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1.  Multifocal Central Serous Chorioretinopathy Associated with Steroids in a Patient with Myasthenia Gravis 
We present a case of bilateral multifocal central serous chorioretinopathy in a 40-year-old male who suffered from myasthenia gravis and was receiving oral prednisolone. Due to the severity of the underlying disease, it was not possible to reduce the corticosteroid dose. After initial unsuccessful treatment with an intravitreal injection of ranibizumab, low-fluence photodynamic therapy was performed, followed by gradual tapering of the corticosteroids. Visual acuity improved significantly in both eyes. Different therapeutic approaches are discussed.
PMCID: PMC3725012  PMID: 23898284
Low-fluence photodynamic therapy; Ranibizumab; Anti-vascular endothelial growth factor; Subretinal fibrin; Central serous chorioretinopathy treatment
2.  Treatment of Myasthenia Gravis Based on Its Immunopathogenesis 
The prognosis of myasthenia gravis (MG) has improved dramatically due to advances in critical-care medicine and symptomatic treatments. Its immunopathogenesis is fundamentally a T-cell-dependent autoimmune process resulting from loss of tolerance toward self-antigens in the thymus. Thymectomy is based on this immunological background. For MG patients who are inadequately controlled with sufficient symptomatic treatment or fail to achieve remission after thymectomy, remission is usually achieved through the addition of other immunotherapies. These immunotherapies can be classified into two groups: rapid induction and long-term maintenance. Rapid induction therapy includes intravenous immunoglobulin (IVIg) and plasma exchange (PE). These produce improvement within a few days after initiation, and so are useful for acute exacerbation including myasthenic crisis or in the perioperative period. High-dose prednisone has been more universally preferred for remission induction, but it acts more slowly than IVIg and PE, commonly only after a delay of several weeks. Slow tapering of steroids after a high-dose pulse offers a method of maintaining the state of remission. However, because of significant side effects, other immunosuppressants (ISs) are frequently added as "steroid-sparing agents". The currently available ISs exert their immunosuppressive effects by three mechanisms: 1) blocking the synthesis of DNA and RNA, 2) inhibiting T-cell activation and 3) depleting the B-cell population. In addition, newer drugs including antisense molecule, tumor necrosis factor alpha receptor blocker and complement inhibitors are currently under investigation to confirm their effectiveness. Until now, the treatment of MG has been based primarily on experience rather than gold-standard evidence from randomized controlled trials. It is hoped that well-organized studies and newer experimental trials will lead to improved treatments.
PMCID: PMC3259491  PMID: 22259613
myasthenia gravis; immunosuppressive agents; immunotherapy
3.  Controversies about the treatment of myasthenia gravis. 
Clinicians treating patients with myasthenia gravis must choose cholinergic drugs, corticosteroids, immunosuppressive drugs, thymectomy, or plasmapheresis. Clinicians must decide the sequence or combination of these therapies and when to deem lack of improvement a sign for a different therapeutic approach. Because controlled trials have not been done to evaluate therapies that may require months or years before benefit is evident, controversy abounds.
PMCID: PMC490631  PMID: 7400825
4.  Efficacy of perioperative high-dose prednisolone therapy during thymectomy in myasthenia gravis patients 
This study aimed to investigate the benefits of administering perioperative high-dose prednisolone in conjunction with thymectomy in patients with myasthenia gravis.
We retrospectively reviewed data from patients with Myasthenia Gravis Foundation of America Clinical Class I to IIIB who had undergone an extended thymectomy between 1992 and 2009. Perioperative high-dose prednisolone was administered at starting doses of 10 to 20 mg and escalated up to 100 mg on alternate days. The treatment group comprised 70 patients receiving perioperative high-dose prednisolone, whereas the control group included 61 patients not treated with preoperative steroids. The two groups were compared with respect to baseline clinical characteristics, incidence of postoperative complications, and follow-up disease status.
