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PubMed1986

[Acetylcholine receptor antibodies in the diagnosis of myasthenia gravis. Study of 406 confirmed cases].

UNLABELLED: Antibodies against acetylcholine antibodies in the IgG fraction were determined in 342 patients with clinically confirmed generalized myasthenia gravis and in 64 patients with purely ocular myasthenia. The standard immunoprecipitation assay was used with detergent-extracted and 125I-alpha-bungarotoxin-marked human acetylcholine receptors as antibodies. 340 patients with generalized myasthenia and 29 with purely ocular myasthenia (99% and 45%, respectively) had titres above 0.5 nmol/l; two with generalized and five with ocular myasthenia had borderline levels (0.4 nmol/l). Titres below 0.4 nmol/l were obtained in patients with non-myasthenic oculomotor diseases (82) and other neuromuscular diseases (111). Abnormal titres were also obtained in five patients with malignant thymoma but no clinical myasthenia and one child with an immunoneuropathy. Using acetylcholine receptors from human eye muscles as antigen there was no difference between ocular myasthenia and myasthenia initially with ocular signs but later becoming generalized. Bungarotoxin-blocking antibodies were found in lower concentrations in 32 of 65 patients examined. Serial examinations demonstrated intra-individual but not linear correlations with myasthenic signs. CONCLUSION: Antibody determination is a useful and highly specific addition to the diagnostic armamentarium and is suitable for controlling treatment in patients severely affected by the disease.

Adult↗

PubMed1970

Ocular myasthenia: evaluation of Tensilon tonography and electronystagmography as diagnostic tests.

The value of electronystagmography (ENG) and of tonography in monitoring the beneficial effect of edrophonium chloride (Tensilon) on the extraocular muscles in myasthenia gravis has been assessed. Studies were performed on 17 patients with myasthenia gravis and on 18 control subjects, of whom nine had extraocular muscle weakness due to myopathic or neurogenic lesions.Electronystagmography recorded the repetitive following movements of the eyes elicited during optokinetic nystagmus. Neuromuscular fatigue with the subsequent beneficial response to Tensilon was clearly seen in 50% of patients with myasthenia. False positive responses were not seen in control subjects but in many of these, as in the remaining myasthenic patients, the amplitude and rate of nystagmus seen in the ENG was very variable. These difficulties suggest that the ENG is of limited value as a diagnostic test in myasthenia gravis.Tonography, recording the intraocular pressure of the eye continuously over four minutes, was found to be of considerable value. We found that the intraocular pressure fell on average by 1·6 to 1·8 mm Hg over a one minute period in the control recordings but increased by a mean of 1·6 mm Hg in patients with myasthenia, with a peak effect 35 seconds after Tensilon. In only one patient was there a complete failure of response. This patient, and also another woman who showed a less striking response, had severe myasthenia with a fixed ocular weakness. It is suggested that an absence of any increase in tension with Tensilon may be seen in patients with permanent neostigmine-resistant myopathic change. A small false positive response was seen on one occasion only in a patient with a sympathetic nerve lesion.Tensilon tonography, as a simple painless procedure, would appear to be of considerable value in the diagnosis of ocular myasthenia and also as a diagnostic test in the exclusion of myasthenia as a cause of isolated extraocular neuromuscular weakness.

Adolescent↗

PubMed1994

Ryanodine receptor antibodies related to severity of thymoma associated myasthenia gravis.

