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Biomedical subjects

R Dom

Publications and source records attributed to R Dom.

At least 109 records · Page 6Linked to original sources

[Shulman's syndrome. Diffuse fasciitis with eosinophilia. A differential diagnosis from polymyositis (author's transl)].

A case of diffuse fasciitis with eosinophilia is reported. The clinical pattern of this disease has some resemblance with polymyositis. In 1974, Shulman described this syndrome as a new entity. The differential diagnosis of those inflammatory and sclerodermatous changes in the perimuscular fascia is considered. An histological, immunological and electrophysiological investigation has been performed. We propose an auto-immunological mechanism in the etiopathogenesis of this syndrome.

Autoantibodies↗

Electrical stimulation of the central gray for pain relief in human: autopsy data.

Anatomopathological studies are reported of 5 brains in which electrical stimulation of the periventricular--periaqueductal gray was applied for the treatment of chronic pain. It was found that the recommended target was reached in 4 cases (only 1 case out of 5 was successful as far as a pain relief is concerned). PtIr electrodes caused little tissue reaction. The chronic stimulation had not provoked lesions observable by light-microscopy.

Brain↗

Familial muscle cramps with autosomal dominant transmission.

A family is described with generalized muscle cramps inherited as an autosomal dominant trait and with maximal expression during adolescence. The age of onset varies between 10 and 15 years. Muscle enzymes are elevated with a peak level between 15 and 25 years. The complaints seem to disappear after the age of 25 years. EMG and muscle biopsies suggest a neurogenic origin of the cramps.

Adolescent↗

Catecholamine secreting tumor of the glomus jugulare.

A case of endocrinologically active glomus jugulare is presented. The localization within the tumor of the catecholamine hyperproduction was demonstrated by selective venous sampling from the vena jugularis externa draining the tumor. The norepinephrine/epinephrine ratio was high and of the same order in blood, urine and cerebrospinal fluid. Bioassay showed high levels of intratumoral norepinephrine and epinephrine. The ways of treatment and their influence on the catecholamines are briefly discussed. In every phaeochromocytoma-like syndrome both the existence of catecholamine hyperproduction and the anatomical site of this hypersecretion have to be precisely documented in order to define the correct treatment to be applied.

Catecholamines↗

Adult ceroid-lipofuscinosis (Kufs' disease) in two brothers. Retinal and visceral storage in one; diagnostic muscle biopsy in the other.

Two brothers developed a neurological condition characterized by homochrony and homotypy: the first symptoms in both were generalized epileptic seizures, occurring at about the same age (30 years in the elder, 32 years in the younger), followed by a cerebellar syndrome with myoclonic jerks and some extrapyramidal symptoms. The elder of the two boys died at the age of 33 years. Histology showed extensive storage of ceroid-lipofuscin in the central nervous system (curvilinear bodies), in hepatocytes, in heart muscle and in the retina. In the younger boy, still living, a muscle biopsy (peroneal muscle) revealed accumulation of membrane-bound osmiophilic inclusions with curvilinear profiles. Retinal storage in Kufs' disease has never been documented. Muscle biopsy as a diagnostic tool for Kufs' disease has not been reported.

Adult↗

Observations on the development of brainstem-spinal systems in the North American oppossum.

The North American oppossum is born 12 to 13 days after conception and and is available for 90 days or more in an external pouch where it can be observed and experimentally manipulated. It is of particular interest that the hindlimbs of the newborn opossum are very immature and remain immobile for a week or more after birth. Degeneration techniques reveal that immature brainstem axons are present within the marginal zone of the lumbosacral cord before hindlimb movements begin (our state I) and material processed for formaldehyde induced fluorescence shows that some of them transport monoamines. Several lines of evidence suggest that part of the fluorescent axons arise within the nucleus locus coeruleus. At this early stage the electron microscope reveals that all brainstem-spinal axons are small (0.1--0.4 micrometer in diameter) and unmyelinated. By the time random hindlimb movements can be observed (stage II), brainstem axons, including those transporting monoamines, can be demonstrated to have grown into limited areas of the intermediate zone of the lumbosacral cord and to arise from most of the areas contributing to them in the adult animal (horseradish peroxidase technique). Such axons are still immature and it is not yet clear that they have formed synaptic terminals. Brainstem axons continue to grow into the intermediate zone of the lumbosacral cord for some time and come to occupy all of their adult territories before thoracic transection produces obvious change in hindlimb motility (beginning of stage III). It is still another 20 days or so before thoracic transection produces spinal shock comparable to that in the adult animal. The relatively mature use of the hindlimbs and the full expression of spinal shock correlate with changes in the technique and survival time needed to demonstrate degenerating brainstem axons in experimental material.