Prednisolone-treated patients presented with more advanced disease compared to controls (Class IIB or greater, 42 [60.0%] versus 7 [11.3%], respectively; P < 0.001). Mean preoperative%FVC was lower and FEV1.0% was higher in treated patients than in controls (%FVC: 92.4 ± 2.3% versus 99.5 ± 2.4%, respectively; P = 0.037, FEV1.0%: 85.2 ± 1.3% versus 81.4 ± 0.9%, respectively; P = 0.017). The groups were similar in other variables including presence of thymoma, and operative procedure. In the treatment group, disease status was significantly improved only by the induction of high-dose prednisolone before the surgery (P < 0.001), and these patients discontinued anti-cholinesterase therapy more frequently than controls (P < 0.001). Moreover, the treatment group demonstrated markedly lower rates of postoperative crisis (12.2% versus 2.9%, respectively; P = 0.045). The incidence of infection, wound dehiscence, and diabetes mellitus were comparable between groups. Survival analysis demonstrated higher rates of treated patients with improved disease status at three and five years (92% and 96%, respectively) compared to controls (57% and 76%, respectively; P < 0.001). Likewise, significantly greater proportions of treated patients achieved complete stable remission or pharmacologic remission at three, five, and ten years (23%, 42%, and 72%, respectively) compared to controls (10%, 20%, and 44%, respectively; P = 0.002).
Perioperative high-dose prednisolone therapy is a safe, promising strategy for managing patients with myasthenia gravis and may reduce the incidence of postoperative crisis while improving disease status.
PMCID: PMC3878862  PMID: 24321421
Myasthenia gravis; MG crisis; Prednisolone; Thymectomy
5.  Safety of prednisone for ocular myasthenia gravis 
Treatment with chronic corticosteroids has been associated with frequent significant adverse effects. We hypothesized that a long-term, low-dose prednisone regimen for OMG would have a low rate of major side effects.
Consecutive OMG patients from a single institution over a 16-year-period and treated with ≥1 month of daily prednisone were included. Steroid-related complications were defined as the development/worsening of conditions requiring alteration to medical therapy. Serious complications included conditions requiring emergency care, hospitalization, or surgery.
83 patients with follow-up ranging from 1-271 months (median: 58 months) were included. Fifty-eight (70%) patients had follow-up ≥24 months. The maximum prednisone dose ranged from 10-60mg. Tapering to ≤10mg per day required ≤4 months for all but two patients. Median average daily dose following the initial course was 5mg daily (interquartile range: 4-7.5 mg). During the first two years, there were 24.5 complications per 100 person-years. Only one patient had a serious complication within the first two years (2 year cumulative risk: 1%), but this individual was not following the recommended regimen.
Low-dose prednisone for OMG has an acceptable side-effect profile and causes few serious complications (∼1% two year risk). However, patients need monitoring to detect the relatively common, but less serious, complications (∼39% two year risk) in order to adjust medical therapy in a timely fashion.
PMCID: PMC3731151  PMID: 22549563
Ocular Myasthenia Gravis; Prednisone; Steroid complication
6.  Ocular motor dysfunction and ptosis in ocular myasthenia gravis: effects of treatment 
The British Journal of Ophthalmology  2005;89(10):1330-1334.
Aim: The optimal treatment of ocular myasthenia gravis (OMG) remains unknown. The authors evaluated the efficacy of prednisone and pyridostigmine in reducing diplopia, ocular motor dysfunction, and ptosis in patients with OMG.
Methods: Review of records from a clinical database from one neuro-ophthalmology service of patients presenting with OMG between 1990 and 2002, excluding those who developed generalised MG within the first month after diagnosis. Institutional review board approval was obtained for this study. Participants/interventions: Non-randomised, unmasked, therapy was given. 55 patients with diplopia in primary or downward gaze and clinically demonstrable extraocular muscle dysfunction received prednisone. 34 patients who had contraindications to steroids or who refused treatment with prednisone received pyridostigmine only. Over 5 days the daily prednisone dose was increased to 50–60 mg and then gradually reduced to 10 mg, followed by further reduction as tolerated. The pyridostigmine dose was begun at 180 mg daily and increased as tolerated. Main outcome measures: Follow up evaluations, performed at 1, 3–6, 12, and 24 months, detailed the frequency of ptosis and diplopia and the amount of ocular motor deviation in primary and downward gaze.
