 121, 93, 72 for three patients; two were microcephalic with undetermined IQ, but severe generalized developmental delay. CT scans for the patient with the IQ of 72 and for one of the two microcephalic subjects showed normal brain structures. The other microcephalic patients had midline prosencephalic dysgenesis. Cephalometric analysis of the cranial base and interorbital dimension using normal standards and self indexing showed hypotelorism and small anterior cranial bases to be apparently related to reduced size of the sphenoid. An intact ethmoid, nasal bone, and crista galli appear to represent key anatomic differences in these patients as compared to classical holoprosencephaly sequence subjects. It was suggested that this pattern of midline facial agenesis may range from a solitary central incisor to the most severe variant presented. Using the embryologic classification, these subjects fall best under the heading of craniofacial dysplasia rather than cerebral craniofacial dysplasia, as a different type of interophthalmic dysplasia or DeMyer sequence.

Cephalometry↗
PubMed1989

Writing posture in right hemiplegic children.

The handwriting posture of 29 children with congenital right hemiparesis was classified according to standard criteria. All 29 children, who ranged in age from 4 to 14 years, were classified unambiguously as noninverted writers. The prevalence of noninverted writing in this sample differs significantly from that reported for the general population of children. The data thus support the claim that neural factors contribute to handwriting posture.

Adolescent↗
PubMed1982

The possible existence of phonemic reading in the presence of Broca's aphasia: a case report.

This case study examined phonemic reading and reading comprehension in the presence of Broca's aphasia. The patient, T.B., a 16 yr old male, suffered an occlusion of the middle cerebral artery. Spontaneous speech was limited, naming difficulties were apparent, and comprehension was mildly impaired. Though the patient could read only a small number of words aloud, he was able to read silently for comprehension on a standardized instrument. Moreover, T.B. was able to match orally-presented pseudowords to their written analogs, a finding which suggests that phonemic processing for nonlexical material was to some degree intact. The results were contrasted to the reading patterns usually reported to be present in "frontal" alexia and deep dyslexia. The possible existence of a dissociation between phonemic processing in reading and speech behavior as well as its mechanism was discussed.

Adolescent↗
PubMed1981

The relationship among handedness, sighting dominance, and acuity dominance in elementary school children.

The present study was an attempt to clarify the relationship between handedness, sighting dominance, and eye-acuity in children. Ninety-four males and ninety-seven females between the ages of five and eleven were assessed on standardized measures of handedness, sighting dominance, and visual acuity. Right-handers were more likely to show right-sighting dominance, whereas left-handers had an equal chance of being right-sighted or left-sighted. Acuity dominance was not consistently associated with handedness or sighting dominance. No effects for sex or age were found. It is suggested that further clarification of the function of various lateralized sensorimotor measures is needed before an understanding of how these measures may be related to cortical dominance is possible.

Child↗
PubMed1978

Neurological and intellectual sequelae of Reye's syndrome.

Eleven survivors of Reye's syndrome ranging in age from 9 months to 12 years were evaluated for neurological and psychoeducational sequelae. Seven children showed significant neuropsychological sequelae, ranging from severe global psychomotor retardation to mild specific perceptual and/or language impairments and from spastic quadriplegia and decorticate posturing to mild dysarthria. The severity of sequelae was inversely related to age of the child at onset of the disease. Whereas those children developing the syndrome when they were less than 1 year of age were seriously impaired, 3 children developing it in late childhood sustained no sequelae. Biochemical and neurological status at disease onset did not predict neuropsychological outcome. The results parallel the pattern of sequelae for other encephalopathies and suggest the importance for both child and family of early developmental evaluation following recovery from the disease.

Child↗