Ryanodine receptor (RyR) antibodies are detected in about 50% of patients with myasthenia gravis who have a thymoma. The RyR is a calcium release channel involved in the mechanism of excitation-contraction coupling in striated muscle. In this study the severity of myasthenia gravis assessed by a five point disability score was compared between 12 patients with myasthenia gravis, a thymoma, and RyR antibodies and 10 patients with myasthenia gravis and a thymoma but without such antibodies. Symptoms of myasthenia gravis were significantly more severe in patients with RyR antibodies. The mean (SD) disability scores were 3.7(0.5) in patients with antibodies and 2.7 (0.9) in those without at peak of illness, (p = 0.01) and 3.4(1.4) v 1.6(0.7) at the end of an average observation period of five years (p = 0.002). The number of deaths due to myasthenia gravis was five of 12 RyR antibody positive patients, and none of 10 RyR antibody negative patients (p = 0.04). RyR antibody levels correlated positively with severity of myasthenia gravis. The presence of circulating RyR antibodies seems to be associated with a severe form of thymoma associated myasthenia gravis.

Adult↗

PubMed1995

Ventilatory muscle strength and endurance in myasthenia gravis.

Patients with generalized myasthenia gravis (MG) often have associated ventilatory muscle involvement. It is not known whether patients with isolated ocular muscle involvement have identifiable involvement of their ventilatory muscles. Most studies have assessed muscle involvement by measuring muscle strength; however, we hypothesized that measures of ventilatory muscle endurance may be more sensitive tests of ventilatory muscle involvement in myasthenia gravis. We studied 17 patients with myasthenia gravis (four with ocular involvement alone and 13 with varying degrees of generalized myasthenia gravis). Spirometry, ventilatory muscle strength (maximum inspiratory and expiratory pressures (MIP and MEP)) and endurance (2 min incremental threshold loading test) were measured before and 20 min after i.m. neostigmine. We compared the results with those of 10 normal controls. We found no difference between patients with isolated ocular involvement and controls. Ocular myasthenia gravis patients did not improve after neostigmine. The patients with generalized myasthenia gravis had reduced baseline ventilatory muscle strength (MIP 67 cmH2O (70% of predicted), MEP 86 cmH2O (50% of pred) and endurance (mean maximal load achieved = 246 g, mean pressure at highest load (P) = 19.4 cmH2O) compared with controls. After neostigmine, there was a significant increase in MIP in patients with generalized myasthenia gravis and a trend towards an increased MEP. As a group, the patients with generalized myasthenia gravis did not demonstrate a change in their ventilatory muscle endurance after neostigmine; however, there was considerable interpatient variability in response. We conclude that patients with isolated ocular MG have normal ventilatory muscle strength when tested conventionally.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2004

Myasthenia gravis and pregnancy: clinical implications and neonatal outcome.

BACKGROUND: The myasthenia gravis is twice as common in women as in men and frequently affects young women in the second and third decades of life, overlapping with the childbearing years. Generally, during pregnancy in one third of patients the disease exacerbates, whereas in two thirds it remains clinically unchanged. Complete remission can occur in some patients. METHODS: To describe the clinical course, delivery and neonatal outcome of 18 pregnant women with the diagnosis of myasthenia gravis. Retrospective chart review of pregnant patients with myasthenia gravis, followed at the National Institute of Perinatology in Mexico City over an 8-year period. Data was abstracted from the medical records on the clinical course during pregnancy, delivery and neonatal outcome. RESULTS: From January 1, 1996 to December 31, 2003 18 patients with myasthenia gravis were identified and included in the study. The mean +/- SD maternal age was 27.4 +/- 4.0 years. During pregnancy 2 women (11%) had an improvement in the clinical symptoms of myasthenia gravis, 7 women (39%) had clinical worsening of the condition of 9 other patients (50%) remained clinically unchanged. Nine patients delivered vaginally, 8 delivered by cesarean section and 1 pregnancy ended in fetal loss. Seventeen infants were born at mean +/- SD gestational age of 37.5 +/- 3.0 weeks and a mean birth weight of 2710 +/- 73 g. Only one infant presented with transient neonatal myasthenia gravis. No congenital anomalies were identified in any of the newborns. CONCLUSIONS: The clinical course of myasthenia gravis during pregnancy is variable, with a significant proportion of patients experiencing worsening of the clinical symptoms. However, neonatal transient myasthenia was uncommon in our patient population.