Results: The prednisone and pyridostigmine groups were similar for age, sex, acetylcholine receptor antibody level, prism cover test results for primary and downward gaze, diplopia in primary and downward gaze, and unilateral ptosis. Bilateral ptosis was present in 32.4% of the pyridostigmine group and 10.9% of the prednisone group (p = 0.02). The prednisone group showed resolution in primary gaze diplopia, downgaze diplopia, unilateral ptosis, and bilateral ptosis in 73.5%, 75.5%, 85.7%, and 98%, respectively at 1 month. The benefit persisted at 3–6, 12, and 24 months except for the bilateral ptosis. The pyridostigmine group showed resolution in primary gaze diplopia, downgaze diplopia, unilateral ptosis, and bilateral ptosis in 6.9%, 17.2%, 50%, and 76.7% of patients after 1 month of treatment. The prism cover results improved (p = 0.003) in the prednisone group only. In the prednisone group, four patients had no response to therapy. Among the 51 prednisone responsive patients, there were 33 recurrences in 26 patients. 12 patients, all prednisone treated, had remissions. Except for three patients who developed diabetes, no patient developed a clinically significant systemic corticosteroid complication.
Conclusion: These results suggest that 50–60 mg daily prednisone followed by lower doses (10 mg or less) has the benefit of resolving ptosis and diplopia that lasts for at least 2 years in approximately 70% of patients.
PMCID: PMC1772854  PMID: 16170126
ocular myasthenia gravis; diplopia; prednisone; pyridostigmine
7.  Current and emerging therapies for the treatment of myasthenia gravis 
Myasthenia gravis (MG) is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.
PMCID: PMC3083988  PMID: 21552317
myasthenia gravis; therapy; immunosuppression; thymectomy; plasmapheresis
8.  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 90minutes 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.

PMCID: PMC1736793  PMID: 10644791
9.  Response of patients with refractory myasthenia gravis to rituximab: a retrospective study 
Introduction: Myasthenia gravis, an autoimmune disorder of neuromuscular transmission, is treated by an array of immunomodulating therapies. A variable response is observed with certain patients being medically refractory. Methods: We report the results of 14 refractory generalized myasthenia gravis patients (6 AChR+; 8 MuSK+) treated with rituximab. Results: Sustained clinical improvement was observed in all patients as well as a reduction of conventional immunotherapies. Prednisone dose decreased a mean of 65.1%, 85.7%, and 93.8% after cycle 1, 2, and 3 of rituximab therapy, respectively. A statistically significant reduction in plasma exchange sessions was seen after cycle 1 with all patients being off of plasma exchange after cycle 3. Acetylcholine receptor antibody titers decreased a mean of 52.1% (p = 0.0046) post-cycle 2. Conclusion: Our results support the hypothesis that rituximab is beneficial and well tolerated in managing refractory myasthenia gravis and nearly doubles published cases. We propose that B-cell-directed therapies may become an attractive option and suggest pursuit of a prospective trial.
PMCID: PMC3187675  PMID: 22010039
B-cell depletion; immunosuppression; myasthenia gravis; rituximab; treatment
10.  A case of myasthenia gravis treated with steroids: some suggestions for the mechanisms underlying their effect. 
Postgraduate Medical Journal  1977;53(620):321-324.
This report deals with a patient with long standing myasthenia gravis treated with a small dose of corticosteroids. The response to treatment was prompt and the post-therapeutic deterioration in function was minimal and cholinergic in nature. It is suggested that the dose of anticholinesterases be reduced concomitantly with the institution of steroid treatment.
PMCID: PMC2496642  PMID: 887530
11.  Cervical thymectomy in the treatment of myasthenia gravis. 
A retrospective review of 22 patients undergoing cervical thymectomy for myasthenia gravis is presented. Their ages ranged from 19 to 67 years. The male and female ratio as well as the severity of illness prior to operation were similar to other reported series. All patients were evaluated from the standpoint of clinical response to thymectomy, and the number and dosage of anti-myasthenic drugs required after operation. There was a statistically significant improvement in clinical status in the immediate postoperative period (P less than 0.05) and a further significant improvement was noted at six months (P less than 0.05). In addition, significant reductions in postoperative drug therapy were noted (P less than 0.05). Complications from cervical thymectomy were minimal and mortality was zero. An attempt was made to correlate histological findings with clinical results. Comparison is made to other series in the literature and the advantages of the cervical technique as the initial surgical approach are described. The data presented support a useful role for cervical thymectomy in the treatment of myasthenia gravis.
PMCID: PMC2493678  PMID: 6486664
12.  Thymectomy for myasthenia gravis 
Thorax  1972;27(5):513-516.
