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Biomedical subjects

R Mantegazza

Publications and source records attributed to R Mantegazza.

68 records · Page 4Linked to original sources

Increased incidence of certain TCR and HLA genes associated with myasthenia gravis in Italians.

In order to study the immunogenetics of myasthenia gravis (MG), we analysed the TCR and HLA-class II genes from Italian and Californian myasthenic patients. We investigated polymorphisms of the TCR using the full length cDNA probes pGA5 and the pT10 for the alpha and beta chains, respectively. The 6.3 kb and 2.0 kb polymorphic markers, revealed by the PssI enzyme and the alpha chain probe, were shown to be significantly associated with MG. Italian MG patients were HLA typed, and allele frequencies showed a significant association of DR3 and DQw2 with MG. The relative risk calculated for DR3 was 7.4. T-cell proliferative responses to peptides of the AchR alpha chain were also studied and no associations with TCR RFLP analysis or HLA-class II typing were observed.

Amino Acid Sequence↗

Prospects for specific immunotherapy in myasthenia gravis.

Myasthenia gravis is an autoimmune disease resulting from a breakdown in T and B cell tolerance to acetylcholine receptor (AChR). Autoantibodies to AChR mediate the disease. Recent advances in experimental immunotherapy of autoimmune disease provide several possibilities for specific intervention in this well-characterized condition.

Humans↗

Chloroquine myopathy and myasthenia-like syndrome.

A young woman on chronic corticosteroid treatment for systemic lupus erythematosus developed a myasthenia-like syndrome 7 weeks after starting chloroquine therapy. Discontinuation of chloroquine allowed symptomatic and immunological remission within 6 months. Ocular symptoms reappeared following a second short course of chloroquine. A motor-point biopsy revealed a vacuolar myopathy with membranous bodies in intramuscular nerves. We discuss the possible role of chloroquine in the pathogenesis of a myasthenia-like syndrome.

Adult↗

Azathioprine as a single drug or in combination with steroids in the treatment of myasthenia gravis.

Azathioprine (Aza) has been used alone or in combination with steroids for two groups of myasthenic patients. Positive responses were noted in 75% of patients on Aza alone and in 70% receiving the combined regimen. The clinical course of the two groups differed in terms of respiratory crisis and need for plasma exchange. With an appropriate Aza administration schedule side-effects were not a limiting factor to its use. Aza treatment induced a reduction in anti-AchR-antibody level that was correlated with clinical improvement and greatly decreased the need for steroids.

Adult↗

Anti AChR antibody: relevance to diagnosis and clinical aspects of myasthenia gravis.

227 sera from myasthenic patients were tested for the presence of anti-AChR antibodies (anti-AChR Abs) by mean of a fetal calf receptor (Fc-AChR); 73.5% of cases proved positive with this method. Significant correlations were found between presence of anti-Fc-AChr Abs and various clinical aspects such as: clinical stage and duration of disease; moreover significantly higher than the median titers were found among younger patients (i.e. age less than 40 yrs).

Adult↗

Species specificity of anti-acetylcholine receptor antibodies elicited by synthetic peptides.

Two peptides corresponding to amino acid residues 351-368 of the alpha-subunits of Torpedo and human acetylcholine receptor (AChR) were synthesized. These peptides contain a segment (residues 355-364) which displays the greatest variability in amino acid sequence between the two species. Antibodies elicited against the two peptides cross-reacted with the respective native AChRs and were shown to be species specific by radioimmunoassay, immunoblotting, and immunofluorescence microscopy. Thus, antibodies against the Torpedo peptide cross-reacted with Torpedo AChR but did not bind to mammalian or chicken AChR. Antibodies against the human peptide proved to be specific probes for mammalian muscle AChR. They cross-reacted with mammalian AChR (human, calf, mouse, and rat) but not with Torpedo or chicken AChR. These antibodies were also shown to react preferentially with the extrajunctional form of muscle AChR, as compared to their reactivity with junctional muscle AChR. In immunofluorescence experiments, the anti-human peptide antibody stained AChR aggregates in sectioned or ethanol-permeabilized rat and mouse myotubes grown in culture but did not stain living myotubes. This indicates that the sequence 351-368 of the alpha-subunit of mammalian AChR is on the cytoplasmic face of muscle cell membranes, as predicted theoretically.

