Biomedical subjects
Publications and source records attributed to P E Marchiori.
Chorea may occur as a neurological manifestation of systemic lupus erythematosus and is often associated with detection of antiphospholipid antibodies. No evidence of chorea as a sign of lupus activity has been established. We describe a patient with systemic lupus erythematosus associated with antiphospholipid antibodies who developed chorea, which has been considered a sign of lupus activity.
The authors report a case of Shy-Drager syndrome in a 53 year-old male patient. Autonomic failure was made evident by physical examination as well as laboratory tests. A sleep recording showed decreased percentage of REM sleep and apneas of the central type. The possible mechanisms for this sleep disorder are discussed.
Immunoglobulins isotypes (IgG and IgM) for myelin basic protein (MBP), cerebrosides (CER), gangliosides (GANG) and cardiolipin (CARD) were detected in the cerebrospinal fluid (CSF) from 33 patients with multiple sclerosis (MS), 18 with Guillain-Barré syndrome (GBS) and 30 with systemic lupus erythematosus (SLE). In MS patients occurred positive and significant levels of IgG-MBP in 51.5% (p less than 0.05) and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels for IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p less than 0.05), 53% for IgM-MBP, 38.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p less than 0.05). Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD, only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphospholipid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.
Radioimmunoassay techniques were used to detect antibodies to the acetylcholine receptor (AAChR) in 164 patients with adult-onset myasthenia gravis. AAChR levels above 0.6 nM/l were considered pathological and were found in 67% of the patients with an average value of 58.99 +/- 125.02 nM/l (0.6-900.0). Correlation, with clinical functional status, the histopathological thymus alterations and the different therapeutics used did not disclose any statistically significant differences.
Recently, cyclosporin-A (Cy-A) has been used in the treatment of myasthenia gravis (MG). This drug could be employed in some patients refractory to classic treatments or that develop undesirable side effects. It is reported the case of a 22 year-old woman with generalized and severe MG, and diabetes mellitus. She had been submitted to thymectomy and reoperated, to the classic ethiopathogenic methods of therapy, and to total body irradiation. No therapeutical results were observed. Also, she developed transient and slow bone marrow depression, and liver dysfunction. Owing to these limitations and to the absence of response to treatments mentioned. Cy-A use was attempted in this case. Unfortunately, Cy-A did not influence the myasthenic symptomatology. Cy-A also failed in suppressing anti-AChR production, which increased during Cy-A therapy. Results observed in this case are in disagreement with literature data on the subject.
A 50-year-old man with positive test for human immunodeficiency virus (HIV) by enzyme-linked-immunoassy and Western-blot, without clinical manifestations of acquired immunodeficiency syndrome (AIDS), developed acute polyradiculoneuritis and was treated by plasmapheresis with improvement. We believe that chemical homologies of antigenic determinants between HIV and P2 protein of peripheral nervous system and myelin basic protein may induce crossed-reaction, thus developing acute polyradiculoneuritis and central nervous system involvement, respectively. The nervous system involvement hy HIV also occur in the HI-viremy, seric conversion alone, and AIDS with or without oportunistic infections.
The case of an 11-year-old boy with external ophthalmoparesia, tetraparesia and bilateral eyelid ptosis is reported. He was 7-years-old when first symptoms appeared. Anticholinesterasic drugs were used. He was submitted to muscle biopsy. The results of histochemistry analysis showed storage of granulous material at the subsarcolemmal region of muscle fibers by SDH. Increase in the number of mitochondria with electron dense bodies was found at electron microscopy. Anticholinesterasic drugs administration was interrupted and consequently he got worse, and bouts of dyspnea occurred. Due to this worsening anticholinesterasic agents were reintroduced together with prednisone, and he improved. Due to clinical and histological expressions we think it is possible that morphological mitochondrial alterations may occur also in myasthenia gravis.
Explore the source record for details and available documents.
A significant decline of CD3 cell detected by rosettes and a significant increased of B cell populations were observed. The total CD3+, helper CD4+ and suppressor CD8+ T-cell subsets showed no significant variation em relation to sex, age thymectomy and corticotherapy by monoclonal antibodies.
Explore the source record for details and available documents.
