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At least 289 records · Page 16Linked to original sources
PubMed2000

Extraocular muscle responses to high dose intravenous methylprednisolone in myasthenia gravis.

Three patients with generalised myasthenia gravis and three with ocular myasthenia gravis received two to five courses of high dose intravenous methylprednisolone because of the failure of standard immunomodulating therapies. Changes in myasthenic signs were assessed using a four step system for grading muscle weakness and fatiguability in 10 test items. Although a brief and modest amelioration was found from day 1 to day 2 after the initial infusion in two patients with generalised myasthenia gravis, all three experienced a prolonged phase of worsening followed by improvement before the next course. Conversely, for two of the patients with ocular myasthenia gravis, a transient but dramatic improvement of ptosis and ocular immobility was noted from 90 minutes to 5 hours after initiating the first infusion, followed by mild or no exacerbation. This 3 hour improvement may be related not only to possible differences in the neuromuscular junction, but also to corticosteroids unmasking the central adaptation for the peripheral ocular muscle weakness by increasing the acetylcholine release.

Acetylcholine↗
PubMed1982

Acetylcholine receptor antibody characteristics in myasthenia gravis. I. Patients with generalized myasthenia or disease restricted to ocular muscles.

We have investigated anti-acetylcholine receptor (AChR) characteristics in three forms of generalized myasthenia gravis, which are distinguishable by their thymic pathology, age of onset, sex incidence and HLA antigen associations, and in restricted ocular myasthenia. Light chain, IgG subclass, avidity for denervated human AChR and reaction with various human and mammalian AChR preparations were examined in 9-12 patients from each group. Characteristics varied between individuals in each group but no significant differences were found in mean values between the three groups with generalized myasthenia. When antibody characteristics in ocular myasthenia were compared with those in generalized disease, however, differences were found particularly in relative reactivity with denervated, normal and ocular human AChR. The nature of the humoral response in ocular myasthenia thus differs from that in generalized myasthenia. The similarity in the humoral response between the three groups of generalized myasthenia suggests, on the other hand, that the distinguishing clinical features may reflect differing susceptibilities to diverse precipitating mechanisms.

Adult↗
PubMed1982

Acetylcholine receptor antibody characteristics in myasthenia gravis. II. Patients with penicillamine-induced myasthenia or idiopathic myasthenia of recent onset.

Anti-acetylcholine receptor (anti-AChR) antibody characteristics including light chain, IgG subclass, avidity for denervated human acetylcholine receptor and reaction with various human and mammalian AChR preparations were examined in 11 patients who developed myasthenia during penicillamine treatment of rheumatoid arthritis. Results were compared with those already reported in 35 patients with generalized idiopathic myasthenia gravis (MG). We found significant differences in the avidity and the light chain of the anti-AChR. However, anti-AChR characteristics in 12 patients with recent onset (less than 4 months' duration) idiopathic MG did not differ significantly from those in patients with penicillamine-induced MG. In the patients with generalized MG a trend was found towards higher percentage of kappa light chain and higher anti-AChR avidity with duration of disease. Anti-acetylcholine receptor antibodies in penicillamine-induced myasthenia gravis therefore appear to be similar to those of idiopathic myasthenia gravis of recent onset.

Adolescent↗
PubMedDate not supplied

Voice spectrography in the evaluation of myasthenia gravis of the larynx.

Myasthenia gravis is a neuromuscular disorder that affects striated muscles especially those innervated by the cranial nerves. The standard diagnostic regimen is to find a reversal of symptoms by acetylcholine esterase administration. The permanent and objective recording of this effect is the key to an accurate assessment of this test. The voice spectrograph is a noninvasive means of evaluating the voice. It has been used successfully to make a clear and specific diagnosis of myasthenia gravis as it affects the larynx. The test can be used to distinguish myasthenia gravis from other functional, anatomical and neuromuscular laryngeal disorders.

Adult↗
PubMed1997

Altered tumor growth factor beta mRNA expression is associated with thymectomy-related clinical remission in myasthenia gravis.

Clinical remission in myasthenia gravis after thymectomy and immune suppressive treatment may be reflected by changes of immunoregulatory cytokines. Mononuclear cells from paired blood samples from fifteen patients with myasthenia gravis before and after thymectomy and immunosuppressive therapy were examined by in situ hybridization for acetylcholine receptor-induced mRNA expression of the T helper type 1 proinflammatory cytokines interferon gamma (IFN-gamma), tumor necrosis factor alpha (TNF-alpha) and TNF-beta, the T helper type 2 cytokines interleukin 4 (IL-4) and IL-10 and the immune response downregulating cytokine tumor growth factor beta (TGF-beta). A significant change was observed for TGF-beta, with increased expression after treatment and induction of clinical remission. Augmentation of TGF-beta may play a role in the induction of remission in myasthenia gravis.

Adolescent↗
PubMed1998

Myasthenia gravis with alopecia totalis.

