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PubMed1985

An evaluation of signs in ocular myasthenia gravis and correlation with acetylcholine receptor antibodies.

Fifteen patients with ocular myasthenia gravis were examined in detail for 21 different signs, and tested for acetylcholine receptor antibodies. The major signs of ocular myasthenia gravis included ptosis, disorders of ocular rotations, weakness of eyelid closure, "pseudosupranuclear" signs and the lid twitch sign. Acetylcholine receptor antibodies were found in eight of the 15 patients. One hundred and four normal, non-myasthenic patients were also examined for the lid twitch response, and the relationship between the lid twitch of ocular myasthenia gravis and that found in normal subjects is discussed.

Adult↗

PubMed1982

Urinary incontinence following prostatectomy in patients with myasthenia gravis.

An unusual incidence of urinary incontinence was observed in patients with myasthenia gravis who required transurethral resection of the prostate for relief of obstruction secondary to benign prostatic hypertrophy. This urinary incontinence was not observed in patients who had open prostatectomies (suprapubic and retropubic). The urinary incontinence did not appear to be related to the control of the underlying muscular disorder. It is suggested that the external sphincter in patients with myasthenia gravis may be more vulnerable to thermal changes that occur when a blended current is used during transurethral resection. If prostatectomy is required in a patient with myasthenia gravis, open surgery should be the procedure of choice. If a TURP is used, then a high frequency current is advised with prudent resection in the area of the prostatic apex.

Aged↗

PubMed1983

Myasthenia gravis in the elderly: report of 37 cases.

The treatment of myasthenia gravis in the elderly is controversial. Thirty-seven myasthenic patients with onset of the disease after the age of 60 were followed for a period of 14 years. All of the 37 patients received anticholinesterase drugs during this period, ten underwent thymectomy, and 24 were treated with corticosteroids. At present, one patient is in remission, 28 are improved, one is unchanged, and seven have died. Only one death was directly related to myasthenia. In the authors' experience thymectomy can be an effective treatment of myasthenia gravis in elderly patients; corticosteroid therapy can also be useful in addition to or as an alternative to surgery. Using a "personalized" schedule the authors obtained good results in 78 per cent of their patients.

Aged↗

PubMed1987

CA-antibody: an immunological marker of thymic neoplasia in myasthenia gravis?

We have examined sera from 141 patients with myasthenia gravis and 11 non-myasthenia gravis patients with thymoma for antibodies to a citric acid extract of skeletal muscle (CA-antibodies). Sera were obtained from 5 different centers. The thymus histology was defined in each case. Sera from 56/66 patients (85%) with thymoma contained CA-antibodies, while such antibodies were only detected in 6/75 (8%) of the non-thymoma patients. None of the patients with thymus hyperplasia had CA-antibodies. One MG patient who developed MG after a bone-marrow transplantation, also developed CA-antibodies. The remaining 5 CA positive non-thymoma MG patients were all greater than 65 years and had thymic atrophy. Two of them had myasthenia gravis and polymyalgia rheumatica. In 2 thymoma patients with non-detectable levels of CA-antibodies before thymectomy, such antibodies were demonstrated in high titres after the operation. In 2 other sera from thymoma patients, the titres of CA-antibodies fell to less than 32 after thymectomy. There were 3 sera with CA-antibodies among 11 sera from non-MG patients with thymic tumours.

Antibodies↗

PubMed1996

Myasthenia gravis in the elderly: a hospital based study.

To evaluate clinical characteristics and outcome of myasthenia gravis (MG) in aged patients (> 60yrs), we retrospectively reviewed a continuous series of 122 myasthenic patients observed from January 1968 through December 1994. Patients with congenital, neonatal, or penicillamine-induced myasthenia were excluded. Twenty-five subjects (20%) were > 60yrs. The male/female ratio was 3:2; 20% of patients had an ocular form and 86% were seropositive. Mediastinum CT scan revealed thymic changes in 14%. During the first five years of disease, 60% of patients with ocular form progressed towards a generalized form and 15% had clinical relapses. At the time of their last visit, 40% of patients were asymptomatic and 60% had improved on medication. No patient died because of myasthenia-related causes. This study shows that MG in aged patients is characterized by prevalence in males, low frequency of ocular forms, low frequency of positive mediastinum CT which suggests low frequency of thymomas, high frequency of progression of ocular forms, and good response to corticosteroid therapy.