Adult↗

PubMed1996

Myasthenia gravis and upper airway obstruction.

Respiratory impairment in myasthenia gravis is usually attributed to weakness of the diaphragm and thoracic chest wall muscles, and is rarely attributed to upper airway obstruction. Myasthenia gravis is characterized by weakness of the striated muscles and usually affects those innervated by the bulbar cranial nerves. Weakness of these bulbar and upper airway muscles can lead to upper airway obstruction. To our knowledge, there are only five case reports in the literature associating upper airway obstruction with myasthenia gravis. Therefore, we attempted to further define its occurrence in myasthenia gravis patients by reviewing their flow volume loops. We present a case of upper airway obstruction causing respiratory symptoms in a myasthenia gravis patient. We then surveyed a total of 61 patients with myasthenia gravis who were tested in our pulmonary function laboratory between February 1990 and August 1993. Of these 61 patients, 12 had flow volume loops and 7 of these 12 disclosed a pattern of extrathoracic upper airway obstruction. The FVC was 80% or more in five of seven patients. Our data suggest that upper airway obstruction is much more common in patients with myasthenia gravis than previously recognized. In conclusion, we recommend the performance of flow volume loops in patients with myasthenia gravis to evaluate their respiratory impairment.

Adult↗

PubMed1987

Myasthenia gravis associated with Graves' disease in Japan.

The prevalence of myasthenia gravis in Graves' disease was 0.14% in the 22,956 patients with Graves' disease who came during 1968-1979 to the Ito Hospital, Tokyo. While age at the onset of Graves' disease in patients with myasthenia gravis was slightly lower than that in those with Graves' disease without myasthenia gravis, age at the onset of myasthenia gravis in patients with Graves' disease was not different from that of myasthenia gravis patients without Graves' disease. Of 33 patients with both myasthenia gravis and Graves' disease, 8 developed myasthenia gravis first, 13 developed Graves' disease first, and in 12 the two diseases occurred concurrently. Most patients in whom Graves' disease developed first had clinical manifestations of myasthenia gravis within 2 years of the onset of Graves' disease. The dosage of an anticholinergic drug required to control symptoms decreased as the thyroid function was normalized, and no cases showed the "see-saw phenomenon".

Adolescent↗

PubMed1995

[Development of myasthenia gravis after thymectomy for thymoma].

Impaired neuromuscular transmission in myasthenia gravis is caused by autoimmune mechanisms. The association of myasthenia gravis with pathological abnormalities of the thymus has been known for a long time and was the reason why thymectomy was used in the treatment of myasthenia gravis before the immunocompetent impact of the thymus and the autoimmune pathogenesis of myasthenia was known. Forms of myasthenia refractory to thymectomy, and in particular the development of myasthenia gravis after thymectomy on account of a thymoma, indicate the fact that in case of destruction of postsynaptic acetylcholine receptors an important part is played by extrathymic humoral and cellular autoimmune mechanisms. In the submitted paper the authors describe three patients where myasthenia gravis was manifested several months to 14 years after thymectomy on account of a thymoma. The presented facts emphasize the importance of immunosuppressive treatment of patients with myasthenia gravis.

Adult↗

PubMed2005

Autoimmune Myasthenia Gravis: Recommendations for Treatment and Immunologic Modulation.

