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Biomedical subjects

F L Mastaglia

Publications and source records attributed to F L Mastaglia.

35 records · Page 2Linked to original sources
PubMed1977

Vacuolar myopathy associated with chloroquine, lupus erythematosus and thymoma. Report of a case with unusual mitochondrial changes and lipid accumulation in muscle.

Pathological and biochemical observations are presented in a 55-year-old woman with lupus erythematosus and thymoma who developed a vacuolar myopathy while being treated with chloroquine. Electromyography showed prominent spontaneous muscle activity including myotonic discharges. Vacuoles were present in all fibre types but, in contrast to previous cases of chloroquine myopathy, were most prominent in intermediate fibres. Electron microscopy showed cytoplasmic sequestration by membranes in proximity to the t-system, many autophagic vacuoles, tubular networks, and a variety of membranous bodies, some identical to those found in certain forms of cerebral lipidosis. Other features not previously described in chloroquine myopathy included prominent mitochondrial vacuolation and sequestration of glycogen within mitochondria. Thin-layer chromatography of muscle homogenates showed an increase in all major neutral and phospholipid fractions.

Animals↗
PubMed1977

Saccadic velocities in multiple sclerosis and myasthenia gravis.

Horizontal saccadic eye movement velocities have been measured in patients with multiple sclerosis and myasthenia gravis using a computerised electrooculographic technique. The ability to detect subclinical abnormalities of eye movement with this technique indicates that it is of diagnostic value in patients with suspected multiple sclerosis who present with only a single symptomatic lesion in the central nervous system. The technique is also of value in the diagnosis of myasthenia gravis as it provides a means of quantifying the response of the extra-ocular muscles to anticholinesterase preparations.

Adult↗
PubMed1977

Electrophysiological and computerised tomography findings in multiple sclerosis: a comparative study.

102 patients with multiple sclerosis (MS) have been investigated by one or more of the following techniques: visual evoked potentials, cervical and cortical somatosensory evoked potentials, horizontal saccadic eye movement velocities, and computerised axial tomography of the cranium and orbits. It has been found that with each technique it is possible to detect abnormalities, which may be subclinical, in a significant proportion of patients. The proportion with abnormal results was greater in patients studied by more than 1 technique and was greatest in those patients who were studied by each of the 5 techniques. It is concluded that the techniques used have a complementary role in the investigation of patients suspected of having MS.

Adult↗
PubMed1977

Computerised tomography findings in multiple sclerosis and Schilder's disease.

Computerised tomography of the brain was performed in 95 patients with multiple sclerosis and in a single patient with Schilder's disease. CT of the orbits was also carried out in 53 of these cases to examine the optic nerves. In 51% of the multiple sclerosis cases low density areas compatible with plaques of demyelination were found in the white matter of the cerebral hemispheres, and less frequently in the brain-stem. A much larger area of reduced intensity was found in the occipital white matter in the patient with Schilder's disease. Varying degrees of central and/or cortical cerebral atrophy were found in 45% of the multiple sclerosis patients, the more severe degrees being found in patients with long-standing disease. Serial observations in 15 patients who were examined on more than one occasion are also presented. Small low density areas were identified in one or both optic nerves in 52% of the multiple sclerosis cases. While these may represent demyelinating lesions their significance is uncertain.

Brain↗
PubMed1977

Computerised tomography of the cranium in patients with epilepsy: a preliminary report.

The findings are presented in 366 patients with seizures who were studied by computerised tomography of the cranium. The relative frequency of normal scans, cerebral tumours, atrophy and other pathological lesions is indicated. A more detailed analysis of the group of patients with atrophy was carried out. The frequency and severity of atrophy correlated significantly with age but not with length of seizure history. However, the more marked degrees of atrophy were found among patients with long-standing or poorly controlled epilepsy, an observation which has important implications with regard to the adequacy of seizure control in epileptic patients.

Adolescent↗
PubMed1977

Ocular motor involvement in post-infective polyneuropathy.

Ocular motor involvement is uncommon in post-infective polyneuropathy. Details of the evolution and recovery of the ophthalmoplegia are presented in 11 patients in whom ocular motor involvement was a major or notable feature of the illness. Follow-up examinations after intervals of up to 6 years in 10 of the 11 patients showed no residual ocular motor impairment. Saccadic eye movement studies carried out in 6 cases at the time of follow-up showed no abnormality in 2 cases while in the other 4 cases there was mild reduction of saccadic velocities and/or increased saccade reaction times with saccade inaccuracy. In spite of the variability of the ophthalmoplegia and of the remainder of the clinical syndrome in these cases, we conclude that they all represent variants of the classical form of the Guillain-Barre syndrome.

Adolescent↗
PubMed1976

Effects of x-radiation on the spinal cord: an experimental study of the morphological changes in central nerve fibres.

The morphological changes in central nerve fibres after irradiation have been studied in the spinal cord of young adult rats exposed to 100-6 000 rad doses of 250 kV x-rays using the technique of single fibre teasing as well as conventional light and electronmicroscopic examination of cord sections. Two groups of degenerative changes were found in myelinated fibres. The first consisted of breakdown of paranodal myelin and nodal widening. These changes were found as early as two weeks after exposure to 500-6 000 rad doses and increased in frequency with dose and time in the first two months after irradiation. Paranodal myelin breakdown was less frequent after two months but nodal widening was more prominent. Increasing numbers of thinly myelinated fibres were found after three months suggesting that paranodal demyelination was followed by remyelination. This early group of changes confined to the myelin sheath provides a possible pathological basis for the self-limited sensory syndrome which sometimes occurs within a few weeks or months of irradiation of the spinal cord in man. The second type of change which was also first detected as early as two weeks after irradiation and which appears to be unrelated to the first, consisted of random Wallerian-type degeneration of fibres of all calibres in the spinal white matter. The number of affected fibres was initially small but increased with time and there was no clear dose relationship. This random fibre degeneration is probably the forerunner of the later-occurring delayed radionecrosis of the spinal cord which other workers have found to occur with a latent period of up to twelve months after exposure to doses exceeding 1900 rads to the rat.

