Myasthenia Crisis Research Articles

Correlation factors of 127 times pre-crisis state in patients with myasthenia gravis

Objective: To investigate the clinical features of the Pre-Crisis State and analyze the correlated risk factors of Pre-Crisis State of myasthenia crisis. Methods: We included 93 patients with myasthenia gravis (MG) who experienced 127 times Pre-Crisis State between October 2007 and July 2016. Those patients were hospitalized in the MG specialize center, Department of Neurological Science, first Affiliated Hospital of Sun Yat-sen University. The information of the general situation, the clinical manifestations and the blood gas analysis in those patients were collected using our innovated clinical research form. Statistic methods were applied including descriptive analysis, univariate logistic analysis, multivariate correlation logistic analysis, etc. Results: (1)The typical features of MG Pre-Crisis State included: dyspnea (127 times, 100% not requiring intubation or non-invasive ventilation), bulbar-muscle weakness (121 times, 95.28%), the increased blood partial pressure of carbon dioxide (PCO(2)) (94 times, 85.45%), expectoration weakness (99 times, 77.95%), sleep disorders (107 times, 84.25%) and the infection (99 times, 77.95%). The occurrence of dyspnea in combination with bulbar-muscle weakness (P=0.002) or the increased blood PCO(2) (P=0.042) often indicated the tendency of crisis. (2) The MG symptoms which were proportion to the occurrence of crisis includes: bulbar-muscle weakness (P=0.028), fever (P=0.028), malnutrition (P=0.066), complications (P=0.071), excess oropharyngeal secretions (P=0.005) and the increased blood PCO(2) (P=0.007). The perioperative period of thymectomy would not increase the risk of crisis. Conclusions: Dyspnea indicates the occurrence of the Pre-Crisis State of MG. In order to significantly reduce the morbidity of myasthenia crisis, the bulbar-muscle weakness, the increased blood PCO(2), expectoration weakness, sleep disorders, infection & fever and excess oropharyngeal secretions should be treated timely.

Myasthenia Gravis and Crisis: Evaluation and Management in the Emergency Department

BackgroundMyasthenia gravis (MG) is an uncommon autoimmune disorder affecting the neuromuscular junction and manifesting as muscle weakness. A multitude of stressors can exacerbate MG. When symptoms are exacerbated, muscle weakness can be severe enough to result in respiratory failure, a condition known as myasthenic crisis (MC). ObjectiveThis review discusses risk factors, diagnosis, management, and iatrogenic avoidance of MC. DiscussionMC can affect any age, ethnicity, or sex and can be precipitated with any stressor, infection being the most common. MC is a clinical diagnosis defined by respiratory failure caused by exacerbation of MG. Muscle weakness can involve any voluntary muscle. MC can be differentiated from other neuromuscular junction diseases by the presence of normal reflexes, normal sensation, lack of autonomic symptoms, lack of fasciculations, and worsening weakness with repetitive motion. Treatment should target the inciting event and airway support. All acetylcholinesterase inhibitors should be avoided in crisis, including edrophonium testing and corticosteroids initially. Respiratory support can begin with noninvasive positive-pressure ventilation, as this has been successful even in patients with bulbar weakness. If intubation is necessary, consider avoiding paralytics or use a reduced dose of nondepolarizing agents. ConclusionsMC should be in the differential of any patient with muscular weakness and respiratory compromise. Emergency department management of MC should focus on ruling out infection and respiratory support. Strong consideration should be given to beginning with noninvasive positive-pressure ventilation for ventilatory support. Corticosteroids, depolarizing paralytics, and acetylcholinesterase inhibitors should be avoided in patients with MC in the emergency department.

Challenges in Diagnosis and Management of Myasthenic Crisis in Resource Limited Health Care Setting

A 23 years old lady with background history of breathing and swallowing difficulty for six months presented with respiratory distress to a district hospital without an in-house neurology service. Her diagnosis remained a challenge as patient presented in severe clinical state without a proper medical history available. She was subjected to bedside ice pack testing and subsequently managed along the diagnosis of myasthenia crisis. To our delight and surprise, she responded to the treatment and survived the critical period. The main purpose of this study is to emphasize the importance of bedside ice pack test in diagnosis of myasthenia gravis in resource limited health setting.

Unusual Presentation of Myasthenia Crisis Following Thymectomy and Thyroidectomy.