Animals↗

Maintenance treatment of chronic schizophrenic patients. A study with the long-acting thioxanthene derivative, cis(Z)-clopenthixol decanoate-sordinol depot.

The clinical effect of clopenthixol decanoate has been assessed in a 5-month controlled including 21 hospitalized chronic schizophrenic patients. The ratings were done with BPRS, NOSIE 30, the two psychological tests of WAIS and Grübaum, and the rating scale of Simpson & Angus to assess extrapyramidal side effects. Clopenthixol decanoate was found an effective and long-acting antipsychotic compound with few autonomic and neurological side effects. Compared with previous maintenance treatment it also showed a positive influence on depression and facilitation of the social adaptation of the patients.

Adult↗

Neuropathology of Huntington's chorea. Studies of the ventrobasal complex of the thalamus.

In seven cases of Huntington's chorea, the ventrolateral thalamus was studied by quantitative cytometry. A selective 50 percent atrophy of microneurons (internuncial cells) was found while the macroneurons did not show significant atrophy. Thalamic microneurons might be presynaptic and postsynaptic inhibitory cells. Their specific atrophy in Huntington's chorea thus could be related to the known decrease of gamma aminobutyric acid (GABA) in Huntington's chorea.

Adult↗

Progressive ponto-bulbar palsy with deafness. A clinico-pathological study.

Progressive ponto-bulbar palsy with deafness is a rare disease. It seems to be an abiotrophic process with autosomal recessive inheritance in most instances. Only one autopsy case had been briefly described (Lelong et al., 1941). The clinical features and the pathological findings of a new case are reported. The structures primarily involved are the grey matter of the brain stem and the spinal cord, including to some extent the optic tracts and most of the fiber tracts in the brain stem with exception of the pyramidal tracts.

Adolescent↗

The inferior olivary nucleus of the opossum (Didelphis marsupialis virginiana), its organization and connections.

Although the inferior olivary nucleus of the opossum is small, sections stained either for Nissl substance, normal axons or cholinesterase activity reveal distinct medial, dorsal and principal nuclei. The medial nucleus contains three major subdivisions (labelled a, b, c after Bowman and Sladek, '73) and a group of neurons which is comparable to the cap of Kooy. In contrast to the cat and monkey, the major portion of the "medial" nucleus (subgroup a) lies lateral to the principal nucleus in rostral sections. The dorsal nucleus can also be subdivided, as can the principal nucleus which contains distinct dorsal and ventral lamellae. A small area is identified which based on position and connections may conform to the dorsal medial cell group. The experimental portion of the study provides evidence for an olivary projection from the motor-sensory cortex and a massive input from the midbrain (red nucleus, pretectum, midbrain tegmentum). In addition, the opossum inferior olive receives fibers from the deep cerebellar nuclei (cerebellar feedback loops), the spinal cord and the dorsal column nuclei. Of particular interest is the finding that fibers from the nucleus cuneatus and nucleus gracilis have distinctly different olivary targets and that those from the nucleus gracilis, but not the cuneate nucleus, overlap (in part, at least) with the direct spinal fibers. Other examples of overlapping fields of terminal degeneration are present and are discussed. In general our results reveal that although certain relationships between the nuclear divisions are different, the opossum olive conforms well to that of placental mammals and provides a basic mammalian model for future experimental electron microscopic and physiological studies.

Animals↗