SUBMITTED BY: Agresor, Suzette B. Bautista Louis Ann Bolando, Jay ar



Far Eastern University

Institute of nursing

Treatment Alternatives in the Successful Management of Myasthenia Gravis
I. Clinical question:

“Among myasthenia Immunomodulatory Therapy effective?”





II. Citation:

1. Gajdos P, Chevret S, Clair B, et al. Clinical trial of plasma exchange and highdose intravenous immunoglobulinin myasthenia gravis. Ann Neurol 1997; 41: 789-96 2. Thomas CE, Mayer SA, Gungor Y, et al. Myasthenic crises: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48: 1253-60 3. Andrews PI, Massey JM, Sanders DB. Acetylcholine receptor antibodies in juvenile myasthenia gravis. Neurology 1993; 43: 977-82 III. Study characteristics

Myasthenia gravis is an autoimmune disease that can occur in a limited form involving the oculofacial muscles or in a more generalised form affecting the entire skeletal muscle system. Acetylcholinesterase inhibitors are the first-line treatment of myasthenia gravis and, in most patients, adjunctive immunomodulatory treatment (e.g. corticosteroids) is also indicated. Corticosteroid-refractory myasthenia gravis can be treated with azathioprine or cyclosporin. In myasthenic crises, where the respiratory muscles are affected and the condition becomes life threatening, immunomodulatory treatment is accompanied by plasmapheresis or intravenous immunoglobulin to reduce the acetylcholine receptor antibody titre.

Pathogenesis Is Clear
Myasthenia gravis is probably the best understood of the human autoimmune diseases characterized by weakness and fatigue ability of the skeletal muscle. It can occur either in a limited form involving the oculofacial muscles or in a more generalized form where the entire skeletal muscle system, including the respiratory muscles, may be affected. Typical symptoms include diplopia, ptosis, dysphagia, and proximal muscle weakness, and there is often a disease

.. repetitive nerve stimulation.. Chest computed tomography (CT) scan is the most common method for detection and since many thymomas ( 30%) are locally invasive at the time of surgery it is generally accepted that all suspected thymomas should be removed. edrophonium test. Before Starting Treatment.. . There is no clinical difference between seropositive and seronegative patients and the same management procedures are followed for both patient groups.Myasthenia gravis has a prevalence of 5 to 15 per 100 000 and an annual incidence of 4 to 11 per million individuals. Immune Response is Well-Defined Myasthenia gravis is a prototypic autoimmune disease with a well-established pathogenesis. Distinguishing myasthenia gravis from other neuromuscular disorders and non-autoimmune forms of the disease is important prior to initiation of treatment.. Most patients (85 to 90%) with generalised myasthenia gravis are seropositive for immunoglobulin G (IgG) AChR antibodies whilst fewer patients with the isolated ocular form of the disease show IgG AChR antibodies (50 to 70%).Confirm Diagnosis..history of fluctuating symptom severity.And Check for Thymoma Thymomas are fairly common in patients with myasthenia gravis ( 10%) especially the elderly. Generally no correlation exists between disease severity and antibody titre in either form of the disease. and single-fibre electromyography).. Confirmation of diagnosis should be accompanied by examining the patient's background for a familial history of symptoms of myasthenia gravis. . however. There are certain factors to be considered when contemplating the introduction of immunomodulatory therapy in patients with myasthenia gravis. clinical improvement can be associated with a fall in antibody titre. and for exposure to precipitating drugs such as penicillamine. Diagnosis should be based on positive results from at least two of the main diagnostic tests (AChR antibody assay. Antibodies bind to nicotinic acetylcholine receptors (AChRs) located on the post-synaptic membrane of the neuromuscular junction. reducing receptor number and disrupting neuromuscular transmission. ..