Respiratory distress after thyroidectomy and thymectomy can be challenging. We encountered a 70-year-old lady with a long-standing goiter with concomitant thymoma. She underwent the surgery and developed respiratory distress upon extubation with loss of laryngeal sensation causing severe aspiration. She was diagnosed myasthenia gravis and symptoms resolved with MG treatments.

Low serum albumin concentrations are associated with disease severity in patients with myasthenia gravis.

Serum albumin (S-Alb) is a widely used biomarker of nutritional status and disease severity in patients with autoimmune diseases. We investigated the correlation between S-Alb and the severity of myasthenia gravis (MG).A total number of 166 subjects were recruited in the study. Subjects were divided into 3 groups (T1 to T3) by S-Alb levels: T1: 21.1 to 38.4 g/L, T2: 38.5 to 41.5 g/L, T3: 41.6 to 48.9 g/L. Regression analysis was performed to determine the correlation of initial albumin concentrations and the severity of disease of MG.Lower levels of S-Alb were observed in subjects with increased disease severity than those with slight disease severity, meanwhile, incidence of myasthenia crisis increased in the lower albumin tertiles (P < 0.001). The disease severity assessment was performed according to the criteria established by the Myasthenia Gravis Foundation of America. After adjusting for age, sex, body mass index (BMI), and duration of disease, it showed that higher S-Alb concentrations were associated with lower disease severity. Odds ratios (ORs) of T2 to T3 were 0.241 (95% CI: 0.103–0.566, P < 0.001), 0.140 (95% CI: 0.054–0.367, P < 0.001) when compared with subjects in the T1, respectively. When subjects were stratified into hypoalbuminemia and normal albumin groups, we found that the association between S-Alb and MG remained significant in the hypoalbuminemia group only (OR: 0.693, 95% CI: 0.550–0.874, P = 0.002) after further adjustment for age, sex, BMI, and duration of disease.This is the first study to demonstrate that S-Alb was independently associated with MG severity. In patients with low S-Alb, S-Alb concentration could be a potential biomarker for MG disability.

Value of the albumin to globulin ratio in predicting severity and prognosis in myasthenia gravis patients

To assess the predictive value of the albumin to globulin ratio (AGR) in evaluation of disease severity and prognosis in myasthenia gravis patients. A total of 135 myasthenia gravis (MG) patients were enrolled between February 2009 and March 2015. The AGR was detected on the first day of hospitalization and ranked from lowest to highest, and the patients were divided into three equal tertiles according to the AGR values, which were T1 (AGR <1.34), T2 (1.34≤AGR≤1.53) and T3 (AGR>1.53). The Kaplan-Meier curve was used to evaluate the prognostic value of AGR. Cox model analysis was used to evaluate the relevant factors. Multivariate Logistic regression analysis was used to find the predictors of myasthenia crisis during hospitalization. The median length of hospital stay for each tertile was: for the T1 21 days (15-35.5), T2 18 days (14-27.5), and T3 16 days (12-22.5) (P<0.01), and Kaplan-Meier curves showed significant difference among the three groups. In the univariate model, serum albumin, creatinine, AGR and MGFA clinical classification were related to prognosis of myasthenia gravis. At the multivariate Cox regression analysis, the AGR (P<0.001) and MGFA clinical classification (P<0.001) were independent predictive factors of disease severity and prognosis in myasthenia gravis patients. Respectively, the hazard ratio (HR) were 4.655 (95% CI: 2.355-9.202) and 0.596 (95% CI: 0.492-0.723). Multivariate Logistic regression analysis showed the AGR (P<0.001) and MGFA clinical classification were related to myasthenia crisis. The AGR may represent a simple, potentially useful predictive biomarker for evaluating the disease severity and prognosis of patients with myasthenia gravis.

The relationship between myasthenia gravis and the different pathological type of thymoma patients' operation and prognosis

To evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients. The clinical data from 228 consecutive patients (median sternotomy were used in 153, video-assisted thoracoscopic themectomy were used in 75) operated on from January 1992 to December 2007 was analyzed retrospectively. These thymoma patients had been subdivided into two groups: thymoma with MG (n = 125) and thymoma without MG (n = 103). All thymic epithelial tumors were classified according to the WHO histologic classification and the Masaoka clinical staging system. The result was evaluated according to the Myasthenia Gravis Foundation of America's criterion. The clinical features of the 2 test was compared between the two groups by χ² test, and the survival were compared between the two groups by Cox analysis. There were no peri-operative deaths. 19 cases were inoperable (6 in the group with MG, 13 without MG (χ² = 4.52, P = 0.035)). The proportions of type A and thymic carcinoma were 0 in the group with MG, 10.5% (11/103) and 11.6% (12/103) respectively in the group without MG. According to the Masaoka's clinical staging, in the group MG, 24.8% (31/125) patients were stage III and IV; in the group without MG, 33.0% (34/103) patients were stage III and IV. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% (36/125) patients with MG and only 5.8% (6/103) in patients without MG (χ² = 20.91, P = 0.000) Microthymoma was identified in the paraneoplastic thymus of 3 patients with MG. There were 198 patients followed up, the rate was 86.8% (198/228). There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 thymoma and thymic carcinoma recurred. The actuarial 5- and 10-year survival rates were 89.3% and 81.2% for patients with MG respectively, and 90.0% and 78.9% for patients without MG respectively. Within 5 years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 out of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and thymic carcinoma. The existence of myasthenia gravis has little influence on the prognosis of thymomas, but it is good for early diagnosis and treatment. Extended thymectomy should be performed to all patients with thymoma, no matter they have myasthenia gravis or not. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and (or) thymic carcinoma for patients without MG.

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis.

This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4+, CD8+, or CD3+ was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae.

Analysis of surgical strategy of treating thymomas: A single center experience.

e18519 Background: Thymomas are epithelial thymic tumors, generally considered to have an indolent growth pattern but are malignant. 20-40% of patients with thymomas have paraneoplastic syndromes,including myasthenia gravis(MG). The objective of this study is to evaluate outcome of surgical treatment to thymoma and determine whether the different pathological and clinical characteristics of thymomas influences the prognosis in thymoma patients. Methods: We retrospectively studied data from 228 consecutive patients operated on from1992 to 2007. All thymic epithelial tumors were reclassified according to the WHO histologic classification, and the Masaoka clinical staging system. Patients with type A and stage I thymoma had no adjuvant therapy. Most patients with types AB and B1 thymomas had radiotherapy postoperatively. Patients (stageII, III) were treated with adjuvant mediastinal radiation therapy, and both adjuvant radiation therapy and chemotherapy was administered to stage IV and type C one month after operation. For patients with tumor nodules found on the pleural surface, cytoreductive surgery was performed. After that, patients had both adjuvant radiation therapy and chemotherapy. Results: There were no peri-operative deaths. 19 cases were inoperable. 16.2% patients had postoperative complications: The proportions of type A, AB, B1, B2, B3, and C thymoma in this data were 0%, 22.4%, 26.4%, 30.4%, 20.8%, and 0% respectively in patients with MG. Microthymoma was identified in the paraneoplastic thymus of 3 patients. There were 198 patients followed up. There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 and C recurred. The actuarial 5- and 10-year survival rates were 89.6% and 80.5% respectively. Within 5 years postoperatively, 6 of 16 deaths died of myasthenia crisis, 6 were attributable to inoperable tumors (stageIV) and type C thymoma. Conclusions: Surgical treatment to thymoma has a sound prognosis. Multimodality treatment of stage III or IV thymomas may be critical to improve the 5-year survival rate. MG seldom occurs in type A and C thymoma. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and /or type C for thymoma patients without MG.

Pegylated Interferon Induced Myasthenia Crisis—A Case Report

Interferons (IFNs) have antiviral, antimitogenic, and immunostimulatory effects and are often used in the treatment of viral hepatitis and some neoplasms. Combination pegylated IFN-alpha and ribavirin therapy is currently recommended for the treatment of hepatitis C. Triple therapy, with the addition of a protease inhibitor, such as telaprevir or boceprevir, has recently become a mainstay of therapy for certain genotypes. There have also been reports outlining side effects associated with conventional IFN therapy and its immunostimulatory effects, which may cause autoimmune phenomena, including but not limited to Guillain-Barre syndrome, polymyositis, acute and chronic demyelinating polyneuropathy, and myasthenia gravis. Although a number of cases of interferon-induced myasthenia gravis have been reported, we present a case of interferon-induced myasthenia crisis that developed soon after retreatment of hepatitis C with combination interferon, ribavirin, and telaprevir.

Myasthenia crisis following percutaneous coronary intervention in a patient with late onset myasthenia gravis: successful treatment of a unique case

A 70-year-old female, known case of late onset myasthenia gravis for last 1year was admitted with effort angina. Coronary angiogram revealed presence of significant lesion in the proximal part of right coronary artery. She had undergone percutaneous coronary intervention. Following angioplasty she developed recurrent ventricular tachycardia and respiratory distress. She developed myasthenia crisis and was put on ventilator. She had undergone through plasmapheresis thrice as anti-acetylcholine receptor antibody titres were very high. Medline search revealed no previous report of similar case. We are reporting the first case where myasthenia crisis was triggered by percutaneous coronary intervention and was treated successfully.

