Electroconvulsive therapy in myasthenia gravis.

BACKGROUND: Myasthenia gravis (MG) is a neuromuscular disease sometimes associated with severe psychiatric complications. The use of electroconvulsive therapy (ECT) in MG raises certain challenges. METHODS: We describe a patient with MG and a steroid-induced major depressive episode with psychotic features treated with ECT. We also review the literature on similar cases and on the safety of ECT with muscle relaxation in this condition. RESULTS: The use of ECT in patients with MG is a viable therapeutic option when psychiatric complications secondary to MG or its treatment do not respond to psychotropic medications. CONCLUSION: ECT with muscle relaxants could be administered safely, with appropriate precautions kept in mind.     

High-dose intravenous immunoglobulin therapy for myasthenia gravis

The objective of this open, retrospective study was to investigate whether intravenous immunoglobulin (IVIg) could induce a clinically obvious improvement in patients with generalized myasthenia gravis (MG), as judged by MG functional status. Fourteen patients with generalized MG were treated during at least one episode with 0.4 g IVIg per kilogram body weight per day for 5 consecutive days. Patients with confounding variables were excluded; this left 11 patients (16 episodes) to be further analysed. We defined improvement as at least a one-step improvement in MG functional status (according to the University of Virginia’s Modification of Osserman’s classification). Of the treatment episodes, 56% were classified as positive responses. If improvement occurred, onset of improvement started after 3 (1–12) days and peak effect was reached after 7 (4– 30) days (median and range). All four patients who required artificial ventilation could be weaned from it 8.5 (6–11) days after the start of IVIg (median and range). Of the patients treated on two occasions, only one patient had a positive response during both. In MG functional status 5, improvement was observed during five of seven episodes. None of the patients with MG functional status 3 responded. Patients with an acute relapse of MG seemed to respond equally well to IVIg compared with patients with subacute deterioration/ chronic-static state (50% versus 60%). The MG functional status at the start of IVIg and on the day of maximal improvement was compared for all episodes together, and significant improvement was noted (P = 0.0052). We did not see any serious side-effects after IVIg treatment. This retrospective analysis suggests that high-dose IVIg is an effective therapy in some patients with deterioration of generalized MG. If improvement occurs, it starts within a few days of the onset of IVIg and the effect seems to peak within 2 weeks. Received: 4 March 1997 Received in revised form: 28 August 1997 Accepted: 1 September 1997     

Plasmapheresis therapy in myasthenia gravis

Plasmapheresis in the treatment of myasthenia gravis has led to disparate results when applied by different investigators because of considerable differences in the volume, number, and tempo of plasmaphereses and in the type and amount of concomitant immunosuppressive drug therapy. Used as short-term crisis intervention, plasmapheresis produces temporary clinical improvement and reduction in titer of antibody to acetylcholine receptor, even without accompanying drug therapy. When applied as long-term primary therapy under optimal conditions, plasmapheresis is capable of generating stable improvement in most patients. This response appears to result from a synergistic action with immunosuppressive drugs, since it is characterized by a sustained reduction in titer of antibody to acetylcholine receptor. Where clinical circumstances warrant cytotoxic immunosuppression in patients with myasthenia gravis, consideration should be given to the simultaneous employment of plasmapheresis, in order to maximize benefit to the patient from a given exposure to drug therapy.     

免疫稳态与重症肌无力的目标治疗

<正>内环境的恒定是一切生命的需要,人类在长期进化过程中发展出一整套平衡调节机制,免疫平衡是其中重要组成部分。重症肌无力(MG)是慢性自身免疫性疾病,其本质是免疫失衡导致的免疫损伤。长期以来,由于重症肌无力的异质性和症状的波动性,大多数患者需长达数年的治疗[1]。因此,需     

Monitoring azathioprine therapy in myasthenia gravis

Azathioprine (AZA) is used increasingly in the treatment of selected patients with myasthenia gravis (MG). The "usual" dose is 2 to 3 mg/kg/d, but guidelines do not exist to determine a specific dose for an individual patient. We reviewed our previously reported MG patients to determine what laboratory studies correlated with therapeutic efficacy. Among the studies examined, red cell mean corpuscular volume (RBC MCV) was the most useful: in 10 patients who responded to AZA, MCV increased by 15 +/- 2 fl (mean +/- SD), while in 6 nonresponders, MCV increased by only 4.5 +/- 6 fl (p less than or equal to 0.01). RBC MCV may be helpful in monitoring AZA therapy in patients with MG.     

Topical therapy for oropharyngeal symptoms of myasthenia gravis

Inhibitors of acetylcholinesterase are commonly used in the treatment of myasthenia gravis. We studied a patient with mainly bulbar myasthenia gravis who did not tolerate oral pyridostigmine despite a clear clinical response. Treatment with nebulized pyridostigmine reduced her symptoms without systemic side effects. This route of therapy may benefit other patients.     

Immunosuppressive drug therapy in myasthenia gravis

We reviewed the records of 37 patients with myasthenia gravis treated with azathioprine (n = 10) or cyclophosphamide (n = 27). All patients had received prednisone and anticholinesterase therapy, and most had undergone thymectomy prior to immunosuppressive therapy. Thirty patients (81%) responded to treatment. Both azathioprine and cyclophosphamide were found to be effective in the treatment of myasthenia gravis. The degree of improvement was more pronounced in patients with thymoma.     

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.     

Late onset forms of myasthenia gravis. Comparison with early-onset myasthenia gravis

INTRODUCTION: The incidence of myasthenia gravis appears to be increasing in elderly but few studies have been devoted to late onset myasthenia gravis. PATIENTS AND METHODS: We retrospectively compared myasthenic patients with an age at onset above or below 35 years which were observed in two departments of Neurology from 1980 to 2002. RESULTS: 81 cases were included, 28 of which were late onset myasthenia gravis. The two populations were similar in terms of sex-ratio, clinical symptoms, course of the disease and therapeutic response. There was a trend for older patients to present more frequently at onset with dysphagia and axial or proximal involvement, and to have extra-ocular symptoms more quickly. Antibodies against acetylcholine receptor and striated muscle were statistically more frequent in elder patients. CONCLUSIONS: A late onset is not a factor of poor prognosis in myasthenia gravis and older patients must be treated in the same way than younger ones.