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PubMed1992

Myasthenia gravis in pregnancy.

OBJECTIVE: Our purpose was to review our experience with nine cases of myasthenia gravis complicating pregnancy. METHODS: We reviewed the records of all patients discharged from the Vancouver Salvation Army Grace Hospital from January 1979 to January 1989 with an admission diagnosis of myasthenia gravis in pregnancy. RESULTS: Nine patients delivered 11 infants. Four suffered antepartum exacerbations. The most severe of these, respiratory failure, occurred in a thymectomized patient. Labor was rapid and uncomplicated except for a single case of cephalopelvic disproportion delivered abdominally. We noted no puerperal exacerbations. Our solitary case of neonatal myasthenia gravis was managed in the special care nursery with intramuscular neostigmine given over a 16-day period. CONCLUSION: There are no predictive factors that identify the mother at risk for peripartum exacerbation of myasthenia gravis or the infant at risk for neonatal myasthenia gravis. Optimal care for the parturient is achieved through a team approach involving the obstetrician, neurologist, and pediatrician.

Adult↗

PubMed1991

[Familial infantile myasthenia gravis].

The several forms of myasthenia gravis that occur in infancy include transient neonatal myasthenia, congenital myasthenia, and familial infantile myasthenia gravis. The latter is inherited in an autosomal recessive pattern. Severe episodes of respiratory distress are frequent in infancy, and are often provoked by mild respiratory infections. 2 sisters with congenital myasthenia gravis are described. Probably 2 other sisters died of the same disease, but no definite diagnosis was made in their cases.

Female↗

PubMed1991

[Central acetylcholine receptor function in patients with myasthenia gravis].

There are some reports on central nervous system involvements in patients with myasthenia gravis, such as abnormal EEG, and memory disturbance. Myasthenia gravis is considered to be an autoimmune disease with antibodies against the skeletal nicotinic acetylcholine receptor (n-AChR). ACh is a neurotransmitter in osmoregulation. Neuronal n-AChR plays an important role in this regulation. In order to investigate the function of neuronal n-AChR in patients with myasthenia gravis, we performed a 5% hypertonic saline infusion test on 9 patients and 9 healthy volunteers. We also carried out an orthostatic stress test (50 degree passive head-up tilt) on 6 patients with myasthenia gravis and 5 healthy controls to evaluate arginine-vasopressin (AVP) release via baroreceptors. Three of the 9 MG patients showed exaggerated plasma AVP secretion, and one revealed a blunt response to hypertonic stimulation. Both patients and controls did not differ significantly in terms of plasma AVP response to orthostatic stress. To conclude, we suggest the possibility that function of neuronal n-AChR in the central nervous system is impaired in patients with myasthenia gravis.

Adult↗

PubMed1989

[Myasthenia and pregnancy. Apropos of 8 cases].

In 10 years we dealt with 8 cases of pregnancy in myasthenic women. Pregnancy does not seem to cause myasthenia to develop. Worsening of the condition, which we found in 40% of our cases, occurs particularly in the first trimester and nearly always regresses. Another critical period is after delivery. Deaths are not unusual in the literature but we did not have to record one in our study. Because myasthenia evolves in different ways it is important to watch these cases carefully and as soon as the diagnosis has been made stabilising treatment is needed. The patient has to be watched for at least two months after the delivery. During the pregnancy the first line of treatment is to use anticholinesterases which do not have any teratogenic effects. In our study all the women carried their pregnancies to term; although in the literature 10-40% of deliveries were premature. This fact makes one consider whether there is an oxytocic effect of anti-cholinesterases. If the mother becomes very tired she should have very little expulsive effort to carry out. Forceps delivery, which is often necessary, is helped if an epidural anaesthetic is given. Breast feeding by the mother can only be carried out if the myasthenia is stable and if there are weak levels of anti-receptor antibodies to acetylcholine. 50% of the newborn are often small for dates and show transitory myasthenia after delivery. The literature, however, shows only 10-20%. This finding is not always correlated with the levels of anti-receptor antibodies for acetylcholine. If they are present, they are useful to diagnose transitory myasthenia.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1987

Misdiagnosis of myasthenia gravis.

Patients with myasthenia gravis (MG) are too often misdiagnosed as having another disorder. Three patients are presented who were thought to have amyotrophic lateral sclerosis, velopharyngeal incompetence, and no diagnosis of MG, but actually each had myasthenia gravis. Their histories illustrate how the diagnosis of myasthenia gravis can be easily missed.There is no typical case of myasthenia gravis but, rather, this entity remains a clinical diagnosis that relies on a well-taken history, adequate examination, and appropriate interpretation of laboratory tests. To miss the diagnosis of myasthenia gravis is to cause the patient social, psychological, medical, and economic suffering.

