Biomedical subjects

L E Becker

Publications and source records attributed to L E Becker.

At least 199 records · Page 11Linked to original sources

PubMed1984

A rare cause of spinal cord compression in childhood from intraspinal mesenchymal chondrosarcoma. A report of two cases and review of the literature.

Two children presented with acute spinal cord compression by primary and metastatic intraspinal mesenchymal chondrosarcoma, a rare pediatric malignancy. Patients with the primary intraspinal tumor usually present early and often respond well to combined surgery, irradiation and chemotherapy. Patients with intraspinal metastases present late in the course of their disease and their prognoses are poor.

Adolescent↗

PubMed1984

Synaptic development in the human fetus: a morphometric analysis of normal and Down's syndrome neocortex.

Postmortem tissue was obtained from six normal and four Down's syndrome brains ranging in age from 12 to 40 weeks postconception. Tissue was processed for electron microscopy using routine osmium and EPTA staining procedures, and to examine synaptic development, photomicrographs were systematically taken throughout the molecular layer of the sensorimotor neocortex. The number of EPTA-stained synapses were consistently greater than the number of osmium-stained synaptic contacts. A progressive increase in synaptic density throughout the range of ages examined was observed for both normal and Down's syndrome tissue. There was also an increase with developmental age in apparent measures of synaptic maturity, e.g., an increased ratio of mature to primitive contacts and asymmetrical to symmetrical contacts. In normal tissue, pre- and postsynaptic membrane lengths were observed to increase with the ages studied, whereas synaptic membrane widths appeared to attain mature values by 29 weeks postconception. Cleft width remained fairly constant to 28 weeks postconception. Although direct statistical comparisons could not be made, in Down's tissue synaptic parameter development was generally less consistent and the parameters appeared to be reduced during the later stages of development studied.

Down Syndrome↗

PubMed1984

Dendritic development in human occipital cortical neurons.

Using quantitative techniques on rapid Golgi impregnations, the dendrites from neurons of visual cortex from 14 infants ranging in age from 20 weeks gestation to 7 years were assessed. Neurons in layers 3 and 5 were separately evaluated. The total dendritic length for all dendritic branches reached a maximum of 2800 microns for layer 3 neurons and 3400 microns for layer 5 neurons. At or before 40 weeks gestation, the dendritic tree of layer 3 neurons had reached 35% of the maximum compared with 55% of the maximum for layer 5 neurons. Dendritic branching occurred earlier in layer 5 and growth continued to be more advanced than in layer 3. After 40 weeks gestation in these layers no new branch orders were added to basal dendrites but 3 branch orders were added to apical dendrites. The determination of normal values for dendritic length, number of orders, and number of branches during early development provides a foundation for comparison of dendritic maturation in children with a variety of neurologic disorders.

Child↗

PubMed1984

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.

Autopsy↗

PubMed1984

Developmental changes of glial fibrillary acidic protein and myelin basic protein in perinatal leukomalacia: relationship to a predisposing factor.

Focal white matter necrosis is frequently seen in the brains of infants with perinatal cerebral hypoperfusion. Periventricular leukomalacia (PL) occurs in the deep white matter of premature and term neonates and subcortical leukomalacia (SL) in the subcortical white matter of young infants. Using immunoperoxidase methods in normal infants, glia positive for glial fibrillary acidic protein (GFAP) were found first in the deep zones of white matter and with increasing age they became more prominent in the subcortical zone. They increased diffusely in the deep or subcortical zones of the cases of PL or SL, respectively. The number of myelin basic protein-positive glia is much larger than that of GFAP-positive glia in the cases of old PL. These findings suggest that an increased number of positive glia may be a reaction to hypoxic, ischemic, or toxic insults, or this shifting, transient increase of positive glia in cerebral white matter may be one of several predisposing factors leading to perinatal leukomalacia. Furthermore, positive staining of GFAP and MBP for reactive astrocytes in old PL suggests that at a certain stage of gliogenesis both GFAP and myelin basic protein may be present within the same cell.

