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Biomedical subjects

W Zeman

Publications and source records attributed to W Zeman.

At least 19 recordsLinked to original sources
PubMed1979

On the ultrastructural diversity and essence of residual bodies in neuronal ceroid-lipofuscinosis.

In 4 patients with neuronal ceroid-lipofuscinoses (NCL) (3 patients with the junvenile type, 1 patient with the late infantile type), the ultrastructural spectrum of residual bodies in the central and peripheral nervous system presented curvilinear profiles in all cases and regions investigated and many more ultrastructural patterns within and beyond regions commonly accessible to biopsy, probably due to age dependence, local tissue and cellular biochemical factors. Sampling from basal ganglia especially yielded combined curvilinear-fingerpint bodies, from peripheral ganglia additional membranous bodies. Residual bodies in NCL were present in almost every cell type, similar to the distribution of regular lipofuscin. Although the classical subgroups of NCL contain electronmicroscopically well defined residual bodies, permitting distinction of the late infantile type from the juvenile type, the ultrastructural differences are more of a quantitative than of a qualitative nature. However, they are not pathognomonic. N.m.r. spectra of ceroid and lipofuscin support the concept of their biochemical similarity, and argue against the proposition that they contain a single major component.

Brain↗
PubMed1979

Effects of high-LET neon (20Ne) particle radiation on the brain, eyes and other head structures of the pocket mouse: a histological study.

A study was made of tissues from 130 pocket mice after a single head-only exposure to high-LET 20Ne particle radiation at 1000, 100 or 10 rad (nominal surface dose) with the view of obtaining base"line data regarding the effectiveness of HZE (cosmic-ray) particles during spaceflight. First seen at 2-3 weeks after exposure, necrotic neurons in the cerebrum reached peak incidence (0 . 04 per cent at 1000 rad, 0 . 003 per cent at 100 rad and less than 0 . 0005 per cent at 10 rad) after 4-5 weeks and decreased to low levels thereafter. Incidence in the cerebellum was lower. Neuroglia, cells of the subependymal matrix and dentate gyrus precursor cells suffered acute damage at 1000 and at 100 rad. At 1000 rad, enlarged hyperchromatic neuroglia, first noted at 3 weeks, increased in number up to 7 months, then declined. Alterations in the retina and olfactory epithelium were seen at 1000 rad, and reaction in the scalp at 100 rad. Damage was incurred by dentinoblasts at 10 rad. Changes similar to those observed in pocket mice were found in the brains of gerbils and C57B1 mice.

Animals↗
PubMed1977

Hallervorden-Spatz syndrome.

Two siblings with Hallervorden-Spatz syndrome showed striking homotypism and homochronism. Neuropathologic examination and electron microscopic studies were done; neutron activation analysis showed an increase in the uptake of iron in the basal ganglia. Of particular relevance is the application of radioactive iron studies in the clinical course of this syndrome. These studies disclosed an increase in the uptake of iron in the area of the basal ganglia in one sibling and in another isolated patient. This procedure will be helpful toward the clinical diagnosis.

Axons↗
PubMed1977

An ultrastructural study of the retina in the Jansky-Bielschowsky type of neuronal ceroid-lipofuscinosis.

A 7 1/2-year-old boy died of the Jansky-Bielschowsky type of neuronal ceroidlipofuscinosis. Although an electroretinogram showed reduced activity, pigmentary retinal atrophy was not demonstrable by ophthalmoscopy two years before death. At autopsy, the retina had severe loss of photoreceptor cells, pigment displacement, gliosis, and uniformly structured lipopigment bodies with an internal structure of curvilinear profiles in ganglionic and Müller cells, in pigment epithelia, in the remaining photoreceptors, and in the elements of the inner nuclear layer. These findings conformed to those in the juvenile type of neuronal ceroid-lipofuscinosis, differing only as to the severity.

Child↗
PubMed1976

Ultrastructural investigations of peripheral nerves in neuronal ceroid-lipofuscinoses (NCL).

Specimens of brachial plexus, sural nerve and two cranial nerves of one patient with Jansky-Bielschowsky type and 3 patients with the Spielmeyer-Sjögren type of NCL were studied by electron microscopy. Significant light microscopic changes were absent in all specimens. Ultrastructurally, curvilinear and/or fingerprint inclusions were present in each case, located chiefly in Schwann cells. These diagnostic findings were, however, overshadowed by masses of lamellar pi-granule-like cytosomes, usually not mixed with curvilinear or finger-print profiles in the juvenile cases and only rarely associated with curvilinear profiles in the late infantile case. Since secondary changes of axons and myelin sheaths were mild, these lamellar cytosomes might indicate chronic damage to Schwann cells, perhaps by "wear and tear" as seen in aging as well as NCL. On account of the abundance of pi-granules in NCL, peripheral nerve biopsy appears less suitable for confirming this diagnosis than biopsy of skin, striated muscle and rectal tissue.

Humans↗
PubMed1976

Editorial: Post-therapeutic radiation injuries of the nervous system. Reflections on their prevention.

The introduction of the concept of Nominal Standard Dose and of Time, Dose Fractionation Factors ostensibly permits definition of tolerance doses for normal tissues in unequivocal terms. However, even with these refinements, tolerance doses remain, at best, guidelines, because radiobiologic effectiveness is governed not only by the effective dose, but also by individual factors, which will modify the response. Attention must be accorded to these biologic parameters, in order to prevent injury to healthy tissues. Of particular significance are the relative size or volume of the irradiated tissue, the possible presence of co-existing pathology in the exposed organ and the development of disease after tge termination of the treatment. Even if these factors are properly respected, the risk of radiation injury cannot entirely be eliminated. The radiotherapist is therefore obligated to use an approach which minimizes the exposure of the healthy nervous tissue, a goal which has become attainable with the advent of modern accelerators as radiation sources.