Adolescent↗

PubMed1977

[Myasthenia gravis. New results on etiology, diagnosis and therapy].

Myasthenia gravis is discussed with reference to recent immunologic findings, improvements in diagnostic procedure, and therapy. Myasthenic reactions can be produced in animals by injection of receptor proteins. In addition, the injection of thymopoietin causes neuromuscular block. The majority of patients with myasthenia gravis have antibodies against acetylcholine receptors, and there is a markedly reduced number of bungarotoxin binding receptors at the endplates. Further, recent immunologic findings indicate that genetic factors may be involved in the etiology of myasthenia gravis. The recently developed single fiber electromyography is useful in the diagnosis of myasthenia gravis. New statistics demonstrate the value of thymectomy in the treatment of myasthenia gravis. Good results have been reported following treatment with corticosteroids and azathioprine.

Adrenal Cortex Hormones↗

PubMed1979

The role of acetylcholine receptor antibodies in myasthenia gravis.

Myasthenia gravis is an autoimmune disease of man characterized by remitting and relapsing muscle fatigability. Although the etiology and pathogenesis are incompletely understood, the presence of circulating antibodies directed against the nicotinic acetylcholine (ACh) receptor in 80--90% of patients with myasthenia gravis and the identification of immune complexes at their neuromuscular junction have helped explain the altered neuromuscular transmission. The ACh receptor antibodies do not block access of ACh to the receptor, but do decrease the number of receptors by accelerating their degradation both in rat myotube cultures and in vivo models. In vitro these antibodies play a major role in myasthenia gravis. However, correlations of antibody titers with the clinical state following thymectomy or in neonatal myasthenia suggest that host factors may be equally important in determining whether the ACh receptor antibodies will result in clinical myasthenia.

Acetylcholine↗

PubMed1984

Autologous mixed lymphocyte reaction in patients with myasthenia gravis: correlation with disease activity.

Enriched subpopulations of peripheral blood mononuclear cells from patients with myasthenia gravis and from normal control individuals were tested for proliferative ability in the autologous mixed lymphocyte reactions. Myasthenia gravis patients were studied at various phases of therapy. Longitudinal patient populations were studied. Purified T cells from untreated patients with active myasthenia gravis demonstrated enhanced proliferative responses when co-cultured with nonmodified autologous non-T cells. Furthermore, treatment by thymectomy with or without prior plasmapheresis was associated with a consequent normalization of the autologous reaction. The enhanced autologous mixed lymphocyte reaction in the pre-treatment group of myasthenia gravis patients was significantly different from the post-treatment and normal control groups (Stimulation Index, p less than 0.01 and p less than 0.02, respectively). This difference in activity was also confirmed in a longitudinal study of 10 patients followed pre- and post-treatment (Stimulation Index, p less than 0.02). There was no difference between the responses of post-treatment patients and normal control individuals. There was no evidence of altered T cell responsiveness or non-T cell stimulation, as measured by the allogeneic mixed lymphocyte reaction or by mitogen-induced lymphocyte proliferation, between pretreatment and post-treatment patients as compared to controls. The responses were not affected by serum substitution experiments whereby myasthenic responder T cells were co-cultured with pooled normal human sera. Other parameters studied included clinical status, anti-acetylcholine receptor antibody levels, and thymic pathology. An intriguing connection between abnormalities in the thymic cell populations and the autoaggressive state in myasthenia may be reflected by the in vitro autologous mixed lymphocyte reaction.

Adolescent↗

PubMed1978

[Myasthenia and pregnancy (author's transl)].

In the past 25 years, we have studied 73 cases of Myasthenia. 19 of these have had 24 pregnancies. We confirm the results that are given in the literature (15). Pregnancy does not trigger off Myasthenia. The action of pregnancy on Myasthenia is variable and there is an equal chance of worsening, improvement or a stationary state of the condition. All the same, patients who are well stabilised and have been for some time do not become worse. Worsening, when it does happen, happens especially in the first trimester of the pregnancy and calls for precise observation of the patient in order to regulate the treatment. Myasthenia has no direct or indirect action on the fetus and the pregnancy is not disturbed. Labour can be normal but the second stage often has to be aided because of the patient's muscle tiredness. In labour and particularly in the second stage an acute crisis can occur with difficulty in breathing. For this it is necessary to have an anaesthetist present who can resuscitate the patient and if necessary give respiratory aid by ventilation. Curare and like substances are absoultely contra-indicated if general anaesthesia is required. Transitory neonatal Myasthenia is rare, but the severity of the respiratory complications can be such that is should be sought for routinely and if there is the slightest doubt the newborn should be transferred to the special care neonatal unit.

Anesthesia, Obstetrical↗

PubMedDate not supplied

[Clinical and instrumental study of familial congenital myasthenia].