Adrenal Cortex Hormones↗

PubMed1996

Myasthenia gravis in childhood.

Clinical features, serum acetylcholine receptor antibody (AChRAb) titres and course were reviewed in a series of 25 congenital (CMG) and 30 juvenile (JMG) myasthenia gravis cases to recognize characteristics of childhood-onset myasthenia and its subgroups. The initial symptom for CMG is ptosis accompanied or followed by generalized weakness; myasthenic crises do not occur and spontaneous remissions are rare. In JMG, the distribution of weakness remains the same, but the severity fluctuates: spontaneous remissions (6 patients) and myasthenic crises (10 patients) are observed. Good response to anticholinesterase drugs is slightly more frequent in JMG (62 versus 41%). AChRAbs were present in 9/26 JMG tested, girls with onset after 11 years being more likely to be Ab-positive. Since patients with autoimmune myasthenia and a young age of onset are often seronegative, clinical features such as changing distribution of weakness, fluctuating severity, or response to treatment might be considered as supportive criteria for differentiating JMG from CMG.

Adolescent↗

PubMed1977

Segregation of HLA haplotypes in 100 families with a myasthenia gravis patient.

An analysis of the HLA data of 100 myasthenia gravis (MG) patients and their first degree relatives and spouses is presented. The family studies confirmed the excess of B8 in the myasthenia gravis patients and showed that the excess was not limited to the A1, B8 haplotype. An excess of B8 homozygous patients was found from the matings of two B8 heterozygous parents, suggesting the existence of a dominant B8 linked MG susceptibility. B8 homozygous individuals run a 4-7 times higher risk of acquiring myasthenia gravis than B8 heterozygous individuals.

Female↗

PubMed1976

The role of humoral and cellular immune factors in neuromuscular block in myasthenia gravis.

The neuromuscular block of myasthenia gravis appears to be due to decreased and abnormal responsiveness of the motor end plates to transmitter, and perhaps a decreased number of functioning end plates. The presence in myasthenic patients of serum globulins that bind to a bariety of cellular and subcellular components, and of thymic abnormalities, has encourage the search for humoral and cellular immune factors that may be responsible for the anatomic and functional defects. Attempts to demonstrate neuromuscular blocking activity in the serum have been inconclusive. While 30% to 48% of myasthenic patients have globulins that react with muscle membrane, striations, ribonucleporotein, and thymic epithelial cells, these globulins have not been shown to react with the end plate or acetylcholine receptor, or to impair neuromuscular transmission. Antinuclear, rheumatoid, antimitochondrial antithyroid, anti-smooth-muscle, and anti-gastric-parietal cell factors are found in the serum of 3% to 16% of myasthenic patients, but these are much more commonly present in other diseases. However, antibodies to acetylcholine receptor from electric tissue, which were recently reported in the serum of three fourths of myasthenic patients, may prove to be more directly related to the neuromuscular block. While the majority of myasthenic patients have thymic abnormalities, including thymoma in 9% of patients and hyperplastic thymus in 66% of patients, the remaining 25% of patients have normal, involuted, or undetectable thymus. Thymectomy has a favorable effect in about two thirds of myasthenic patients, but about one third of patients have no benefit. Thirty-two patients have been described who developed myasthenia gravis after total thymectomy and presumably in the absence of the thymus. Thymus lymphocytes of myasthenic patients have some differences from those of normal subjects, including a greater proportion of B cells, but their significance is not known. Attempts to demonstrate neuromuscular blocking activity in the thymus of myasthenic patients have been inconclusive. Blood lymphocytes of myasthenic patients also have some differences from those of normal subjects, including a lower proportion of T cells. The proportion of both T and B cells increased following thymectomy. While studies on the immunological reactivity of lymphocytes from myasthenic patients have shown some differences from those of normal subjects, neuromuscular blocking activity has not been demonstrated in these cells or in their extracts. There is increasing evidence that the neuromuscular block of myasthenia gravis is due to alteration of the acetylcholine receptor. The recent reports of antibodies to acetylcholine receptor in the serum of myasthenic patients suggests that these may be responsible for the neuromuscular block, but such action, and the cause of antibody release, remain to be determined.