Evaluation of the New Classification and Surgical Strategy for Myasthenia Gravis

The aim of this study was to discuss the new methods of clinical classification and staging of patients with myasthenia gravis (MG) proposed by our group and to summarize the experiences of surgical treatment of MG with a novel incision by cutting the sternum cross-sectionally at the second intercostal level. A retrospective analysis was made for the clinical data from the patients with MG who underwent thymectomy from July 1988 to May 2009. The surgical procedures were designed into three groups, a group with Osserman classification and median incision of the sternum (Group 1), a group with MGFA typing (Myasthenia Gravis Foundation of America) and a small transverse sternal incision at the second intercostal level (Group 2), and a group with new typing and a smaller transverse sternal incision at the second intercostal level (Group 3). Observation of the clinical typing and staging was made in the patients with myasthenia crisis. The parameters such as procedure duration in Group 2 and 3 was significantly lower than those in Group 1 (P < 0.05). The incidence of myasthenia crisis in Group 3 was significantly lower than that in Groups 2 and 3 (P < 0.05). The procedure with a smaller transverse sternal incision at the second intercostal level (Group 3) is a safer method for patients with MG. The combination of this procedure with the new typing and staging methods proposed by our group could facilitate the selection of operation indications and opportunity, resulting in the lower incidence of myasthenia crisis and mortality. Our new procedure is well deserved to be a preferential selection by other hospitals.

Different Characteristics of Thymomas With and Without Myasthenia Gravis

The objective of this study was to evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients. We retrospectively studied data from 228 consecutive patients operated on from 1992 to 2007 in Beijing Tongren Hospital. These thymoma patients have been subdivided into two groups: group A with MG (n=125) and group B without MG (n=103). There were no perioperative deaths. There were 19 inoperable cases (6 in the group with MG, 13 without MG, P=0.035). The proportions of types A, AB, B1, B2, B3, and C thymoma in this data were 0, 22.4, 26.4, 30.4, 20.8, and 0%, respectively, in the group with MG, and 10.5, 13.4, 23.6, 24.5, 16.4, and 11.6%, respectively in the group without MG. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% patients with MG and only 5.8% in patients without MG. Microthymoma was identified in the paraneoplastic thymus of three patients with MG. There were 198 patients followed up. There was no recurrence in patients with type A, and a few patients with types AB, B1, B2, B3, and C recurred. The actuarial 5- and 10-year survival rates were 89.3 and 81.2% for patients with MG, respectively, and 90 and 78.9% for patients without MG, respectively. Within 5years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and type C thymoma. MG seldom occurs in types A and C thymoma. MG of some thymoma patients was not caused by thymoma, but by the paraneoplastic thymus. The prognosis of thymomas with MG is similar to that without MG. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and/or type C for thymoma patients without MG.

Reasons of life-support machine relevant pneumonia in myasthenia crisis patients and its nursing intervention

Objective To enhance the understanding of myasthenia crisis in patients with ventilatorassociated pneumonia, and provide meticulous care, active prevention and treatment.Methods 20 cases of patients with myasthenia gravis crisis were collected in past year in our hospital, and ventilator-associated pneumonia occurrence was analyzed. Early and actively targeted nursing interventions were implemented.Results Patient stability, security ex-tubation were achieved during the implementation of careful, thoughtful, targeted and timely care.Conclusions Ventilator-associated pneumonia in patients with myasthenia gravis crisis occurred for various reasons.At the same time ventilator-associated pneumonia will further decrease the patient's condition and increase the treatment difficulty.Therefore, it was significant to strengthen understanding, basic and targeted care as soon as possible to reduce its incidence. Key words: Myasthenia crisis; Life-support machine relevant pneumonia; Nursing

