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Biomedical subjects

J M Mussini

Publications and source records attributed to J M Mussini.

14 recordsLinked to original sources

Degeneration and subsequent regeneration of mouse skeletal muscle after a single injection of chlorpromazine: changes in mitochondrial calmitine and calcium content.

We studied experimental models capable of showing muscle degeneration and subsequent regeneration and observed the changes in calmitine, calcium uptake and calcium concentration in mitochondria during these processes. The results presented here are based on the study of mitochondria of mouse skeletal muscle after a single intramuscular injection of chlorpromazine. This drug induces myotoxic effects followed by muscle regeneration. Our results show that the muscle degeneration process, as shown by histological studies, was associated with some changes in mitochondria: a decrease in calmitine, a calcium overload and a decrease in calcium uptake; the subsequent regeneration process was associated with an increase in calmitine, a decrease in calcium concentration and an increase in calcium uptake, these 3 parameters returning to normal values. It seems that there is a correlation between a decrease in calmitine and muscle degeneration, and an increase in calmitine and muscle regeneration, as shown by our biochemical and histological observations.

Animals

Ketamine and midazolam neurotoxicity in the rabbit.

Ketamine and midazolam can produce analgesia following intrathecal administration in rabbits. However, neurotoxicity studies are required before these agents can be considered safe for clinical use. The aim of this study was to evaluate by histologic and blood-brain barrier (BBB) studies whether ketamine or midazolam could be used as an alternative to local anesthetics or opioids to produce spinal analgesia. Forty white New Zealand rabbits were randomly assigned to four groups of 10. In the conscious animal, 0.3 ml 0.9% saline solution, 1% lidocaine, 1% ketamine, or 0.1% midazolam was intrathecally injected intracisternally using a modification of the technique of Yaksh et al. Light and fluorescence microscopy were performed on transverse spinal cord sections by a neuropathologist unaware of the administered agents. All spinal cord section slides were scored within four zones: upper cervical, lower cervical, median thoracic, and lumbar segments. Spinal cord homogeneous lesions with higher scores than those of lidocaine-treated animals were considered abnormal. The BBB study showed evidence of neurotoxicity for ketamine, whereas light microscopy indicated no significant differences in comparison with saline and lidocaine. Midazolam-treated rabbits showed significant changes in both BBB and light microscopy studies. In view of these results, the intrathecal use of midazolam should be avoided in humans. Lesions observed following ketamine suggest the need for further experimental studies of the solvent and different ketamine enantiomers to establish definitively the safety of intrathecal free ketamine in humans.

Animals

[Vascular manifestation of thoracic outlet syndrome. Prospective study of 104 patients].

On the basis of a prospective study of 104 patients, the authors discuss the diagnostic value of the clinical symptoms revealing the thoracic outlet syndrome (TOS), as well as the specificity of the vascular functional exploration carried out to establish the diagnosis. Non-systematized pain and dysesthesia in the upper limb, with a postural or nocturnal onset, and Raynaud's sign are the most frequently observed signs. The "candlestick" maneuver still is the most reliable clinical triggering maneuver. The clinical features and the vascular functional explorations (capillaroscopy and digital plethysmography) allow demonstrating the existence of a true Raynaud's syndrome secondary to the TOS. The results of the arterial Doppler study distinguish the symptomatic and asymptomatic sides in the same patient, though without any correlation with the symptoms observed. The Doppler examination therefore seems to be reliable to demonstrate an anatomical duct, but remains insufficient to establish a causal relationship with the signal symptoms in most cases.

Adolescent

Adult sphingomyelinase deficiency: report of 2 patients who initially presented with psychiatric disorders.

We studied 2 unrelated adult patients under neuroleptic treatment who met all phenotypic and biochemical criteria for Niemann-Pick disease type B. In addition, they had chronic psychiatric disorders and low blood levels of HDL cholesterol. The marked and persistent deficiency of acid sphingomyelinase and the disturbance of sphingomyelin metabolism in skin fibroblast subcultures ruled out a pure drug-induced lipidosis. The association of Niemann-Pick disease type B with psychiatric disorders and with low levels of HDL cholesterol could be a chance association of 2 diseases, a new phenotype of Niemann-Pick type B, or the revelation by the neuroleptic treatment of a subclinical inborn sphingomyelinase deficiency.

Adult

Immunofluorescence studies of intra cytoplasmic immunoglobulin binding lymphoid cells (CILC) in the central nervous system. Report of 32 cases including 19 multiple sclerosis.