Animals↗

PubMed1981

Aspects of the natural history of myasthenia gravis: crisis and death.

In an analysis of myasthenia crises during the past 20 years, the incidence of crisis remained fairly constant at 12-16%, but the fatality rate of crises declined from 42% to 6%, and the overall mortality of myasthenia declined from 12% to 3.3%. Most crisis episodes were caused by aspiration pneumonia or occurred in patients with severe dysphagia, who were at risk of aspiration. Cholinergic drug therapy clearly was not useful during crisis, and should probably be discontinued while the patient is on a respirator. Fifteen of 28 patients with thymectomy had 21 episodes of crisis months or years after surgery. Ultimately, 42% of crisis survivors achieved a state of improved myasthenia or remission after one or more crises.

Adolescent↗

PubMed1987

Clinical experience in more than 2000 patients with myasthenia gravis.

The vast strides in terms of pathophysiologic understanding which have been made in the past 25 years of research in myasthenia gravis are remarkable. This period of time has also seen the evolution of many applicable technological advances to better our care of these patients. Myasthenia's place in the autoimmune family of diseases has been demonstrated. No clear-cut strategy resulting from these discoveries has, however, been more than one of temporary relief or clinical improvement. In our center over these years the performance of early thymectomy in all cases of generalized myasthenia seems to be the one demonstrably reliable technique available. The effect of this procedure on coexisting neoplasia and other autoimmune disease suggests continuing avenues of investigation.

Adrenocorticotropic Hormone↗

PubMed1993

Immunoadsorption plasmapheresis for severe generalised myasthenia gravis.

Two patients with severe generalised myasthenia gravis of childhood were successfully treated with immunoadsorption plasmapheresis. One patient was a 5 year old girl and the other was a 12 year old girl. A dramatic improvement in bulbar symptoms and generalised muscle weakness was achieved without any side effects. Plasma exchange may also yield clinical improvement for myasthenia gravis, but the treatment is accompanied by the risk of viral infections and hypersensitivity reactions. Immunoadsorption plasmapheresis is an effective treatment for severe generalised myasthenia gravis of childhood and is superior to plasma exchange with respect to safety.

Child↗

PubMed1991

Extraocular muscle surgery in myasthenia gravis.

Myasthenia gravis is typically a disease of young people in active employment who need a field of binocular single vision. Although it is systemically controllable with a good chance of spontaneous remission, persistent loss of binocularity may cause chronic disability. We report our experience of extraocular muscle surgery in five patients with stable myasthenia gravis and persistent double vision. Extraocular muscle involvement was selective, giving rise to incomitant and concomitant squints, with individual muscle overactions as well as underactions. Treatment was by conventional recession and resection procedures with the additional use of Faden and adjustable sutures where appropriate. In all five cases a larger, stable field of binocular single vision was established. It is concluded that extraocular muscle surgery may be beneficial in selected cases of myasthenia gravis.

Adolescent↗

PubMed1975

Myasthenia gravis associated with penicillamine treatment for rheumatoid arthritis.