Bronchopulmonary Dysplasia↗

PubMed1984

Developmental neuropathology in bronchopulmonary dysplasia: alteration of glial fibrillary acidic protein and myelination.

Developmental changes of glial fibrillary acidic protein (GFAP) and myelination were examined in the brains of 25 children with bronchopulmonary dysplasia (BPD). Widespread GFAP-positive, bouquet-shaped glia occurred in the cerebral white matter and medulla oblongata of most cases and increased during the perinatal period, independent of the presence of luekomalacia. Acceleration of myelination was suggested in five of 10 cases during the period of 40 to 48 weeks postconceptional age, just before myelin sheaths normally appear. However, in two of four cases seen during late infancy myelination was delayed. The increase of GFAP-positive glia may be related to the induction of myelination. On the other hand, increased astroglial processes may occur secondary to chronic hypoxia and may interfere with myelination, accounting for the poor myelination seen in late infancy.

Bronchopulmonary Dysplasia↗

PubMed1984

Fatal nemaline myopathy in infancy.

The clinical and neuropathological findings in two infants with congenital nemaline myopathy are described. One patient presented at birth with severe hypotonia, respiratory failure and contractures and died shortly after the neonatal period. The other presented at age two months with hypotonia and, following a period of clinical stability, died at age seven months from respiratory failure. Pathological findings in the fatal neonatal case revealed numerous rod bodies in lingual, pharyngeal, diaphragm and limb muscles, correlating with clinical findings. Significant, but less rod body involvement was found in the diaphragm and limb muscles of the second patient. Although a neural basis has been suggested for this disorder, no abnormalities were found in the central nervous system or in the peripheral nerves of these two severely affected patients.

Female↗

PubMed1984

Experience with pineal region tumours in childhood.

Sixty-one children with pineal region tumours were managed at the Hospital for Sick Children during the period 1950-1982. Histologic verification of these tumours was possible in forty-six children. Although germ cell tumours were the commonest form, germinomas only made up one third of the pineal region tumours. Since 1967, an aggressive approach has been adopted in the management of pineal region tumours at the Hospital for Sick Children with thirty-four patients having direct surgery on their pineal region mass out of a total of forty-one patients with such tumours. The operative mortality for pineal tumour surgery has steadily fallen so that the operative mortality between 1975 and 1982 was 4.3% and there have been no operative deaths since 1977 among the thirteen patients with pineal region tumours who have been operatively treated. Pineal tumours are operable lesions which should be removed if they are benign. Histologic confirmation should be obtained if they are malignant in order to provide for rational management of these lesions.

Adolescent↗

PubMed1984

Neurochemical abnormalities in a patient with ataxia-telangiectasia.

We describe biochemical abnormalities found in autopsied brain of a patient with ataxia-telangiectasia. Neuropathologic changes were limited to the cerebellum and spinal cord. The atrophic cerebellum showed marked loss of Purkinje's and granule cells, and moderate loss of stellate and basket cells. Glutamic acid content was markedly reduced, and taurine content somewhat reduced in the cerebellar cortex, while gamma-aminobutyric acid (GABA) content was greatly reduced in the dentate nucleus. GABA receptor binding was reduced by 70% in cerebellar cortex. Phosphoethanolamine content was greatly reduced in the cerebellar cortex and inferior olivary nucleus. This compound was also deficient in 10 other brain regions and was the only extracerebellar neurochemical abnormality observed.

Ataxia Telangiectasia↗

PubMed1984

Myxopapillary ependymoma of the filum terminale and cauda equina in childhood: report of seven cases and review of the literature.