Between 1951 and 1971 thymectomy was performed on 41 patients with myasthenia gravis. They were selected from a total group of 95 myasthenic patients receiving anticholinesterase therapy. The criteria for selecting patients for thymectomy and the assessment of their progress after operation are described. All the patients have been examined by us at intervals and the results of this follow-up are presented. The survey has shown that substantial improvement after thymectomy occurred in 21 patients (group A), seven of whom had complete remissions without medication for periods of up to 12 years after operation. Considerable benefit from the operation was also observed in seven patients (group B); although their response was less spectacular, in that their daily requirement of anticholinesterase drugs was not changed, their functional activities were significantly enhanced. The most favourable results were seen in female patients aged 15 to 40 years, six of whom had one or more successful pregnancies. Thymectomy did not improve the general condition or progress of six patients (group C). There were three deaths within 16 days of operation, and two patients died within one year. Eight patients survived for periods of 3 to 16 years; six of these died from causes other than directly from myasthenia, and two died at home without established cause.
PMCID: PMC470538  PMID: 5083719
13.  Myasthenia gravis: clinical and histological features in relation to thymectomy. 
The clinical and thymic histological features of 23 patients who underwent thymectomy for myasthenia gravis have been examined and compared. Eighty-two per cent of patients with a non-neoplastic gland containing numerous germinal centres improved postoperatively, whereas 83% of patients with a non-neoplastic gland containing no germinal centres deteriorated or died. Glands with only slight involution and containing numerous germinal centres were more commonly seen in young female patients. The evidence relating thymic histological appearances with the postoperative progress of patients with myasthenia gravis is reviewed.
PMCID: PMC492211  PMID: 1255210
14.  The long term experience of thymectomy for myasthenia gravis. 
A retrospective survey of the records of 287 patients with generalised myasthenia gravis treated at New End Hospital and later at the Royal Free Hospital by anticholinesterase drugs, with or without thymectomy, between the years 1942 and 1976, shows that 62% of patients were improved. The timing of the operation, the grading of disease and the age and sex of the patient did not greatly influence overall results. The poor diagnosis of thymic tumours was confirmed in this series. A decrease occurred in the number of patients achieving complete remission after 1961, although the proportion of patients improving did not fall. It is possible that anticholinesterase therapy may alter the response to thymectomy.
PMCID: PMC1028325  PMID: 3998750
15.  Myasthenia Gravis: A Review of Available Treatment Approaches 
Autoimmune Diseases  2011;2011:847393.
Patients with autoimmune myasthenia gravis (MG) should be further classified before initiating therapy, as treatment response varies for ocular versus generalised, early onset versus late onset, and acetylcholine receptor antibody positive versus MuSK antibody positive disease. Most patients need immunosuppression in addition to symptomatic therapy. Prednisolone and azathioprine represent first choice drugs, whereas several second choice options are recommended and should be considered. Thymectomy should be undertaken in MG with thymoma and in generalised, early-onset MG. For MG crises and other acute exacerbations, intravenous immunoglobulin (IvIg) and plasma exchange are equally effective and safe treatments. Children and females in child bearing age need special attention regarding potential side effects of immunosuppressive therapy. MG pathogenesis is known in detail, but the immune therapy is still surprisingly unspecific, without a pin-pointed attack on the defined disease-inducing antigen-antibody reaction being available.
PMCID: PMC3189457  PMID: 22007295
16.  The relationship of plasma levels of pyridostigmine to clinical effect in patients with myasthenia gravis 
The relationship between plasma levels of pyridostigmine to clinical evaluation of muscle power was examined in nine patients with myasthenia gravis during treatment with pyridostigmine in doses of 60 to 1040 mg daily. Five of the nine subjects demonstrated a trend towards a positive correlation, but in only two of them was this statistically significant at p < 0.05. In addition, the presence or absence of a possible correlation between muscle power and plasma concentration was not related to the duration of the disease, additional prednisolone therapy or thymectomy.
PMCID: PMC491235  PMID: 7334410
17.  Myasthenia Gravis: Comparative Evaluation of Medical and Surgical Treatment 
Sixty-eight patients with myasthenia gravis were evaluated and compared to determine the results of medical and surgical treatment; eight patients with thymoma were evaluated separately. In the group of 30 non-thymoma patients treated medically 50% of patients derived moderate to good improvement over a mean follow-up period of 11 years. Ten per cent of patients in this group died from myasthenia.
In the group of 30 non-thymoma patients treated by thymectomy, 83% achieved good to excellent improvement. There was no surgical or myasthenic mortality over a mean follow-up period of nine years.