Four patients with rheumatoid arthritis (R.A.) developed myasthenia gravis after taking penicillamine. In one patient withdrawal of the drug was followed by spontaneous remission of the myasthenia, and in two the dose of anticholinesterase was subsequently reduced. In the fourth patient continuing penicillamine treatment was associated with increasingly severe myasthenic features, but on withdrawal of the drug these resolved. As myasthenia gravis rarely complicates R.A. its onset in these patients shortly after the start of penicillamine treatment suggested that penicillamine may have precipitated this condition.

Adult↗

PubMed1975

Single fibre electromyographic studies in myasthenia gravis with repetitive nerve stimulation.

A method is described for repetitive stimulation studies in myasthenia gravis using submaximal stimulation and single muscle fibre recording techniques. It was found that there were no impulse blockings due to neuromuscular transmission factors in normal subjects with 2 Hz stimulation, although there was a decrease or increase in the number of potentials which was caused by axonal stimulation factors. In myasthenia gravis a pathological picture was obtained, consisting of impulse blockings and facilitation at this rate in all of the eight patients studied, even those with only the ocular form of myasthenia and without surface decrement in the ADM. This technique allows study of both the minimally involved motor endplates and those with pronounced neuromuscular disturbances.

Adolescent↗

PubMed1979

Lymphocyte function in myasthenia gravis.

Mitogen-induced blastoid transformation of peripheral blood lymphocytes from patients with myasthenia gravis was studied using a microplate culture technique and evaluated with 3H-thymidine incorporation. It was found that both phytohaemagglutinin and pokeweed mitogen responses decreased significantly in patients with myasthenia gravis. In myasthenic crisis, indices of stimulation by phytohaemagglutination became very low. The autologous plasma neither inhibited nor facilitated mitogenic responses of lymphocytes. The decreased mitogen responsiveness of lymphocytes suggests that part of the T lymphocyte function is subnormal in myasthenia.

Adult↗

PubMed1980

Immune regulation of experimental myasthenia.

Experimental autoimmune myasthenia gravis (EAMG) is an appropriate model for studying the molecular origin, immunological mechanism and regulation of myasthenia gravis. Several approaches are being utilised for the regulation of the immune response to AChR and for immunosuppression of EAMG: Corticosteriods and azathioprine can suppress EAMG concomitantly with suppression of immune responses to AChR. High dose cyclophosphamide treatment in mice facilitates the onset of EAMG and results in a selective suppression of the humoral response to AChR whereas the cellular response is enhanced. Specific immunosuppression of EAMG is achieved by using a nonmyasthenic, denatured AChR preparation which cross reacts with the intact receptor. Various degradations and modifications of AChR are being performed in order to identify the smallest molecular entity responsible for the myasthenic activity of AChR. Studies on specific monoclonal antibodies, anti-idiotypes, and on the effect of measles virus on EAMG are being described and their possible significance in regulating myasthenia are being discussed.

Acetylcholine↗

PubMed1981

Correlation between serum myoglobin and thyroid status in myasthenia gravis.

In 52 patients with myasthenia gravis serum myoglobin showed a significant inverse correlation to circulating thyroxine and triiodothyronine levels. The highest myoglobin concentration (240 ng/ml) was found in a myasthenia gravis patient with hypothyroidism. Slightly elevated myoglobin (54-60 ng/ml) was measured in four euthyroid myasthenic patients. The data suggest that a concomitant hypothyroid state must be excluded whenever high myoglobin levels are found in myasthenia gravis.

Adolescent↗

PubMed1982

Familial myasthenia gravis.

A family is reported in which myasthenia gravis and thyroid disease occur over three generations. The grandmother and granddaughter have ocular myasthenia and an aunt in the second generation had generalised myasthenia gravis with a thymoma. The pattern of histocompatibility antigens (HLA) haplotypes, anti-AChR antibodies, anti-striate muscle antibodies and thyroid disease is described. The haplotype HLA-A1, B8 was found in affected members of the first and third generation but the family study showed that this was not the same haplotype because the HLA-A1, B8 haplotype in the third generation was contributed by an unaffected person marrying into the family in the second generation.

Adult↗