A retrospective survey of the records of 33 patients with generalized acquired myasthenia gravis treated at Hospital das Clínicas, Medical School of The University of São Paulo, Brazil and in private practice, with and without thymectomy, is reported. Nineteen were thymectomized and 14 non operated. Both groups were demographically homogeneous and were followed from 8 to 24 years. Parameters for evaluating the natural history of disease and other ones for a comparative analysis of the improvement or remissions were established. The influence of the individualized therapy was evaluated by McNemar test (chi 2); the U-Mann-Whitney was used for evaluation of the populational behavior groups; variable parameter with possible influence in the useful life were studied by multivariate analysis. The difference of distance between both groups was not significant: (D2 = 0.08894); T2 = 7.17 (Hottelling test). There were differences of the isolate clinic parameter response in both groups, but the global analysis of the parameters did not permit to discriminate them, possibly because of interaction of the parameters influencing the analytic result. Inspite of the fact that superposition of the parameter occurred when analysed for a long period, we believe the early thymectomy to be beneficial. Our patients who had their operation at a younger age and specially those with recent disease had a maximum improvement.
Sixty-three patients with dermatopolymyositis were evaluated from the clinical, laboratory and therapeutical aspects during a period of 15 years: 39 are women and 24 men. The mean age was 36.8 +/- 15.6 years. No correlation was observed between clinical and isolated therapeutics employed; when corticosteroids and cytolytic drugs were used simultaneously, the clinical response was satisfactory. No special fact was seen that can predict the therapeutical response.
The authors describe one patient with Behçet's disease who had pseudotumor cerebri as a neurological manifestation. The possibility of pseudotumor cerebri due cerebral venous thrombosis in Behçet's disease is emphasized.
Estrogen (ER), progesterone (PR), androgen (AR) and glucocorticoid receptors (GR) were evaluated in thymus cytosols from 15 myasthenia gravis (MG) patients, using a dextran charcoal assay. ER was found in 40%, PR and AR in 53% and GR in 100% of MG thymi. Competition studies demonstrated steroid specificity for all receptors. Mean ER and AR levels in MG thymi were higher than those in the thymi of 4 control children. Female patients have higher AR titers. Myasthenic thymi presented lower GR values than in normal controls: Differences in steroid receptor content observed in the MG thymus as compared to the child thymus may reflect variations in cell populations.
A purified citric acid soluble extract from human skeletal muscle (AEMA) and a phosphate-buffered saline extract from rabbit muscle acetone powder (EMA) were used to coat polystyrene beads in an enzyme-linked immunosorbent assay (ELISA). From 54 patients with myasthenia gravis, positive results were observed in 14. Five of 6 patients with thymoma had high levels of antibodies. With the diagnostic difficulties in detecting small and medium-sized thymoma, a sensitive assay for detection of antibodies to muscle antigen may be an important supplementary tool to detect tumors at early stages.
Fourteen patients (twelve of them were women) with severe myasthenia gravis who had not responded to any treatment at all, were treated by cytostatic drugs. Both azathioprine and cyclophosphamide single or in combination are used. All patients except two were thymectomized and almost all were treated by plasmapheresis and corticosteroid before and during the cytostatic treatment. All patients take cholinesterase inhibitors. No serious complications were observed. The patients received 100-200mg of azathioprine and/or 100-200mg of cyclophosphamide daily by oral route, the first during 20 months and the second one during 6 months. Three patients received cyclophosphamide 1g daily by venous route during 6 months at 15-20 days intervals. The preliminary results were favourable occurring important improvement in 71.4% of the cases. Their performance increased considerably. The progress of the disease was generally stabilized. Exacerbations and crises did not recur, except in one case. Only three patients had not responded to the treatment, one responded poorly but better later and one had an exacerbation after the 24th month. This patient returned to azathioprine.
The present study was performed to analyse glucocorticoid receptor (GR) binding in peripheral blood mononuclear leukocytes (MNL) from 39 myasthenia gravis (MG) patients (unoperated patients (n = 13), thymectomized patients (n = 14) and patients receiving glucocorticoids: thymectomized (n = 11) and unoperated (n = 6]. A whole cell binding assay with 3(H) dexamethasone was used. GR mean values were significantly higher in the MNL of MG patients (thymectomized or not) not receiving glucocorticoid than in the MNL of healthy donors. Affinity was within the normal range. Sex, age or clinical forms of illness did not influence the results. In patients receiving prednisone (Pd) the GR values were significantly lower than in MG patients without Pd therapy, independent of Pd dose or time of administration. No differences in receptor binding between normal subjects and MG patients receiving Pd have been found.
A comparative study was conducted on two groups of patients with the generalized severe form of myasthenia gravis. The first group of 20 patients received oral daily doses of 60-100 mg of prednisone prior to thymectomy. The control group of 20 were submitted to surgery without prior corticosteroid treatment. The study included statistical analysis of the clinical results and surgical complications for both groups. The authors concluded that the use of steroids preoperatively is beneficial.