commonly pyridostigmine. Azathioprine can be used as adjunctive therapy in patients with corticosteroid-refractory disease. larger randomised. The principal complications of corticosteroid therapy are cushingoid features. Patients receiving medium-to long-term therapy require bone density scans and osteoporosis prophylaxis with bisphosphonates.5 to 12. hyperglycaemia. Corticosteroids are particularly useful in the long-term treatment with clinical responses becoming evident within 2 to 4 weeks and peaking after 6 to 12 months. Options for Immunomodulatory Therapy Several alternative immunotherapies are available for the treatment of myasthenia gravis (see Patient care guidelines). cataracts and osteoporosis with at least one adverse effect occurring in 60% of patients with myasthenia gravis. Although the use of corticosteroids in myasthenia gravis has been widely reported. Clinical improvements are initially seen 3 to 6 months after starting therapy and reach a maximum effect up to 36 months later. treatment can be tapered over several months with the intended maintenance dosage as low as possible ( 7..Followed by Azathioprine. comparative trials with prednisone and azathioprine have shown responses to corticosteroid treatment in the order of 70 to 90%. High dosages can cause cholinergic adverse effects such as cramping abdominal pain. In patients with mild disease. Thus patients are started at a relatively low daily dosage (10 to 20 mg/day) and escalated (at 5 to 10 mg/week) until a satisfactory clinical improvement is detected or until a maximum dosage of 50 to 60 mg/day is reached. hypertension.. in combination with immunomodulatory therapy..5 mg/day). Dose escalation of prednisone is used to minimise the exacerbation of weakness that can occur when initial high dosages are used. although additional therapy may be needed if response is inadequate after one or two weeks. . Patient Care Guidelines Corticosteroids First. Once a clinical improvement has been maintained. bradycardia and weakness. pyridostigmine can be used alone.. The use of azathioprine in myasthenia gravis is supported by several randomised controlled trials showing response rates of 70 to 90%.. Nevertheless. as corticosteroidsparing therapy or as primary therapy in patients with a relative contraindication to .. only one placebo-controlled randomised trial has been conducted which returned inconclusive data.Cholinesterase Inhibitors at Onset Patients with severe myasthenia gravis at onset are treated with acetylcholinesterase inhibitors.

. separation of red blood cells from the plasma. IVIg is generally well tolerated. Other adverse effects include nausea. In myasthenia gravis this facilitates the removal of pathogenic antibodies and has generally been used as acute therapy particularly in myasthenic crises. In general. . It is generally used in cases where azathioprine is contraindicated and has the advantage of a more rapid rate of onset. There have been no large randomised trials of this treatment and as yet it is not widely available.corticosteroids. .. The usual starting dosage is 50 mg/day. Dose reduction can be attempted when clinical symptoms are stable. Last-line of Defense Several alternative treatment options remain which may be suitable in specific cases. increased by 25 to 50mg daily per week to a maintenance dosage of 2 to 3 mg/kg/day. Cyclophosphamide and mycophenolate mofetil have both shown some efficacy in the treatment of myasthenia gravis in a small number of patients. relapse occurs in 50% of patients in whom treatment is discontinued. and adverse events were less than half as frequent with IVIg than with plasmapheresis. onset of action was slower. Intravenous immunoglobulin (IVIg) is effective in the treatment of uncontrolled myasthenia gravis and myasthenic crises and has been evaluated in a direct comparative study with plasmapheresis. although anaphylaxis is a risk in IgA-deficient patients because of development of antibodies to IgA.Or Cyclosporin Two double-blind. Azathioprine also has a risk of teratogenicity and so is contraindicated in women of child-bearing potential. headache. Plasmapheresis is the process of removal of the blood. Long-term effects of nephrotoxicity and hypertension have limited the use of cyclosporin particularly in patients with pre-existing renal impairment and in the elderly. hirsutism and increased risk of malignancy. Gastrointestinal and haematological adverse effects are seen in 35 to 55% of patients and therefore liver enzymes and full blood count should be monitored regularly. and reinfusion of red blood cells in a colloid solution. randomised. The recommended dosage is 5 mg/kg/day given in two divided doses. in association with thymectomy. and this can be tapered to a maintenance dose once the patient is clinically stable. and while awaiting response to slower-acting immunosuppressants. Anecdotal reports suggest that mycophenolate mofetil is effective in patients refractory to all previous therapies whilst the use of cyclophosphamide is generally reserved for severe and corticosteroid-refractory cases. however. placebo-controlled studies have shown cyclosporin to be effective in treating myasthenia gravis.