Myasthenia gravis was diagnosed on the basis of a positive Tensilon test in a 4-year-old male child with alopecia totalis. His scalp hair and eyebrows had began to disappear at the age of 10 months. No other physical abnormalities such as motor paralysis, were seen except for left ptosis and alopecia totalis. His serum titer of anti-acetylcholine receptor antibody was elevated. No immune system abnormalities (C3, C4, CH50, C-reactive protein, antinuclear antibody or lymphocyte function disorders) were detected. Although alopecia areata and alopecia totalis are sometimes present in adults with autoimmune diseases and myasthenia gravis, this association is rare in children. The present case represents the youngest patient with myasthenia gravis associated with alopecia totalis.

Alopecia↗
PubMed1979

Orbicularis fatigue: the 'peek' sign of myasthenia gravis.

Three patients with myasthenia gravis demonstrated a distinctive sign of orbicularis fatigue. After momentary opposition on gentle sustained lid closure, the lid margins separated, resulting in widening of the palpebral fissure and scleral exposure. The patient thus appeared to "peek" at the examiner. The "peek" sign was not observed in 200 control patients and in 49 of 50 patients with facial weakness secondary to a spectrum of neuromuscular disorders. Three of 25 patients with myasthenia gravis displayed this sign, and in one patient it was the major diagnostic finding. The presence of the "peek" sign suggests the diagnosis of myasthenia gravis.

Adult↗
PubMed1989

Cervical thymectomy in the treatment of myasthenia gravis.

Treatment modalities in myasthenia gravis consist of surgery, chemotherapy and plasmapheresis. Thymectomy can be accomplished either through a median sternotomy or through a small, transverse cervical incision. Forty patients who underwent cervical thymectomy for non-thymomatous myasthenia gravis were studied retrospectively. Twenty-six patients (65%) showed a favourable response to thymectomy and there were statistically significant improvements in myasthenic symptoms and reductions in medication requirements. Age, sex, duration of symptoms and thymic histology were not predictive of response to thymectomy. Operative mortality was zero and operative morbidity was minimal. During the last 6 years, only two of 22 patients required admission to the intensive care unit postoperatively. The postoperative hospital stay ranged from 2 to 23 days. Cervical thymectomy does not preclude later sternotomy in those patients who fail to respond favourably. We therefore recommend cervical thymectomy as the initial surgical procedure in the treatment of non-thymomatous myasthenia gravis.

Adolescent↗
PubMed1992

Role of thymectomy in myasthenia gravis.

Thirteen patients with myasthenia gravis underwent total thymectomy between January, 1988 and December, 1991. The duration of symptoms prior to surgery varied from 2 months to 20 years. In a follow-up ranging from 2 months to 4 years, 11 patients showed a significant improvement with either complete discontinuation of medication or a marked reduction in doses. One patient with a small benign thymoma showed some improvement but subsequently required stepping up of anticholinesterase medication and addition of steroids and immunosuppressants; another patient with atrophic thymic tissue had complete remission after thymectomy but developed myasthenic symptoms six months later requiring medication again. Thymectomy is recommended for all patients with generalised myasthenia gravis with or without thymoma regardless of the duration of disease unless the patient is a very high risk candidate for surgery. It is not recommended for isolated ocular myasthenia gravis.

Adolescent↗
PubMed2004

Myasthenia gravis: how to treat?

Myasthenia Gravis is an acquired autoimmune disorder caused by a neuromuscular transmission defect which is clinically characterized by fluctuating weakness of voluntary muscles and fatigability. It can be diagnosed by clinical features, clinical, pharmacological and electrophysiological tests and serological evaluation. Treatment modalities include symptomatic treatment in the form of cholinesterase inhibitors and plasmapheresis and immunotherapy in the form of immunosuppressant medications, immunomodulating therapy and thymectomy. No single regimen is appropriate for all patients and up to now no mode of therapy has been proven to be clearly superior. The response to any form of treatment is difficult to assess because the severity of symptoms fluctuate. We retrospectively analyzed the clinical records of 33 myasthenia gravis patients which were managed at our clinic between 1995-2003. All patients were treated with anticholinesterase medications sometime during their treatment. Most patients recieved immunosupressant and/or immunomodulator therapy. Patients were referred for thymectomy when indicated. We evaluated the outcome with different treatment modalities, focusing on the role of thymectomy. We also investigated the possible correlations between clinicopathological features and clinical outcome. We conclude that as for the medical treatment of myasthenia gravis azathioprine plus steroid improves the outcome; and for the surgical treatment, early thymectomy should be performed in all generalize myasthenia patients.

Adolescent↗
PubMed1991

Autoantibodies in D-penicillamine-induced myasthenia gravis: a comparison with idiopathic myasthenia and rheumatoid arthritis.