Treatment for myasthenia gravis should be individualized to each patient based on the clinical characteristics of myasthenia including the distribution, duration, and severity of weakness and resulting functional impairment; the risks for treatment complications related to age, gender, and medical comorbidities; and the presence of thymoma. Acetylcholinesterase inhibitors provide temporary, symptomatic treatment for all forms of myasthenia gravis. Immune modulators address the underlying autoimmune process in myasthenia gravis, but are associated with potential complications and side effects. Most patients with generalized myasthenia who have significant weakness beyond the ocular muscles and who remain symptomatic, despite treatment with cholinesterase inhibitors, are candidates for immune modulation. Although corticosteroids are effective for long-term immune modulation in myasthenia gravis, several more contemporary immunomodulators including azathioprine, cyclosporine, and mycophenolate mofetil have shown efficacy in myasthenia gravis and are used increasingly as first-line treatments and as steroid-sparing agents. Plasma exchange is used to achieve rapid improvement in patients with myasthenic crisis or exacerbation, to improve strength before a surgical procedure or thymectomy, and to minimize steroid-induced exacerbation in patients with oropharyngeal or respiratory muscle weakness. Intravenous immunoglobulin represents an alternative to plasma exchange in patients requiring relatively rapid short-term improvement in the setting of poor venous access. Because of a lack of controlled trials, the role of thymectomy in nonthymomatous myasthenia gravis is unclear, although evidence suggests that thymectomy increases the probability for myasthenic remission or improvement.

Journal Article↗

PubMed2004

The effect of prednisone on the progression from ocular to generalized myasthenia gravis.

Fifty percent of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia, 90% within 3 years from the onset of ocular symptoms. This study was performed to determine whether treatment with oral prednisone initiated and completed within 2 years from the onset of ocular symptoms would affect the progression of ocular myasthenia to generalized myasthenia gravis (GMG). Fifty-six patients were included in this review, with 27 patients in the prednisone-treated group and 29 patients in the untreated group. The treated group was initiated on 60 mg of prednisone daily with a slow taper over 3-6 months. At 2 years, significantly fewer patients in the treated group (3 of 27) progressed to generalized myasthenia when compared to the untreated group (10 of 29) (chi(2), p=0.04). Our results suggest that the early use of steroids may decrease progression of ocular to generalized myasthenia gravis. The decision to use steroids should be considered early in the course of patients diagnosed with ocular myasthenia gravis. This study should be considered preliminary and a prospective trial is warranted to confirm our observations.

Cholinesterase Inhibitors↗

PubMed2002

Masticatory performance in patients with myasthenia gravis.

Masticatory muscle electromyograms (EMGs) were recorded while patients with bulbar myasthenia gravis chewed artificial food and compared with those of patients with ocular myasthenia gravis, patients in clinical remission who had previously suffered from bulbar myasthenia gravis and healthy individuals. Masticatory performance and EMGs were significantly smaller in the bulbar group. There were no indications of subclinical masticatory muscle weakness in patients with bulbar myasthenia gravis in remission and in patients with ocular myasthenia gravis. Patients with bulbar myasthenia gravis barely compensated for muscular weakness by chewing at a higher percentage of their maximal EMG. These quantitative findings, when combined with subjective reports of masticatory muscle weakness, show that a need to support the jaw is characteristic of patients with bulbar myasthenia gravis who produce low EMG activity.

Adult↗

PubMed1986

Early-onset myasthenia gravis.

Signs of myasthenia gravis developed by age 3 years in 11 children. Six of these patients had persistent neonatal myasthenia gravis, a familial abnormality of neuromuscular transmission that is not immunologically mediated. Five patients had juvenile onset myasthenia gravis, an autoimmune disorder similar to myasthenia gravis in adults. Autoimmune myasthenia has rarely been recognized by age 3 years, but the presence of five cases in our series suggests that the disorder may be more common in young children than once believed. The development of anti-acetylcholine receptor antibody assays makes it easier to distinguish autoimmune myasthenia gravis from the congenital forms. This distinction is important, because the prognosis, treatment, and risk of recurrence in family members is different for each type of myasthenia.

Antibodies↗

PubMed2005

Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravis.