patients with suspected thymoma was removed since the treatment is invasive to the patients with thymoma. and initiation of corticosteroids. Methodology The method that was used is experimental.the study was done in the United States of America. Myasthenic Crises Are Life Threatening Myasthenic crises is an acute deterioration of myasthenia gravis affecting respiratory muscle function leading to respiratory failure. many crises also remain unexplained. It is generally accepted that pyridostigmine is withdrawn during intubation because of the increasing number of life-threatening cardiac complications. however. V. and single-fibre electromyography for the diagnosis. they have used different diagnostic procedure and study the background of the patient to confirm the disease to the patient. It is unclear whether early immunosuppression in these patients reduces the rate of development into the generalised form and thymectomy is only routinely used in the presence of a thymoma. Treatment requires respiratory support whilst any infections are treated and therapy with IVIg or plasmapheresis is administered. thymectomy. Result of the study The Elderly Respond Well The elderly generally respond well to corticosteroid treatment but often require continuous therapy.Special Cases Several specific patient groups warrant separate discussion because of the differences in the management of myasthenia gravis. They used AChR antibody assay. . edrophonium test. IV. Isolated Ocular Myasthenia Gravis Isolated ocular myasthenia gravis is one of the few instances where symptomatic treatment with pyridostigmine alone is widely used. hypertension or postmenopausal osteoporosis. Particular attention should be paid to the occurrence of adverse events in these patients because of the high likelihood of comorbidities such as diabetes mellitus. repetitive nerve stimulation. It can be triggered by infections.

VIII. Reviewers conclusion/commentary . The diagnosis and clinical management are well characterized and usually successful. Treatment of juvenile myasthenia gravis is similar to treatment in adults. VI. Authors conclusion and recommendation Myasthenia gravis is undoubtedly one of the best understood of all human autoimmune diseases. The study can be applied here in the Philippines for the patient with myasthenia gravis.Impact in Pregnancy Unclear The effect of pregnancy on myasthenia gravis is unclear with approximately one-third of patients each experiencing improvement. Differential Diagnoses in Children The most important consideration in treating children is distinguishing juvenile myasthenia gravis from transient neonatal myasthenia gravis and also from congenital myasthenic syndromes. Transient neonatal myasthenia gravis can occur where there is a high ratio of foetal to adult AChR antibodies in the mother. deterioration or no change in their condition. Potentially teratogenic immunosuppressants such as azathioprine should be avoided. These congenital myasthenic syndromes are due to genetic defects affecting the function of AChR and are distinguished by a family history. seronegative AChR. VII. This study is a big help for the nurses and medical team because this will be another choice to treat the disease. and can lead to respiratory and feeding difficulties in the baby for several weeks. thymectomy is avoided because of the risk of inducing immunodeficiency and corticosteroid therapy from an early age can be associated with growth retardation.Applicability The nursing specialization will benefits most from this study because this is a big help to manage this immunologic disease. The major challenge in treating this disease is finding a balance between the beneficial effects of corticosteroids and azathioprine and their potential long-term adverse effects. and lack of fluctuation in symptoms.

330 (25): 1797-810 4. Appropriateness-appropriate treatment for the disease Efficiency-it is efficient Accessibility-it is not that accessible because only some hospitals is offering this treatment. they should find another study or treatment that will have consistent result for it to be effective. Drachman DB. Reference 1. X. et al. Duane DD. Hemmer B. 15 (3): 173-83 2. Lara PN. 274: 596-607 . Effectiveness. Immunosuppresive treatment of ocular myasthenia gravis. Alternate day prednisone: preliminary report of double-blind controlled study. Spring PJ. et al. Ann N Y Acad Sci 1976. For the children and pregnant mothers. The study is good. Tackenberg B. Biodrugs 2001 June. Howard FM. IX. Cancer Treat Rev 2000. Lambert EH. Myasthenia gravis: options and timing of immunomodulatory treatment. N Engl J Med 1994. Myasthenia gravis. Acceptability-it is acceptable for it is effective especially to the elderly patients. 26: 127-31 5. Malignant thymoma: current status and future directions. Spies JM.The treatment is good but it should only be done to elderly client with myasthenia gravis because it is effective to them as the result says. 15 (6): 369-78 3.only competent doctors should do the study for it to be safe to the client. Evaluating nursing care practice • • • • • • • Safe-the study is safe Competent. and somehow effective to pregnant. Biodrugs 2001 March.it is effective to elderly. Oertel WH.

Hohlfeld R. et al.6. Phillips JT. Heininger K. et al. Lecky B. Azathioprine toxicity during longterm immunosuppression of generalized myasthenia gravis.Tindall RSA. 316: 719-24 10. N Engl J Med 1987. Neurology 1998. Randomized trial of azathioprine or prednisone for initial immunosuppresive treatment of myasthenia gravis. Michels M. 38: 258-61 9. 681: 539-51 Treatment Alternatives in the Successful Management of Myasthenia Gravis XI. Wald JJ. Bromberg MB. Phillips JT. Ann N Y Acad Sci 1993. 150: 59-62 7. et al. Palace J. Neurology 1988. et al. A clinical trial of cyclosporine in myasthenia gravis. Preliminary results of a double-blind randomized. et al. Forshew DA. Newsome-Davis J. Clinical question: . 50: 1778-83 8. J Neurol Sci 1997. placebo-controlled trial of cyclosporine in myasthenia gravis. A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Rollins JA. Rollins JA. Tindall RSA.