Animals↗
PubMed1976

Evoked potential studies in neurological disorders.

Techniques for recording and analysing visual and somatosensory evoked reponses using an on-line PDP 11/40 computer have been developed and applied to a group of subjects with established or suspected multiple sclerosis as well as to patients with a variety of other lesions of the visual pathways, myoclonic epilepsy and functional neurological deficits. The most consistent responses were obtained using the pattern reversal visual evoked response and the spinal somatosensory evoked response, abnormal responses being found with both techniques in a significant number of patients with suspected demyelinating disease even in the absence of symptoms or signs referable to the visual or somatosensory pathways. The complementary role of these techniques in the detection of sub-clinical abnormalities of conduction in sensory pathways in patients with suspected MS is emphasized.

Adolescent↗
PubMed1976

Computerized axial tomography findings in a group of patients with migrainous headaches.

Computerized axial tomography of the cranium has been carried out in 46 patients referred because recurring migrainous headaches. Increasing frequency or severity of headaches or a change in headache pattern were the usual reasons for referral. Abnormalities were found in 37 cases and fell into 4 categories. The most frequent (21 cases) consisted of a mild degree of oedema in the white matter of one or both cerebral hemispheres. This was usually bi-frontal (15 patients) but was more extensive in 2 patients. Varying degrees of cerebral atrophy, as determined by widening of the Sylvian, brain-stem and interhemispheric cisterns, and/or widening of the third and lateral ventricles as compared to a group of normal scans, was found in 8 cases. Areas of occipital infarction were found in 4 patients with permanent visual field defects. Unexpected small areas of infarction were found in the temporal lobe in 2 other cases. Cerebral tumours were found in 2 cases. The significance of these findings is discussed, as well as the possible role of migrainous vaso-spasm with consequent changes in cerebral blood flow in the pathogenesis of oedema and atrophy in migraine subjects.

Adolescent↗
PubMed1975

Morphological changes in skeletal muscle after transplantation. A light and electron-microscopic study of the initial phases of degeneration and regeneration.

The morphological changes in subcutaneously implanted muscle homografts in mice were studied by light and electron microscopy 1-23 days after transplantation. The initial degenerative changes were identical in isografts and allografts and were essentially thesame as those found in muscle explants in tissue culture and aftervarious forms of muscle injury. Regnerative changes were prominent at the periphery of graftsby 48-72 hr beforeevidence of graft revascularization could be demonstated by India ink perfusion. Active regeneration occurred in isografts and in allografts during the first week resulting in the formation of a new population of muscle fibres. Rejection subsequently occurredin allografts between days 7-14. Regeneration was retarded or completely inhibitied by exposure of the donor animal to 1,500 rad of X-irradiation 1-2 hours prior to transplantationsuggesting that regeneration is brough about by cells dervied from the graft. Eectron-microscopic observations in 48-72 hr grafts suggested that primitive mononucleated myogenic cellsmay form within degeneratiang muscle fibres by a process of myonuclear sequestration. Inactive satellite cells were present in the muscle from which grafts were taken in some donoranimals but no definite conclusions could be reached as to the role of these cells in graftsregeneration.

Animals↗
PubMed1975

Mechanisms of cell-mediated myotoxicity. Morphological observations in muscle grafts and in muscle exposed to sensitized spleen cells in vivo.

Fragments of rectus abdominis muscle or diaphragm from AKR or Prince Henry mice were transplanted beneath the skin of the anterior abdominal wall of BALB/C mice and were examined by light and electron microscopy 1-23 days after transplantation. Observations were made on the mode of entry of lymphoid cells into grafts, the nature of the inflammatory cells present in grafts and the interactions between mononuclear cells and regenerating muscle fibres during the rejection phase 7-14 days after transplantation. Lymphoid cells were found to migrate through the endothelium of venules and to enter the space between the basement lamina and the plasma membrane of muscle fibres to make contacts of varying degrees of complexity with the muscle cell membrane. Evidence of penetration of lymphoid cells into muscle fibres (emperipolesis) was found and was usually associated with signs of damage to the muscle fibre. Interactions between mononuclear cells and mature muscle fibres were studied after inoculation of sensitized spleen cells into the musculature of the tongue in mice. Both lymphoid cells and mononuclear phagocytes were found to invade muscle fibres by penetrating the basement lamina. The muscle cell membrane was usually broken down in zones of invasion by mononuclear cells but generally remained intact at sites of invasion by lymphoid cells.

Animals↗
PubMed1975

Internuclear ophthalmoplegia in progressive supranuclear palsy.

Internuclear ophthalmoplegia has not previously been described in progressive supranuclear palsy. The present report draws attention to the occurrence of varying degrees of anterior internuclear ophthalmoplegia in 4 out of 13 cases of this condition studied over a 4-yr period. This finding suggests that the medial longitudinal fasciculus may be involved in the degenerative process in some cases of progressive supranuclear palsy.

Aged↗