Animals↗

Single donor plasma in therapeutic plasma exchange for myasthenia gravis.

Thirty-seven myasthenic patients were treated with therapeutic plasma exchange (TPE) on different performance schedules using either random or single donor plasma. The two schedules had the same effects in terms of efficacy but single donor replacement gave significantly less side effects in the short and long term. A two exchange procedure on alternate days associated with immunosuppression seems to be an optimal schedule for the treatment of myasthenic patients. A single donor two-exchange on alternate days procedure appears to be safe, useful and can be repeated in patients who need frequent TPE.

Adolescent↗

Thymectomy for myasthenia gravis: a fourteen-year experience.

Over the last 14 years we have thymectomized 106 myasthenic patients, 62 by transcervical and 44 by transsternal approach. At the end of the follow-up 49 patients (46.2%) had improved, 12 of these (11.2%) being in remission. Our data supply no guidance regarding the prognosis of individual patients at the time of thymectomy. In our hands neither operation seemed to modify the natural course of myasthenia gravis.

Adolescent↗

Myasthenia gravis: prolonged treatment with steroids.

We have evaluated chronic corticosteroid treatment in 60 myasthenic patients; 92% were followed for more than 3 years and 82% longer than 4 years. Improvement was noted in 72% of the patients. The best results were seen in those whose symptoms started after the age of 40 years. There was a correlation between the starting dose of prednisone and the rate of improvement. Complete withdrawal of steroids was possible only for 3 patients.

Adolescent↗

New antigen for antibody detection in myasthenia gravis.

We assayed anti-acetylcholine receptor (AChR) antibodies in 61 myasthenic patients using human and fetal calf muscle AChR as antigen. Anti-AChR antibodies were detected in 85% of patients with human AChR and in 80% with fetal calf AChR. In 27 patients, the titers detected with fetal calf AChR were higher than those obtained with human AChR. Titers with different fetal calf AChR preparations were reproducible. Fetal calf AChR is a suitable antigen for immunologic analysis of myasthenic patients.

Antigens↗

Multiple sclerosis and migration in Italy: a case/control study. Preliminary reports.

The present study was performed on Milan residents, in order to evaluate the risk of acquiring multiple sclerosis between patients born in Milan and patients born in Southern Italy. For this purpose, 216 consecutive subjects with definite or probable multiple sclerosis, inmates of the Neurological Institute C. Besta (Milan) from 1968-1976, were matched with 216 controls, uniform as regards age, sex, profession and residence, who were hospitalized at the same time for other neurological diseases. Comparing the case/control pairs of the migration, we observed that the North-Centre born had a relative risk of acquiring the disease significantly superior to one (RR = 1.9; P = 0.01) as compared to migrants from the Southern regions of Italy.

Adolescent↗

Detection and morphology of thymic remnants after video-assisted thoracoscopic extended thymectomy (VATET) in patients with myasthenia gravis.

Thymectomy is often an extremely useful therapeutic procedure in myasthenia gravis (MG) and is usually indicated for adult patients with generalized disease. Because remnants of thymus may remain in extrathymic fat, an extended thymectomy is recommended. A new surgical approach without sternotomy: video-assisted thoracoscopic extended thymectomy (VATET) was performed in 30 MG patients. The weight of removed thymus ranged from 10.8 to 113 grams. The weight of fatty tissue removed from pretracheal, anterior mediastinal and costophrenic areas ranged from 6.3 to 74.8 grams. Histological examination revealed thymic remnants in 14.8% of pretracheal fat samples and in 33.3% of samples from anterior mediastinal plus costophrenic areas. These findings indicate that VATET is a radical procedure and may be the first choice surgery for young female MG patients, since aesthetic sequelae are reduced compared to procedures involving sternotomy.

Adipose Tissue↗