1-18. 841.. fatigue. Neuberger. L. 31. Cassmeyer. & Bryan..L. L.B.. Citation: Aaronson. and a Demographic and Clinical Data Form. Herbelin. C. Quality of life in multiple sclerosis: The impact of depression. These results lay the groundwork for further investigation of fatigue in MG patients and identification of mitigating or coping methods. D. V. the fatigue severity (MGFS) score correlated moderately with depression. .W. G. (1998). S. L. M. & Lightner. Amato. Ponziani.. J. Three days after the clinic visit. Journal of Behavioral Medicine.. M. Image: Journal of Nursing Scholarship.. Pallikkathayil.I. The MGFS.“Among myasthenia gravis patient. the Center for Epidemiologic Studies-Depression Scale. what is the best predictor that causes fatigue?” XII. L.. Reliability testing of the quantitative myasthenia gravis score.S.A. Rossi... 45-50.P. A convenience sample of 67 MG patients was approached on return visit to the Neuromuscular Disease Clinic.. Stefanile. Findings showed that the MGFS demonstrated adequate internal consistency and test-retest reliability.J. Pierce. (1999). R. et al.S. Liedl. Study characteristics The purposes of this study were to validate psychometric properties of the Myasthenia Gravis Fatigue Scale (MGFS) and to use the MGFS to study the relationship of selected demographic and clinical variables to fatigue in a myasthenia gravis (MG) patient population.. W. 21.. & Rossi. XIII. was developed to measure fatigue severity in MG patients. In addition. Nations. Multiple Sclerosis. Off-treatment fatigue in breast cancer survivors: A controlled comparison. and disability. Wolfe. 7. After giving consent. R. McIntire. participants filled out four questionnaires: the MGFS. S. We adjusted for the effect of depression and noted a relationship between the fatigue score and two of nine selected demographic and clinical characteristics--activity restriction and number of years since diagnosis. the Chalder Fatigue Scale. Barohn. (2001).. Defining and measuring fatigue. Annals of the New York Academy of Sciences. 769-772.. (1998)... 340-344. a copy of the MGFS was mailed to participants to allow retest at 5 to 7 days after initial testing. C.. Participants' muscle weakness was evaluated using the Modified Quantitative MG Score for Disease Severity assessment form (13 muscles tested). Curran. G. C.L. Andrykowski.. Teel. G. R. an existing scale.

such as duration. mainly. Results revealed that the women with MG experienced more severe fatigue than the men. Fatigue has been found to correlate with physical and psychological parameters in patients with multiple sclerosis.. between MG patients who reported that aerobic exercise relieved fatigue and those who reported that aerobic exercise did not relieve their fatigue. the Fatigue Assessment Inventory (O'Dell et al. in an unpublished study. 1998. exhaustion after usual activity. Jantos. 1997.. and Gilchrist (2000) administered a cognitive intervention to study fatigue and the effect of fatigue on quality of life in MG patients (n = 28) and control participants (n = 24). O'Dell. 1998). Monkman. swimming.61). Sauriol. Reilly. Goldstein. McDonald. there was not a significant difference in fatigue score. or all of the above. Packer. 1997). 1996. Ricci. Becker. and it has been shown to be associated with poor outcomes (Amato et al. & Passik. in contrast to normal fatigue. 1997). and AIDS (Breitbart.. visual analog scales and questionnaires with Likert-scale format. and Danehy (1994). & Ricci. surveyed 250 MG patients regarding the characteristics of their fatigue. In the same study.. at each level of functional status measured. lack of energy to complete tasks. 1994). Fatigue may negatively affect a person's ability to perform physical or mental activity as well as quality of life. The results showed that the MG patient group demonstrated significantly greater cognitive (mental) and physical fatigue than the control group (p < . Vercoulen et al. the Fatigue Severity Scale (Packer. Hwang. HIV infection. Chang. time of occurrence. women reported more fatigue than men in various age categories. & Anson. However. 1998). including the Checklist of Individual Strength-Fatigue (van der Werf et al. Cogswell. Fatigue severity was measured by the Myasthenia Gravis Fatigue Scale (MGFS). 2001. is a common complaint in both ill and healthy people (Aaronson et al. Foster. Grohar-Murray. & Riggs. does not subside with rest and is characterized by a feeling of tiredness before activity. Paul. Cohen. Meighen. Polak. Pathological fatigue.********** Fatigue. chronic fatigue syndrome. 2002. & Basimis.. and running helped MG patients relieve their fatigue (Grohar-Murray. Rosenfeld. Another study by the same group showed that low-impact aerobic exercise such as walking. an adaptation of an instrument used for multiple sclerosis patients.. level of physical activity was found to correlate with fatigue severity (r = . Michael. 1999) and is especially common in those with neurological disorders and cancer. The current study was designed to investigate these relationships using an instrument adapted to measure fatigue in MG patients. as measured by MGFS.. 1997. 1998. Ford.05) both before and after the intervention. & Brouwer. a subjective experience that ranges from tiredness to exhaustion. Trigwell. There is only one published study on the correlation between physical and psychological factors and fatigue in myasthenia gravis (MG) patients. Becker. and exacerbating factors. and in the aerobic exercise group. Walker. 1996). Fatigue has been measured with various instruments. Vercoulen et al. the Chalder Fatigue Scale (Ford et al. 1996. 1998. 2002). & Brouwer. Age category did not significantly affect fatigue score. as well as self-care management techniques. McGown. & Johnson. . Also.