In 32 cases of human neurological disorders, including 19 cases of Multiple Sclerosis, an analysis was performed on formalin fixed paraffin embedded brain tissue by using fluorescein labelled antibodies and Nomarski optics. This method, which allows the reduction of technical artifarcts, permitted to establish the presence of intracytoplasmic immunoglobulin binding lymphoid cells with IgG and IgA, more rarely IgM, in the majority of the multiple sclerosis cases. This supports the theory of the intrathecal secretion of antibodies within the central nervous system but does not allow to explain the function of those B. Lymphocytes. The same immunological picture was found in Subacute Sclerosing Panencephalitis. On the contrary, it was different in Progressive Multifocal Leucoencephalopathy.

Adult

[Electron microscopic study of nerve, muscle and skin lesions induced by perhexiline maleate (author's transl)].

The pathological findings in four nerves and muscles and in one skin biopsies from four patients treated with perhexiline maleate for angina pectoris are reported. In every case, a muscular denervation atrophy and a decrease in the large diameter myelinated fibers were observed. Only one case showed a decrease of the total number of myelinated fibers, on quantitative studies. The electron microscopic study of each nerve displayed findings consistent with a predominant schwannian degeneration, associated with a few onion bulbs formations and, in two cases, with a mild wallerian degeneration. The most striking finding consisted in the presence of polymorphous membrane-bound inclusions reminding the morphology of lysosomal complex lipids. These structures were very abundant in Schwann cells, but they were seen also in fibrocytes, endothelial and pericytic cells. Similar inclusions were present in the single muscle and skin biopsies studied by electron microscopy. In the muscle, they were seen in muscular cells as well as in endothelial and pericytic cells. In the skin, similar inclusions were observed in endothelial, smooth muscle and sweat gland cells. These inclusions were difficult to identify in one micron thick sections, emphazing the need of ultrastructural study for diagnostic purposes.

Aged

[The myofibroblast. Recent advances (author's transl)].

The myofibroblast morphology is analyzed from the literature. The absence of the specific structural characters previously used for identification is emphasized and the physiological properties are analyzed. The different conditions which influence the appearance of this cell under normal and pathological conditions are reviewed and shown to be associated specially with inflammatory processes. As a conclusion, the evolution of the cell is described to the perspective of mesenchymal dynamics.

Fibroblasts

Combined Nomarski interference contrast and immunofluorescent study of neuropathological specimens: CSF sediments and paraffin embedded brain tissues.

The direct immunofluorescent technique may be easily improved by the use of the Nomarski optics. This contrast allows accurate identification of fluorescent CSF cells and structures in formalin fixed paraffin embedded brain tissues; in the latter, the combined optical procedure is fruitfull in order to avoid fluorescent artifacts misinterpretation. Furthermore, it is emphazised that the conditions in which routine neuropathological specimens are removed and stored usualy does permit the application of the immunofluorescent technique.

Brain

[Quantitative study of the peripheral nerve in semi-thin section by the Nomarski interference contrast technic].

The Normarski interference-contrast microscope is well suited to observations of transverse thick sections of epon-embedded peripheral nerves. The quantitative study of myelinated fibers is possible on photomicrographs with a final magnification of X 500. The induced distorsion is very small as compared with the results obtained by conventional bright field and positive phase contrast optics. The comparative study of the same field with the three devices allows a better interpretation of marginal pictures.

Humans

[Striato-nigral degeneration. Clinical and anatomic study of a case which responded favorably to L-Dopa].

The authors have reported the case of a 65 year old woman followed for almost 4 years with an akineticrigid Parkinsonian syndrome which responded well to levodopa. Waning of response finally occurred and despite the addition of a peripheral dopa decarboxylase inhibitor the patient died suddenly. Autopsy examination revealed pure nigro-striatal degeneration without any additional pathology. The report deals primarily with purity of the Parkinsonian picture and especially with the conspicuous and prolonged effect of levodopa.

Aged

Becker-type muscular dystrophy.

This is a review of clinical, cardiologic, electrophysiologic, pathologic, and serum creatine kinase changes in eight families with slowly progressive X-linked Becker-type muscular dystrophy. All but one of the patients were able to walk until the age of 16 years, and most lived beyond 20. In every family, electromyography and muscle biopsy showed features which, on the basis of classical criteria, were interpreted as those of both myopathy and denervation, although among patients and among families, one or the other of these processes predominated. The most frequent biopsy picture was of fiber atrophy and hypertrophy, with many split and angulated fibers, and clumps of pyknotic nuclei. Necrosis, phagocytosis, regeneration, endomysial fibrosis, and some fatty infiltration were commonly seen. Review of a family originally described by Becker showed a similar biopsy picture; These pathologic changes are separable from those of Duchenne muscular dystrophy, but they often overlap with those seen in other chronic neuromuscular diseases.

Adolescent