The distribution of autoantibodies was studied in patients with rheumatoid arthritis (RA) treated by D-penicillamine and who developed myasthenia gravis (MG). The anti-human acetylcholine receptor (AChR) antibodies were specifically associated with clinical symptoms of MG without any difference in the pattern of specificities in idiopathic (id-MG) or in induced MG (DPen-MG). Conversely, anti-nuclear antibodies were elevated in DPen-MG sera compared to id-MG sera (P less than 0.001) but were also compared to patients with RA treated by D-penicillamine (or thiopronine) and who did not develop MG. Anti-denatured DNA antibodies were enhanced in sera from treated patients, whether they had presented or not a MG disease. Anti-histone antibodies were associated with RA. These observations suggest that the immunological imbalance in RA patients, can be increased by a drug treatment which may trigger the appearance of a second autoimmune disease such as MG, where anti-AChR antibodies are associated with anti-nuclear antibodies.

Adult↗
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↗
PubMed2005

[Atypical ocular myasthenia gravis].

The diagnosis of ocular myasthenia gravis is rarely in doubt in patients with a proper history and typical clinical findings. However, myasthenia gravis can mimick any pupil-sparing eye movement disorder and several diseases may masquerade myasthenia gravis. We review the atypical presentations and differential diagnoses in ocular myasthenia gravis, describing four patients with some of these conditions (4th nerve palsy, near spasm reflex, one-and-a-half syndrome, orbital meningioma). The correct interpretation of the clinical findings associated with appropriate neuro-imaging studies allowed the appropriate diagnosis in these cases.

Adult↗
PubMed1987

Ice pack test for myasthenia gravis.

A common symptom of myasthenia gravis is eyelid ptosis. Often, the edrophonium test is negative, particularly in ocular myasthenia gravis. Myasthenic weakness is often improved by cold. We applied ice packs to the eye in 10 myasthenic patients and 7 disease controls. Eight of 10 patients with myasthenia gravis improved, whereas none of the controls improved. The ice pack test is useful in the diagnosis of myasthenia gravis.

Adolescent↗
PubMed2005

Myasthenia gravis and pregnancy.

Myasthenia Gravis with pregnancy is a rare concomitance. This case report describes successful outcome of all pregnancies in a lady with this problem. The management is described.

Adult↗
PubMed1998

A case of myasthenia gravis in childhood associated with chronic inflammatory demyelinating polyradiculoneuropathy.

We report a 14-year-old girl who developed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) during the course of myasthenia gravis. Myasthenia gravis, which was clinically of ocular type, but with waning phenomenon of the extremities, occurred at 2 years and 4 months of age. Muscle weakness of the lower extremities gradually developed over the next 6 years. The electrophysiological and pathological findings fulfilled the criteria of "possible CIDP" with severe axonal involvement. The signs of myasthenia gravis and CIDP fluctuated synchronously. A common immunological abnormality was suggested to underlie this rare association of myasthenia gravis and CIDP in childhood.

Adolescent↗
PubMed1996

An elusive weakness: myasthenia gravis.

Individuals with myasthenia gravis often enter the health care system in crisis without a "working diagnosis". Since this disorder is potentially life threatening, especially if undiagnosed, it is essential that the front-line care provider be sensitive to the symptom patterns and complex needs of these special patients.

Adult↗
PubMed1981

Myasthenia gravis. A survey.

Myasthenia gravis is a chronic disease characterized by a fluctuating weakness of voluntary muscle, with a preference for the muscles innervated by cranial nerves. The pathophysiological mechanism is a loss of postsynaptic acetylcholine receptors to less than 20-30% so that the safety margin of neuromuscular transmission is lost. It is probable that the function of the remaining acetylcholine receptors is impaired by antibodies against receptor proteïn, which can be demonstrated in the serum in 80-90% of the patients, and which are highly specific for the disease. An experimental autoimmune myasthenia can be induced in many animal species by immunization with purified receptor proteïn and this disease is remarkably similar to the human myasthenia with exception of the fluctuating course. The human disease has to be considered as an autoimmune disease, although the initiating mechanism is unknown. The occurrence of tumors of the thymus in 10-15% and the presence of germinal centres in about 70% of the thymus glands removed by operation are highly suggestive of the importance of the thymus in the pathogenesis, but the definite mechanism (harbouring of an abnormal antigen in myoid cells, or/and false instruction of thymocytes with lack of suppressor cells) is essentially unknown. In most patients the disease tends ot have a favourable course from 5-10 years after onset and complete remission occur in about 20% after 10-20 years. Therapy with anticholinesterases, providing an increase in acetylcholine, is of partial benefit in most patients. Thymectomy has an excellent effect in about 30% of the patients without thymoma under the age of 40 during the first three years of the disease, and is of benefit in still another 30-40%. The use of prednisone and immuno-suppressive drugs has improved the prognosis of the 20% of the patients with severe life threatening symptoms, half of whom have a thymoma.

Acetylcholinesterase↗