Walker et al. Luciano. age. Among the demographic and clinical factors chosen. write. and Becker (1994) combined and modified two unpublished questionnaires. Hench. Phillips. additional data on psychometric parameters for the MGFS were collected to further its usefulness as a fatigue scale for MG patients. the 26-item adapted instrument. Measurements . number of medications taken daily. Otero.. 1998). & Woods. for use with MG patients. Muir-Nash. O'Dell et al. Taylor. 1997). (c) between the ages of 19 and 70 years. gender. Libbus.the Piper Fatigue Scale (Cupler. However. recruitment of participants through their physician. and the Multidimensional Assessment of Fatigue (Schwartz. 1989. et al. but only parts of their data have been published (Grohar-Murray et al. Gladman. Hubsky. Sears. Becker. In the current study. 1996. Protection of participants was ensured by approval of the study by the University of Alabama at Birmingham Institutional Review Board.. LaRocca. Methodology Participants Sixty-seven patients who were making a return visit to the Neuromuscular Disease Clinic at the University of Alabama at Birmingham Medical Center agreed to participate in the study. 1994. and understand English. Therefore. although there are no studies on these factors in MG patients. these instruments are not specific for measuring fatigue in the MG patient population. The specific purposes of this study were to (a) validate psychometric properties of MGFS and (b) use the MGFS to study the relationship of selected demographic and clinical variables to fatigue in a MG patient population. XIV. Depression has been reported to correlate with fatigue in both healthy persons and those with multiple sclerosis (Lee. Lentz. 1996). Mitchell. which were used to measure fatigue in multiple sclerosis patients. & Steinberg. systemic lupus erythematosus (Krupp. 1996. Schwartz et al. Osgood. this variable was also included in the current study. (1994) and Grohar-Murray. and (d) not subject to any other neuromuscular or other debilitating disease such as heart failure or hemolytic anemia.. duration of MG. To be eligible they were (a) diagnosed with either ocular MG or generalized MG. and a disease severity score were expected to relate to fatigue. Wang. and physical activity had been investigated by Grohar-Murray. 1998). Baker. and HIV/AIDS (O'Dell et al. Sears. (b) able to read. 1995. history of thymectomy. 1996). et al. Body mass index (BMI). & Zeng. & Valentine. They have done preliminary psychometric studies on MGFS. Grohar-Murray. 1996). Coulthard-Morris. & Dalakas. & Urowitz.. and signed consent forms. (1994)..