Congenital familial myasthenia occurred in two male brothers, born from non affected mother. The diagnosis was based on anamnestic and clinical data, on electromyographic features, and on the effect of specific therapy. The differential diagnosis and the classification of congenital familial myasthenia are discussed. This type of disease represent 1% of all cases of myasthenia, but--excluding the "transferred" myasthenia to the newborn by affected mother--the percentage raises to 3--4%. The possibility of identifying two distinct formes of familial myasthenia is emphasized, the first with "early onset" where the symptoms become evident before two years of age, the second "late onset" clinically evident later in life seems doubtful that the two forms of the disease might be due to the same underlying pathological condition.

Echoencephalography↗

PubMed1994

Myasthenia gravis after allogeneic bone marrow transplantation.

A 37-year-old Japanese man with chronic myeloid leukemia (CML) developed myasthenia gravis 29 months after bone marrow transplantation (BMT) from an HLA one locus-mismatched brother. Proximal muscle weakness and bilateral ptosis occurred along with the exacerbation of chronic GVHD shortly after sudden cessation of cyclosporine (CYA) and prednisolone. The diagnosis of myasthenia gravis was made based on clinical symptoms and elevation of an anti-acetylcholine receptor antibody titer and all symptoms related to myasthenia gravis promptly diminished with the start of treatment for chronic GVHD. In most previously reported cases, the underlying disease was aplastic anemia (6 of 7 cases) and donors were of the opposite sex (6 of 7 cases). The haplotypes HLA B7 (3 of 5 cases), B35 (3 of 5 cases), and DR2 (3 of 3 cases) were common. All cases suffered from chronic GVHD. The present case had only chronic GVHD and HLA B7 as a background for myasthenia gravis after BMT. The abrupt cessation of immunosuppressive therapy may also be related to the development of myasthenia gravis after BMT.

Adult↗

PubMed1994

[Thirty years of experience in the surgical treatment of patients with generalized myasthenia].

The article deals with 30-year experience of the Burdenko Facultative Surgical Clinic in surgical management of generalized myasthenia. The analysis is based on 2,500 cases. This complex disease was worked out in stages, taking into account the advancements of the medical science in the given stage. It is noted that the immediate and late-term results of surgery improved after introduction of the newest methods of examination and treatment of patients with myasthenia into practice. Among these methods are CT and MRT, immunohistochemical study, and treatment with steroids and immunodepressants. The role and significance of sorption methods and splenectomy in combined treatment of serious forms of myasthenia are pointed out. The elaboration of the clinico-immuno-morphometric criteria allowed the authors to prognosticate the results of surgical treatment with high authenticity and distinguish two types of myasthenia, as a result of which the indications for surgery could be determined more precisely. The authors direct the way to further research in this field for improvement of the diagnosis and the results of treatment of generalized myasthenia.

Combined Modality Therapy↗

PubMed1994

[D-penicillamine-induced myasthenia gravis].

D-penicillamine has been used in the treatment of rheumatoid arthritis for years. As a rare complication of this treatment the occurrence of myasthenia gravis has been described, the clinical features of this complication being indistinguishable from that of idiopathic myasthenia gravis. Both D-penicillamine induced and idiopathic myasthenia gravis show elevated titers of acetylcholine receptor antibodies and respond to acetylcholinesterase inhibitor treatment. We report on a patient with rheumatoid arthritis who, under treatment with D-penicillamine, developed severe myasthenia gravis which required temporary acetylcholinesterase inhibitor therapy. 8 months after D-penicillamine was discontinued the acetylcholine receptor antibodies had disappeared and the acetylcholinesterase inhibitors could be withdrawn. Clinical findings and possible pathogenetic aspects of D-penicillamine induced myasthenia gravis are discussed.

Aged↗

PubMed1996

[Follow-up and immunologic findings in drug-induced myasthenia].

PATIENT AND METHOD: We report 5 patients, who developed myasthenia, four of them after treatment on D-penicillamine, one after treatment on chloroquine. 3 patients suffered from rheumatoid arthritis, one from a psoriatic arthritis and one from cirrhosis of the liver. Three patients developed an ocular myasthenia, one patient an oculopharyngeal and one patient has had generalized myasthenic syndrome. RESULTS: Four patients showed an improvement of clinical status within days up to 18 months following discontinuation of the therapy, whereas one patient deteriorated. The quickest improvement was observed in the patient with chloroquine induced myasthenia. Four patients had raised acetylcholine-receptor antibody titers. The patient with chloroquine induced myasthenia had had a normal acetylcholin-receptor antibody titer. The human leucocyte antigen type was compared with the results of literature. CONCLUSION: To what extent human leucocyte antigen type HLA DR 4 has a correlation with the development of myasthenia following treatment on chloroquine can not yet be answered with respect to the very small number of cases at the moment.