Myasthenia Gravis Development and Crisis Subsequent to Multiple Sclerosis

During the last decade, sporadic combination of multiple sclerosis (MS) and myasthenia gravis (MG) has been reported repeatedly. Although these are anecdotal, they are important enough to raise concerns about co-occurrence of MG and MS. Here, we present a case of an MS patient who developed an MG crisis. She had received interferon for relapsing remitting MS. Interestingly, she developed an MG crisis 4 years after the diagnosis of MS. MS and MG have relatively the same distribution for age, corresponding to the younger peak of the bimodal age distribution in MG. They also share some HLA typing characteristics. Furthermore, some evidences support the role of systemic immune dysregulation due to a genetic susceptibility that is common to these two diseases. The association may be underdiagnosed because of the possible overlap of symptoms especially bulbar manifestations in which either MG or MS can mimic each other, leading to underestimating incidence of the combination. The evidence warrants physicians, especially neurologists, to always consider the possibility of the other disease when encountering any patients either with MS or MG. Anecdotal and sporadic reports of combination of multiple sclerosis (MS) and myasthenia gravis (MG) have been raised concerns about co-occurrence of them.

Analysis of prognostic factors and efficacy of plasmapheresis in the treatment of myasthenia crisis

To examine the prognostic factors and efficacy of myasthenia gravis (MG) in crisis on plasmapheresis and detect the reasons for ineffective plasmapheresis. The investigators analyzed a total of 69 MG patients in crisis on plasmapheresis by case control study. Gender, age at onset of myasthenic symptoms, duration between the onset of crisis and plasmapheresis, pre-therapeutic use of glucocorticoids, pulmonary infections, other complications, nutritional status, history of thymectomy in 48 hours before crisis, thymic pathology, combined intravenous immunoglobulin (IVIG) and total sessions of plasmapheresis were measured retrospectively. Univariate analysis showed that pulmonary infections (P = 0.000, OR = 29.250), history of thymectomy in 48 hours before crisis (P = 0.046, OR = 0.267), combined intravenous immunoglobulin (P = 0.003, OR = 0.136) and total sessions of plasmapheresis (P = 0.022, OR = 0.498) were all influencing factors of plasmapheresis. However the analysis of multivariate logistic regression revealed that pulmonary infections (P = 0.000, OR = 23.600) was an independent risk factor and combined intravenous immunoglobulin (P = 0.047, OR = 0.192) was an independent protection factor of plasmapheresis. Plasmapheresis is ineffective in MG crisis with pulmonary infections. Control of pulmonary infections and combined intravenous immunoglobulin can improve the response to plasmapheresis in patients with MG crisis.

The pattern and presentation of myathenia gravis in Al-Shaab and Omdurman Teaching Hospitals

Introduction: Myasthenia gravis (MG) is the most common acquired autoimmune disorder of neuromuscular transmission. It has different patterns of presentation.Objective: to study the pattern and mode of presentation of Myasthenia Gravis in Al-Shaab and Omdurman Teaching Hospitals, Khartoum Sudan.Methods: The demographic and clinical characteristics of 50 patients of myasthenia gravis (MG) were reviewed in Alshab and Oumdrman Teaching Hospitals for five months period from May to October 2008.Results: Out of 2400 patients attending neurology clinics 50 were found to have Myathenia Gravis [MG] with female; male ratio of 2.5:1. The age at presentation was the second decade of life in 34%. Most of the patients [42%] were from Khartoum State followed by North Kordofan State [14%]. High occurrence of late onset fatigability and weakness was seen in 92 % of our patients. Ocular muscles involvement was detected in 78%. MG was aggravated by hot weather and fever in 12% of the patients. Quinine represented the most common drug which aggravated the myasthenia symptoms in 4%. Diabetes mellitus and thyroid diseases were the most associated auto immune diseases seen in 12% and 4 % respectively. The diagnosis was made by classic history and neurological examinations in 88% and positive neostigmin test in64%. The majority of patients were treated with steroids. Thymectomy was done in 10% patients. The outcome was excellent as 82% improved and only one [2%] patient died.Discussion: Going with literature diagnosis of MG was made depending on classical history, full neurological examination and confirmed by tensilon test. Because of different reasons more sophisticated tests were not done in our patients. Only patients with malaria who were treated withquinine showed deterioration of their symptoms in our study. Strikingly, myasthenia crisis which were reported in 27% of patients were not seen in our population. Because of lack of usage of steroid sparing agents the vast majority [96%] of our patients were treated with steroids.Conclusion: Generalized myasthenia gravis with ocular involvement is common in our patients. However, pure OMG was not seen. Thymectomy was done in a small number of our patients with reference toother studies.Keywords: autoimmune, tensilon, neostigmin, thymectomy, diplopia, ptosis.