Seven of fourteen children with spinal cord ependymoma had myxopapillary tumors of the filum terminale. These tumors made up 15.9% of all primary spinal neuroectodermal tumors in children (44 cases) seen during a 62-year period (1919 to 1981). Their clinical presentation, radiological features, pathological findings, treatment, and outcome are reported. Six of the seven patients were known to be alive at the time of writing. The seventh patient was lost to follow-up after 3 years without tumor recurrence. Of 5 patients whose primary mode of treatment was operation alone. 3 had intraspinal or intracranial recurrences. Despite tumor recurrences, 2 patients were long term survivors after further operation and irradiation, whereas the third patient recently received craniospinal irradiation for intracranial tumor recurrence. The 2 patients who did not have tumor recurrence after operation alone had been followed for 3 and 7 years, respectively. Two children with subtotal tumor resection and spinal irradiation had no recurrences at 1 and 17 years, respectively. Our data suggest that this unusual subtype of spinal ependymoma is not uncommon during childhood and has a good prognosis. All patients with this tumor require prolonged follow-up for tumor recurrence after operation and irradiation.

Adolescent↗

PubMed1984

Primary intracranial choriocarcinoma: a report of two cases and a review of the literature.

Among 848 cases of primary intracranial malignancy seen during a 63-year period at a pediatric hospital, there were 2 cases of primary pineal choriocarcinoma. The clinical and laboratory findings of these 2 cases were similar to those of 33 cases of intracranial choriocarcinoma reported in the literature. In 1 patient with precocious puberty, the diagnosis was confirmed by labeling human chorionic gonadotropin (HCG) within the tumor, which had been stored for 25 years. In the other patient, who is alive and well 18 months after diagnosis, the response to cranial irradiation and intensive chemotherapy was monitored with serial measurements of serum, cerebrospinal fluid, and urinary HCG and with computed tomography. We conclude that intensive chemotherapy, radiation, and tumor resection, if feasible, offer the best chance of curing this otherwise fatal disease.

Adolescent↗

PubMed1984

Lambdoid synostosis. Part 1. The lambdoid suture: normal development and pathology of "synostosis".

The microscopic development of the normal lambdoid suture was studied in autopsy specimens from 19 normal subjects ranging in age from 20 weeks' gestation to 60 years. The cellular activity at the suture varied considerably with age; however, maximal activity was seen in specimens approximately 3 months of age. There were several unusual features, including a high incidence of cartilaginous differentiation and the presence of intrasutural Wormian bones. Forty-one specimens from 37 patients with isolated lambdoid synostosis were also studied pathologically. Only three cases showed bone union across the suture, which appears to be a result of closure rather than fusion as in other synostoses. The remainder of the cases showed varying degrees of increased cellular proliferation at the suture line, resulting in exaggerated and prolonged sutural activity. Morphologically, this produced increased interdigitation and fibrous adhesion between the suture margins.

Adolescent↗

PubMed1984

Ultrastructure of carotid bodies in sudden infant death syndrome.

Recent studies have implicated an abnormality in carotid body structure and function in the pathogenesis of sudden infant death syndrome (SIDS). In the present investigation, the light and electron microscopic findings in carotid bodies from ten victims of SIDS were compared with those in six control infants and five infants dying of congenital heart disease. The cross-sectional area of carotid body chemoreceptor cells and the frequency, distribution, and size of neurosecretory granules were assessed morphometrically. The area of carotid body occupied by chemoreceptor cells (the functional area) was comparable in SIDS victims, control infants, and infants with congenital heart disease. By electron microscopy, the carotid body chief cells from all groups contained numerous electron-dense neurosecretory granules. Distribution, frequency, and size of neurosecretory granules in SIDS victims and control infants did not differ significantly. Morphology of carotid bodies from SIDS victims was found to be normal. The presence of neurosecretory granules in chemoreceptor cells of SIDS victims suggests that the cellular mechanism of neurotransmitter synthesis and storage is not altered.

Carotid Body↗

PubMed1983

S-100 protein in tumors of cartilage and bone. An immunohistochemical study.