Aged↗

PubMed1997

A rare association of myasthenia gravis and mitochondrial myopathy: a clinical, biochemical and morphologic study of one case.

Myasthenia gravis and mitochondrial myopathy may present with similar clinical symptoms as inconstant palpebral ptosis, ophthalmoparesis, and muscle weakness. A few case initially diagnosed as myasthenia gravis by a positive decremental response on EMG and successful anticholinesterase therapy revealed to be affected by mitochondrial disease. We report a new case initially found to be affected by myasthenia gravis in whom muscle biopsy, performed because of symptom worsening, disclosed a mitochondrial myopathy. It is not clear if the association of mitochondrial myopathy and myasthenia gravis is coincidental or if there is a pathogenic link between the two pathologies. We suggest that muscle biopsy should be performed in cases with atypical myasthenia gravis signs.

Aged↗

PubMed1997

[Electroglottographic studies in myasthenia gravis patients--swallowing in exacerbation and remission stage].

We quantitatively evaluated the electroglottographic changes in swallowing in patients with generalized myasthenia gravis during exacerbation and remission stages. Five patients with myasthenia gravis (Osserman's stage IIB, 55.3 +/- 3.1 years old) and 6 healthy control subjects (55.3 +/- 1.1 years old) were examined. The instrument delivered a 450 microA. 52 kHz carrier signal to a pair of surface electrodes on each side of the larynx at the level of the thyroid lamina. Tissue impedance across the neck was recorded via electroglottography (EGG) while swallowing 10 ml of water. Four trials were performed for each subject, and the EGG waveform was analyzed. The EGG waveform during the exacerbation stage exhibited a more significantly increased number of phases and longer duration than that during remission. In myasthenia gravis patients, the number of phases and the duration from exacerbation to remission was more significantly reduced than those in controls at an interval of 3 years (p < 0.001, p < 0.02). These findings suggest that changes in the number of phases and duration of the EGG waveform are objective and quantitative parameters for measuring therapeutic effectiveness in treating swallowing disturbance due to myasthenia gravis. EGG is a useful and noninvasive technique for evaluating swallowing change in myasthenia gravis patents.

Deglutition↗

PubMed1998

[Ocular myasthenia: clinical course and strategies for treatment].

INTRODUCTION: Ocular myasthenia gravis is a localized form of myasthenia in which only the extra-ocular muscles are clinically affected, namely the levator palpebrae superioris and orbicularis oculi. Two years after onset of the ocular condition, it became generalized in 44-53% of the patients. OBJECTIVE: 1. To describe the clinical features, diagnostic characteristics and clinical course of seven patients who fulfilled the criteria for diagnosis of ocular myasthenia and in whom the condition did not become generalized: 2. Review recent papers on this. Material and methods. We studied seven patients (two men and two women) diagnosed as having ocular myasthenia gravis, and followed them up for at least three years. RESULTS: The average age was 56.5. The clinical findings were of ptosis of the eyelids and diplopia. All seven patients were treated with pyridostigmine. In six cases prednisone was also given and in one patient thymectomy was done. There was a satisfactory result in all cases. CONCLUSIONS: The basic treatment of ocular myasthenia is with anticholinesterases and corticosteroids. Occasionally other immunosuppressives may be required. Prednisone seems to reduce the number of patients who go on to develop the generalized form.

Adult↗

PubMed1998

Influence of age on extended thymectomy as a treatment for myasthenia gravis.

This retrospective study examines the influence of age on extended thymectomy as a treatment for myasthenia gravis. From 1979 to 1997, 58 patients with myasthenia gravis underwent extended thymectomy at Miyazaki Medical College Hospital. We used the onset age of the disease to divide these patients into two groups: Group A included 14 patients >/= 50 years of age; Group B, 44 patients < 50. Using Osserman's classification and the duration of the disease prior to hospitalization, we observed no significant differences between the two groups although thymoma were more prevalent in Group A. All 58 patients underwent extended thymectomy, with no surgical mortalities. The mean duration of tracheal intubation after thymectomy for Group A was 6.25 days; for Group B, 4.66 days without statistically significant differences between both groups. Using Masaoka's criteria, we evaluated the clinical course of myasthenia gravis following extended thymectomy for each of the 58 patients. The remission rates in Groups A and B were 28.6% and 29.5%, respectively; the improvement rates, 71.4% and 79.5%, respectively with no significant differences among groups. These findings suggest that the clinical course of myasthenia gravis following extended thymectomy is not age-dependent and that extended thymectomy is a clinically safe and effective treatment option for myasthenia gravis patients >/= 50 years of age.

Adolescent↗