Humans↗
PubMed1976

Peripheral motor and sensory neuropathy of early childhood, simulating Werdnig-Hoffmann disease.

A five-year-old girl developed neurogenic muscular atrophy during infancy. On the basis of clinical findings and a muscle biopsy study a diagnosis of infantile spinal muscular atrophy, benign type II, was entertained. As the disease progressed, involvement of sensory pathways was detected by electrophysiological studies. Examination of a sural nerve specimen suggested a chronic polyneuropathy rather than spinal muscular atrophy. These findings were confirmed at autopsy by demonstrating severe loss of myelinated axons in ventral and dorsal spinal roots, peripheral nerves and dorsal columns of the spinal cord. In addition, islands of astroglial fibers were found in ventral and dorsal spinal roots, regarded as a secondary reaction to the breakdown of myelinated axons. This unusual scarring process seems to result from nerve fiber loss during the perinatal period, since radicular glial scar tissue is not known to occur in the spinal muscular atrophies of later onset. Examination of the sensory nervous system in patients with Werdnig-Hoffmann disease appears mandatory to clarify the precise disease entity leading to infantile neurogenic muscular atrophy.

Child, Preschool↗
PubMed1975

Enzymuria in gentamicin-induced kidney damage.

To assess their potential value as early indicators of gentamicin-induced kidney damage, lysosomal hydrolases were measured in the 24-h urines of rats receiving 30 or 60 mg of gentamicin per kg per day for 15 days. Proteinuria, urine osmolality, blood urea nitrogen, and creatinine clearance were also measured. Kidney tissue was examined by both light and electron microscopy. Beta-galactosidase, beta-n-acetyl-hexosaminidase, and alpha-fucosidase were sensitive indicators and were significantly elevated above control values by day 3 at both doses (P < 0.01). Proteinuria, urine osmolality, and tests reflecting glomerular filtration rate were later indicators of nephron damage. Changes by light microscopy were detected on day 5. Necrosis was most prominent in the proximal convoluted tubules on day 10. Electron microscopy revealed numerous cytosomes with myeloid bodies within the proximal tubular epithelium on day 5. Lysosomal enzymuria appears to be an early manifestation of gentamicin nephrotoxicity and may possibly be related to the lysosomal abnormalities seen on electron microscopy.

Animals↗
PubMed1975

Significance of muscle biopsies in neuronal ceroid-lipofuscinoses.

Muscle specimens obtained at necropsy from four cases of neuronal ceroid-lipofuscinosis (NCL), three of the juvenile and one of the late infantile type, and a muscle biopsy from a fifth patient with the juvenile type of NCL, all showed curvilinear bodies typical of NCL within the muscle fibres. The pigments were autofluorescent. It appears that skeletal muscle is a reliable tissue source for the diagnosis of these disorders by biopsy.

Adolescent↗
PubMed1975

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: III. Dosimeter design, construction, and implantation.

To detect the passage of cosmic ray particles through the heads of the pocket mice during the Apollo XVII flight, a "monitor" (dosimeter) composed of plastics was prepared and implanted under the scalp. The monitor was mounted on a platform, the undersurface of which fitted the contour of the skull. Numerous tests were run to assure that the presence of the monitor assembly beneath the scalp would be compatible with the well-being of the mice and that the capacity of the monitor to detect the traversal of cosmic ray particles would be preserved over the several weeks during which it would remain under the scalp.

Animals↗
PubMed1975

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: VI. launch, flight, and recovery.

The final phase to fly five pocket mice in the Apollo XVII command module was carried out at the NASA Kennedy Space Center. Upon completion of the 13-d space flight, the package was removed from the spacecraft and, after having been purged with an oxygen-helium gas mixture, was flown to American Samo. Four of the five mice were recovered alive from the package. Analysis of the mouse that died during the flight revealed several factors that could have contributed to its death, the chief of which was massive hemorrhage in its middle ear cavities.

Animals↗
PubMed1975

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: IX Results of examination of the nasal mucosa.

The olfactory epithelium, but not the nasal respiratory epithelium, of the four pocket mice (Perognathus longimembris) that survived their flight on Apollo XVII showed both diffuse alterations and numerous disseminated focal lesions. The olfactory mucosa of the mouse that died during flight was also affected, but to a minor degree insofar as could be determined. All this was in contrast to the normal appearance of the olfactory mucosa of the numerous control animals. A number of possible causes were considered: systemic or regional infection; inhaled particulate material (seed dust); by-products from the KO2 bed in aerosol or particulate form; gas contaminants originating in the flight package; volatile substances from the dead mouse; weightlessness; and cosmic ray particle radiation. Where feasible, studies were conducted in an effort to rule in or rule out some of these potentially causative factors. No definitive conclusions were reached as to the cause of the lesions in the flight mice.

Aerosols↗
PubMed1975

The effects of cosmic particle radiation on pocket mice aboard Apollo XVII: X. Results of ear examination.

In the five pocket mice flown on Apollo XVII, no evidence was found that the inner ear had been damaged, though poor fixation precluded detailed study. On the other hand, the middle ear cavity was involved in all the mice, hemorrhage having occurrred in response to excursions in pressure within the canister that housed the mice during their flight. The same occurred in flight control mice which had been subjected to pressure excursions of much the same magnitude. A greater degree of exudation into air cells and greater leukotaxis were noted in the flight animals than in the control animals. There was no increase in leukocyte population along the paths of the 23 cosmic ray particles registered in the subscalp dosimeters that traversed the middle ear cavities of the flight mice. The increased exudation and the greater response by leukocytes in the flight mice may have been causally related to the lesions found in their olfactory mucosa but there were no data in support of this possibility.

Animals↗