PURPOSE OF REVIEW: Some of the 20% of myasthenia gravis patients who do not have antibodies to acetylcholine receptors (AChRs) have antibodies to muscle specific kinase (MuSK), but a full understanding of their frequency, the associated clinical phenotype and the mechanisms of action of the antibodies has not yet been achieved. Moreover, some patients do not respond well to conventional corticosteroid therapy. Here we review recent clinical and experimental studies on MuSK antibody associated myasthenia gravis, and summarize the results of newer treatments for myasthenia gravis. RECENT FINDINGS: MuSK antibodies are found in a variable proportion of AChR antibody negative myasthenia gravis patients who are often, but not exclusively, young adult females, with bulbar, neck, or respiratory muscle weakness. The thymus histology is normal or only very mildly abnormal. Surprisingly, limb or intercostal muscle biopsies exhibit no reduction in AChR numbers or complement deposition. However, patients without AChR or MuSK antibodies appear to be similar to those with AChR antibodies and may have low-affinity AChR antibodies. A variety of treatments, often intended to enable corticosteroid doses to be reduced, have been used in all types of myasthenia gravis with some success, but they have not been subjected to randomized clinical trials. SUMMARY: MuSK antibodies define a form of myasthenia gravis that can be difficult to diagnose, can be life threatening and may require additional treatments. An improved AChR antibody assay may be helpful in patients without AChR or MuSK antibodies. Clinical trials of drugs in other neuroimmunological diseases may help to guide the treatment of myasthenia gravis.

Autoantibodies↗

PubMed1984

Myasthenia gravis in Chinese children.

A clinical study of 50 Southern Chinese myasthenic children observed for periods of two to 18 years (six years on average) revealed manifestations different from those of caucasian patients. Onset was early, at an average of 4.8 years. 82 per cent had ocular myasthenia. Ophthalmoplegia followed ptosis between three months and 10 years later. Additional facial and isolated limb-muscle fatigability developed in only 6 per cent within three months to 3 1/2 years. Only 12 per cent developed generalized myasthenia. Although extension from ocular to the generalized form did not occur later than 20 months after onset, a deterioration in ocular symptoms, without extension into generalized myasthenia, occurred in nine of 18 children during adolescence. Ptosis and generalized myasthenia responded better to anticholinesterase and/or prednisone. Ophthalmoplegia was difficult to treat. The natural clinical course was benign. Spontaneous remission occurred in 62 per cent of cases, but 54.8 per cent of these relapsed, all confined to ocular muscles. Although there was no familial occurrence of myasthenia gravis, an association was found between myasthenia and thyroid disorders in some patients and their relatives. The association with HLA BW46 antigen was striking. Acetylcholine receptor antibodies were absent in the majority, but mildly elevated titres were found in three of five patients whose ocular symptoms deteriorated during adolescence, without extension into generalized myasthenia.

Adult↗

PubMed1992

Repetitive stimulation of phrenic nerves in myasthenia gravis.

BACKGROUND: In the investigation of patients with myasthenia gravis, repetitive supramaximal stimulation of an affected peripheral nerve is commonly performed to detect abnormal transmission at the neuromuscular junction. A study was undertaken to determine whether abnormal transmission could similarly be detected during stimulation of the phrenic nerves. METHODS: The phrenic nerves were stimulated supramaximally with surface electrodes in 13 patients with myasthenia gravis and in 16 control subjects (six control patients with diaphragmatic weakness but not with myasthenia and ten normal subjects). The amplitude of diaphragm muscle action potentials was measured with surface electrodes during phrenic nerve stimulation at frequencies of 1-5 Hz for 3-4 seconds. RESULTS: In five patients with myasthenia gravis, a significant decrement (15-43% decrease) occurred in the amplitude of diaphragm muscle action potential during stimulation at 3 Hz. When stimulation frequency was reduced to 1 Hz, diaphragm muscle action potentials returned to their original amplitude within 4-5 seconds. The decrement in the amplitude of the diaphragm muscle action potential was reduced temporarily in three of four patients after the administration of intravenous edrophonium chloride (Tensilon). There was no significant change (< 10% decrease) in the amplitude of diaphragm muscle action potentials during stimulation at increased frequencies either in the 16 control subjects or in eight of the patients with myasthenia gravis. CONCLUSION: A significant reduction in the amplitude of diaphragm muscle action potential occurred in five of 13 patients with myasthenia gravis during phrenic nerve stimulation at 3 Hz but in none of the control subjects. This may be a useful and non-invasive method for identifying patients with myasthenia gravis in whom weakness of the diaphragm is suspected.