The Role of Non-invasive Ventilation and Factors Predicting Extubation Outcome in Myasthenic Crisis

Myasthenic crisis is a great threat to patients with myasthenia gravis. Usage of non-invasive ventilation (NIV) to prevent intubation and timing of extubating of patients in myasthenic crisis are important issues though not well documented. To explore the factors predicting NIV success and extubation outcome in myasthenic crisis, we reviewed the records of 41 episodes of myasthenia crisis. NIV was applied to 14 episodes of myasthenic crisis and eight (57.1%) of them were successfully prevented from intubation. An Acute Physiology and Chronic Health Evaluation (APACHE) II score of <6 and a serum bicarbonate level of <30 mmol/l were independent predictors of NIV success. For patients undergoing invasive mechanical ventilation, extubation failure was observed in 13 (39.4%) of 33 episodes, and the most common cause was sputum impaction due to a poor cough strength (61.5%). A maximal expiratory pressure (Pemax) of > or = 40 cmH(2)O was a good predictor of extubation success. Extubation failure led to poorer outcomes. NIV may be applied to those patients with a low APACHE II score and a lesser degree of metabolic compensation for respiratory acidosis. For patients undergoing invasive mechanical ventilation, extubation failure is associated with significant in-hospital morbidity in myasthenic crisis. Adequate levels of Pemax and cough strength correlate significantly with extubation success.

Apport des échanges plasmatiques dans le traitement de la myasthénie : étude de 11 cas

The use of plasma exchange (PE) constituted an advance in the treatment of myasthenia. The objective of our study was to determine the relevance of PE in the treatment of myasthenia and to study the different complications which can be observed during PE. We studied retrospectively 11 patients who have generalized myasthenia and underwent PE. We used an intermittent flow cell separator and we performed PE three times a week. Biological assessment was performed before and after PE for all patients. The exchange volume was calculated according to the patient weight, gender and the value of hematocrit. Our series included six women and five men. The mean age at onset of the disease was 41.4+/-14.1 years (range: 18 to 68). Indication of PE was myasthenia crisis (eight cases), resistance to classic treatment (two cases) and exacerbation after thymectomy (one case). An improvement was observed rapidly in five cases and delayed in three cases. The remaining three patients did not improve. The most frequent side effects of PE were hypotension (four cases), heart arrhythmia (two cases) and hypoglycemia (one case). Three patients dead in the seven days after the first PE. PE represents an interesting tool to treat severe forms of myasthenia and improve prognosis. High incidence of complications in our series can be explained by the initial disease severity, the used method of PE, the existence of associated illness, and a long stay in intensive care unit.

MYASTHENIA GRAVIS IN IRANIAN CHILDREN

Objective This study was undertaken to evaluate the clinical spectrum of myasthenia gravis in children and determine factors that help the clinician in his/her diagnosis and management. Materials & Methods A retrospective review was performed on all pediatric patients suffering from myasthenia gravis (M.G) admitted in the department of pediatric neurology of the Mofid Hospital of the Shaheed Beheshti University, between 1994 and 2002. Results Of the thirty-two children with M.G. enrolled in our study, seven were suffering from the congenital type while the remaining (25 cases) had the juvenile M.G. Initial symptoms of congenital M.G were ptosis (7/7), limitation of eye movement (2/7) and mild generalized weakness (6/7). Although the Tensilon test was positive in 85% of congenital M.G cases, no myasthenia crisis or spontaneous remission was observed in any of them. In children with juvenile M.G, the age of presentation was 1.2 to 12.5 years, mean age 5.7+4.2 years (15 girls and 10 boys). The most common presenting symptoms in juvenile group were ptosis in 96% and generalized weakness in 76%. Eight of them (32%) had had at least one myasthenia crisis. EMG was diagnostic in 83% and one case the tensilon test was positive in 84%. One patient had hyperthyroidism and had already been diagnosed with hypothyroidism; two of them were epileptics. Eight patients underwent thymectomy microscopically; in specimens examined, five (62%) showed thymic follicular hyperplasia while in remaining three results were normal. One patient (12.5%) recovered completely after thymectomy with no need for medication during the follow up. Four patients (50%) showed relative improvement and in three cases (37%) improvement was negligible. Conclusion The results showed a female to male ratio of 1.5/1 which was correlated to adult M.G. The most common presenting symptoms consisted of ophtalmoplegia, with bilateral ptosis being the most significant. Although this study revealed that thymectomy lacks any remarkable prognostic influence, all patients had thymectomy after two years of disease onset. Some reports have indicated positive results if surgery was performed within two years of onset of disease. Key words: Myasthenia gravis, Children, Thymectomy, Congenital