Using the peroxidase-antiperoxidase (PAP) immunohistochemical method, S-100 protein was found in well-differentiated chondrocytes of chondroma, chondroblastoma, mesenchymal chondrosarcoma, and osteosarcoma. It was not detected in osteoma, osteoblastoma, giant cell tumor, and Ewing's tumor. The presence of S-100 protein in tumorous chondrocytes and chondroblasts suggests that this protein may be a marker of chondrocyte origin and should not be considered a specific marker for nerve tissue.

Adolescent↗

PubMed1983

Developmental changes of glial fibrillary acidic protein in cerebral white matter.

Developmental changes were observed in the glial fibrillary acidic protein (GFAP)--positive glia of cerebral white matter (including myelination glia) in the frontal lobes of 38 normal cases. With age, GFAP-positive processes gradually became larger and their location shifted from preponderantly in the deep white matter to mostly in the superficial white matter. This shift in pattern of GFAP-positive glia may be related to the parallel changes in perinatal brains in the localization of leukomalacic lesions. However, the shift is a normal developmental phenomenon on which such lesions are superimposed.

Aging↗

PubMed1983

Craniotelencephalic dysplasia in sisters: further delineation of a possible syndrome.

We describe two sisters with a complex of anomalies involving the cranium and brain. The changes in the former are consistent with those previously described as craniotelencephalic dysplasia and those in the latter indicate primary developmental abnormalities of the central nervous system including septo-optic dysplasia, absent olfactory nerves, agenesis of the corpus callosum, and lissencephaly. Per se, these cerebral malformations are causally heterogeneous, but their occurrence in association with craniotelencephalic dysplasia suggests that this combination is a distinct, probably autosomal recessive, syndrome.

Brain↗

PubMed1983

Subependymal giant-cell tumor: astrocytic or neuronal?

Tissue from seven patients with tuberous sclerosis and subependymal giant-cell tumors was examined with special stains, immunohistochemistry, and electron microscopy. Immunoreactive glial fibrillary acidic protein (GFAP) was not found in the giant cells of four tumors, but was present in some tumor cells in the other three. Immunoreactive S-100 protein was present in tumor cells of six cases; it was also seen in more tumor cells than was GFAP. Electron microscopy was similar in all cases and showed that the tumor cells had numerous organelles - many dense bodies thought to be primary lysosomes, swollen mitochondria, Golgi complexes, rough and smooth endoplasmic reticulum, free ribosomes, and sparsely distributed intermediate filaments. In one case, neurosecretory granules, microvilli, and synapses were observed. In another subject, prominent, thick bundles of glial filaments were seen. These findings suggest that the tumor is made up of unique cells in addition to cells with recognizable neuronal or astrocytic features.

Brain Neoplasms↗

PubMed1983

Hypoxic/ischaemic cerebral injury in the neonatal brain. A report of sonographic features with computed tomographic correlation.

Ultrasound has been used in 11 neonates whose history or clinical features suggested the possibility of hypoxic/ischaemic lesions. The ultrasound findings were correlated with computed tomographic findings in nine infants and with pathological findings in two. On ultrasound scan, areas of increased echoes represented both hypoxic/ischaemic and haemorrhagic lesions. However, the distinction between them could not be made with certainty. Cystic changes were shown clearly by ultrasound as were cerebral vascular pulsations in and adjacent to the areas of increased echoes. With computed tomography, hypoxic/ischaemic lesions were represented by areas of decreased density and haemorrhagic lesions by areas of increased density. Computed tomography failed to clearly demonstrate the cystic changes. Three types of lesions, viz. diffuse, focal and periventricular were based on the location of brain injury, the former two occurring in term infants and the latter in premature infants. Ultrasound has been shown to be of value for definition of the site and extent of hypoxic/ischaemic cerebral lesions in the newborn and for observation of their evolution.

Brain Ischemia↗