Action Potentials↗

PubMed1994

Breathing pattern and central ventilatory drive in mild and moderate generalised myasthenia gravis.

BACKGROUND: Myasthenia gravis is a specific autoimmune disease characterised by weakness and fatigue. Respiratory muscle weakness has been studied using the determination of maximal respiratory pressures, but the response of respiratory centres is not well characterised. This study was undertaken to determine the breathing pattern and the central ventilatory drive in patients with mild and moderate generalised myasthenia gravis. METHODS: Twenty four patients with myasthenia gravis were studied, divided into two groups. Group 1 included 13 subjects (eight women and five men aged 23-64) with mild generalised myasthenia gravis, and group 2 was composed of 15 patients (11 women and four men aged 23-69) with moderate generalised myasthenia gravis. A control group comprised 15 healthy persons with a similar age and sex distribution. Spirometric measurements and maximal respiratory pressures were performed under basal conditions in all subjects, and the rate and depth of breathing and the inspiratory occlusion pressure in the mouth in the first 0.1 second (P0.1) were measured. RESULTS: No difference was detected for parameters of breathing pattern between patients in group 1 and control subjects, although P0.1 was higher in those in group 1. Subjects in group 2 had a lower tidal ventilation, shorter inspiratory time, and a higher frequency with a higher P0.1 than control subjects. CONCLUSIONS: Mild myasthenia gravis causes increased neuromuscular drive with a normal breathing pattern. Moderate myasthenia gravis is characterised by a more rapid shallow breathing pattern.

Adult↗

PubMed1992

Follow-up study of muscle function in children of mothers with myasthenia gravis during pregnancy.

Most infants whose mothers have myasthenia gravis are healthy at birth, but 10% to 15% have a transient neonatal form of myasthenia gravis. In this study, the muscular function and neuromuscular transmission were examined in 31 children, aged 3 months to 31 years (median, 10 years), of 15 myasthenic mothers. Eleven of these children had had the neonatal form of myasthenia gravis. The children were examined clinically and with neurophysiologic methods. Blood samples were taken for HLA typing, creatine kinase levels, and myoglobin and acetylcholine receptor antibody studies. Twenty-nine of the 31 children had no signs of neuromuscular disease. Two children (who had had neonatal myasthenia gravis) had a moderate stationary myopathy, probably unrelated to the myasthenia gravis of their mother. Creatine kinase levels were normal for all subjects. Acetylcholine receptor antibody levels were similar to those of a control population. The HLA type B8 antigen was not significantly more prevalent in the children who had had neonatal myasthenia gravis than in the healthy children. Neonatal myasthenia gravis in a previous sibling was the only factor in the material that predicted the occurrence of myasthenic symptoms in the neonatal period.

Adolescent↗

PubMed1979

Limb muscle endplates in ocular myasthenia gravis: quantitative ultrastructural study.

Motor endplate ultrastructure in the biceps brachii was quantitatively analyzed in five patients with ocular myasthenia gravis (56 endplates), six patients with generalized myasthenia gravis (83 endplates), and five controls (64 endplates). Ultrastructural changes of the motor endplates were observed in nonweak limb muscles of patients with ocular myasthenia gravis as well as in generalized myasthenia; these changes were mostly restricted to the postsynaptic region. Decrease of the mean presynaptic and postsynaptic membrane length, and postsynaptic membrane density, were more remarkable in generalized myasthenia than in ocular myasthenia. Widening of the primary and secondary synaptic clefts was also observed more frequently in generalized myasthenia. There was no correlation between ultrastructural alterations and the duration of symptoms or treatment, severity of disease, or titers of antiacetylcholine receptor antibody.

Adolescent↗