The results of treatment in the eight thymoma patients were decidedly inferior and there was no significant difference between the medically and surgically treated patients. Fifty per cent showed only moderate improvement during a mean follow-up of five years and 50%, after initial improvement, deteriorated later and died from myasthenia between three and four years after thymectomy.
Two additional patients had thymoma without myasthenia. Neither of them had developed myasthenia, two years following thymectomy in one case and after 25 years in the other, despite recurrence of the tumour with extensive invasiveness in the very long-standing case.
PMCID: PMC1946533  PMID: 5348881
18.  Extracorporeal Immunoglobulin Elimination for the Treatment of Severe Myasthenia Gravis 
Myasthenia gravis (MG) is a neuromuscular disorder leading to fluctuating muscle weakness and fatigue. Rarely, long-term stabilization is not possible through the use of thymectomy or any known drug therapy. We present our experience with extracorporeal immunoglobulin (Ig) elimination by immunoadsorption (adsorbers with human Ig antibodies). Acetylcholine receptor antibodies (AChRAs) were measured during long-term monitoring (4.7 ± 2.9 years; range 1.1–8.0). A total of 474 samples (232 pairs) were analyzed, and a drop in AChRA levels was observed (P = .025). The clinical status of patients improved and stabilized. Roughly 6.8% of patients experienced clinically irrelevant side effects. The method of Ig elimination by extracorporeal immunoadsorption (IA) is a clinical application of the recent biotechnological advances. It offers an effective and safe therapy for severe MG even when the disease is resistant to standard therapy.
PMCID: PMC2840412  PMID: 20300435
19.  Effects of alternate-day prednisone therapy on respiratory function in myasthenia gravis. 
Thorax  1976;31(4):410-413.
To determine the effects of alternate-day prednisone therapy on respiratory function in myasthenia gravis, eight patients were evaluated during "days on" and "days off" prednisone. The patients were treated with long-term (up to three years), high-single-dose, alternate-day oral prednisone. After patients had been controlled with alternate-day prednisone they had no episodes of acute respiratory insufficiency or myasthenic crises. Although a small reduction in respiratory function during the day off prednisone was seen in some patients, the change was not statistically significant for the group and was probably physiologically unimportant in most patients. The data indicate that in myasthenic patients who have no underlying lung disease respiratory function is not significantly compromised by administering the prednisone on alternate days.
PMCID: PMC470450  PMID: 968798
20.  Reversible oropharyngeal dysphagia secondary to cricopharyngeal sphincter achalasia in a patient with myasthenia gravis: a case report 
Cases Journal  2009;2:6565.
Bulbar weakness and fatigue resulting in dysphagia and dysarthria is common in myasthenia gravis. In chronic MG it is often assumed that these symptoms herald an exacerbation of the patient's disease and doses of cholinergic agents and immunomodulatory therapies may be increased, along with initiation of plasma exchange. A case is presented in which dysphagia was refractory to standard MG therapy, leading to the subsequent discovery of cricopharyngeal sphincter achalasia as the primary cause of the patient's symptoms rather than an assumed myasthenia gravis exacerbation. The patient's dysphagia resolved after esophageal dilatation. Cricopharyngeal sphincter achalasia is a common disorder producing dysphagia in the elderly and needs to be considered in the evaluation of a myasthenic patient with worsening dysphagia when standard myasthenia gravis therapy fails. Discussion of myasthenia gravis, cholinergic therapy and cricopharyngeal sphincter achalasia is undertaken. Clinicians are encouraged to consider non-neurologic causes of worsening dysphagia in the myasthenic patient.
PMCID: PMC2827116  PMID: 20184678
21.  Phase II trial of methotrexate in myasthenia gravis 
Prednisone is a frequently used treatment for myasthenia gravis (MG) but it has numerous side effects. Methotrexate is a selective inhibitor of dihydrofolate reductase and lymphocyte proliferation and is an effective immuosuppressive medication for autoimmune diseases. Given the negative results of the mycophenolate mofetil study, search for an effective immunosuppressant drug therapy is ongoing. The objective is to determine if oral methotrexate is safe and effective for MG patients who take prednisone. We have initiated a randomized, double-blind, placebo-controlled multicenter trial of methotrexate versus placebo in patients taking at least 10 mg/day of prednisone at enrollment. The methotrexate dose is increased to 20 mg and the prednisone dose is adjusted per protocol during the study. Clinical and laboratory evaluations are performed monthly for 12 months, with the primary efficacy measure being the nine-month prednisone area under the curve (AUC) from months 3 to 12. Secondary outcome measures include MG outcomes, quality of life measures, and a polyglutamation biomarker assay. A total of 18 U.S. sites and 2 Canadian sites are participating, with 48 screened cases, 42 enrolled, with 19 still active in the study.
PMCID: PMC3564221  PMID: 23278574
methotrexate; myasthenia gravis; area under the curve; prednisone
22.  MYASTHENIA GRAVIS—Problems in Diagnosis 
California Medicine  1957;87(6):373-379.
The possibility of myasthenia gravis must be considered in patients persistently complaining of weakness and fatigue. There may be many difficulties and pitfalls in differentiating myasthenia gravis from other disorders in which muscular weakness is a common complaint.
Observation of a group of 36 patients with myasthenia gravis, and another group of 30 cases involving the differential diagnosis of myasthenia gravis, led to a conclusion that a physician should apply criteria carefully before arriving at a diagnosis of myasthenia gravis and instituting drug therapy, since nonmyasthenics may frequently respond with subjective improvement temporarily following administration of cholinergic drugs.
Myasthenia gravis may be a more common disorder than was suspected in the past.
PMCID: PMC1512196  PMID: 13489495
23.  Immunoadsorption therapy for myasthenia gravis. 
The results of a multicentre trial were analysed to evaluate the efficacy of immunoadsorption therapy for severe generalised myasthenia gravis. Twenty patients with myasthenia gravis who were concurrently receiving high dose prednisolone and azathioprine therapy were treated with an affinity-type adsorbent, using tryptophan-linked polyvinyl alcohol gel (IM-TR), according to a standardised treatment protocol. The 20 patients received five adsorption treatments within a period of 10 days. In 11, pronounced improvement of myasthenic weakness was seen and long-term remission was maintained. The treatment was especially effective in patients with thymic hyperplasia. Circulating acetylcholine receptor (AChR) antibodies were reduced by about 60% by treating one plasma volume. There was no difference in the rate of removal of the AChR antibodies between patients with thymic hyperplasia and patients with thymoma. No serious complications occurred during 100 procedures. It was concluded that the immunoadsorption therapy with IM-TR is useful in controlling symptoms in patients with severe myasthenia gravis who are otherwise unresponsive.
PMCID: PMC1072918  PMID: 8201327
24.  Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis: a case report 
Although myasthenia gravis is frequently associated with other disorders, it has not been reported together with primary sclerosing cholangitis, complicating the administration of liver-toxic immunosuppressive therapy.
Case presentation
A 73-year-old Caucasian woman with a history of arterial hypertension, thyroid dysfunction, glaucoma, right-sided ptosis and later generalized weakness, was diagnosed with myasthenia gravis. Additionally, primary sclerosing cholangitis was detected, initially prohibiting the administration of immunosuppressants. Despite treatment with steroids and pyridostigmine she repeatedly experienced myasthenic crises. After the fifth crisis and after antibody titers had reached levels > 100 nmol/L during two years of follow-up, it was decided to restart azathioprine. Interestingly, low-dose azathioprine (1.5 mg/kg/day) was well tolerated, had a positive clinical and immunological effect and did not worsen primary sclerosing cholangitis.
Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient. Mild immunosuppression with azathioprine is feasible and effective in such a patient, without worsening myasthenia gravis or primary sclerosing cholangitis.
PMCID: PMC2988809  PMID: 21059205
25.  Acute myopathy associated with large parenteral dose of corticosteroid in myasthenia gravis. 
A 13 year old Greek girl with myasthenia gravis developed widespread muscle paralysis and atrophy after large parenteral doses of corticosteroids (5.48 g methylprednisolone). An electromyogram showed myopathy, creatine kinase concentration below normal, and a muscle biopsy showed severe myopathy with selective loss of the thick filaments (myosin). Previous reports of myopathy associated with large steroid doses have mostly been in patients who were also receiving non-depolarising neuromuscular blocking drugs. This patient is unique in that severe myopathy was associated with neuromuscular blockade caused by antibodies to acetylcholine receptors. The findings in this case suggest that high doses of parenteral corticosteroids in patients with myasthenia gravis may be dangerous and that blocking the neuromuscular junction with drugs or antibodies predisposes skeletal muscles to the injurious effects of corticosteroids.
PMCID: PMC489625  PMID: 8509789

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