Test-retest reliability of the MG group and the control group was determined. Each item has four options: (a) better than usual. The score can be computed by summing the ratings on items.Four questionnaires were used to measure or record participants' fatigue. Content validity indexes of MGFS.80. . and 9 items measure the observable motor signs or symptoms resulting from fatigue (MGM). and .92 for MGP. et al. Internal consistencies of the total scale and physical fatigue and mental fatigue subscales were evidenced by alpha coefficients of . and has excellent psychometric properties.90. and selected demographic and clinical characteristics. The CES-D scale is designed to assess the frequency of occurrence of depressive symptoms during the past week (Radloff..85 for each group. 1977). and . respectively (Chalder et al. Center for Epidemiologic Studies-Depression (CES-D) Scale. It contains 26 items that fall within three subscales: 9 items measure perception of fatigue (MGP). (c) worse than usual. A score of 16 or greater indicates clinical depression. MGT. The total scale and subscale scores were calculated by Cronbach's alpha. and MGM. High scores indicate greater fatigue. 821. Myasthenia Gravis Fatigue Scale. and 1 = never). p = . The CES-D has adequate psychometric . determined by a panel of experts.).908. 2 = sometimes. 3 = frequently. 8 items measure the task avoidance behaviors resulting from fatigue (MGT). were . is relatively easy to administer. respectively.. possible scores range from 0 to 14.88 to . 8 of which measure physical fatigue and 6 of which measure mental fatigue. 1993).89 for the overall scale. and (d) much worse than usual.. Construct validity of the MGFS was investigated using a group of MG patients and a group of normal adults. A 4-point Likert scale is used to indicate frequency. In addition. respectively (Grohar-Murray et al. Items on the MGFS are answered with a 5-point Likert scale (5 = always. .916. with alpha coefficients of .70. were found. (b) no more than usual. possible scores range from 0 to 60. The CFS is designed to measure fatigue severity and can be used with both hospital and community populations (Chalder et al.845. It is contains 14 items. 4 = usually. The MGFS was designed to measure fatigue severity for MG patients (Grohar-Murray. a protocol recommended by Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America (Task Force. 2000) was used to assess muscle weakness. alpha coefficients of . In the current study.98. Sears. Internal consistency of the MGFS was calculated. possible scores range from 26 to 130. With this method of scoring. with correlation coefficient of . and .). 1994). A bimodal response system is commonly used to score this questionnaire (0 = better than usual or no more than usual. The CFS was chosen as an additional fatigue assessment because it measures both physical and mental aspects of fatigue. with higher scores indicating greater fatigue.90 and . Chalder Fatigue Scale (CFS). 1998). 1 = worse than usual or much worse than usual).001 (Grohar-Murray et al. and . depression. Internal consistency and test-retest reliability of the MGFS have been examined with both MG patients and volunteers without MG. The difference in scores was significant.937. It consists of 20 items that describe characteristics of depression. The MGFS score is then computed by summing the ratings on items. .

Alpha coefficient of the CES-D scale. Higher scores indicate greater muscle weakness or disease severity. The Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America (2000) has recommended that QMG be used in all prospective studies of therapy for MG patients. the scores could range from 0 to 39. Therefore. arm and leg movement. two of the 13 items were modified.. 4 without MG). swallowing. It takes approximately 30 minutes to complete the 13-item QMG (Wolfe et al. gender. and history of thymectomy for the participants. instead of asking the participant to swallow 4 oz of water and observing possible choking. To make the QMG safer and more convenient for use in the current study. was . current medications. Some information for the Demographic and Clinical Data form was obtained from medical records. which was 1. facial expression. hand grip.34 out of 39. (1998) to assess muscle weakness in MG patients. It was calculated as the average of standard deviations of each participant's scores. The QMG comprises 13 physical tests of different muscle groups involved in head lifting.84 and . The modified QMG used in the current study was named the Modified Quantitative MG Score for Disease Severity (MQMG). the investigator examined each participant's muscle weakness using the MQMG protocol. and CES-D scale. Modified Quantitative MG Score for Disease Severity (MQMG). and ventilation. body mass index (BMI). The Quantitative Myasthenia Gravis Score (QMG) instrument was developed by Barohn et al. 1999). if needed. Procedure Patients on a return visit to the Neuromuscular Disease Clinic were given an explanation of the study via letter from their clinic physician and a verbal explanation by the investigator. A Demographic and Clinical Data Form was developed by the investigator. Tindall. 1993). It was used to record selected demographic and clinical characteristics. 1987. MGFS.865. As each participant completed the questionnaires. Performance on each item is scored on a 4-point Likert scale (0 = no weakness to 3 = severe weakness). The investigator was present in the clinic waiting room or examination room to assist with the questionnaires. the Quantified Myasthenia Gravis Strength Score (Tindall et al. the investigator asked the participant about his or her response to swallowing liquids. Rollins. Internal consistency calculation in the current study yielded an alpha coefficient of . Wells. speech. rather than the suggested spirometer. For one item. Interrater reliability of the QMG was calculated using two to five raters for each of 9 participants (5 with MG. for another item. The spirometer commonly used in the clinic was used. . Those who consented to participate were asked to fill out the four questionnaires: the Demographic and Clinical Data form. This instrument was derived from a previous instrument. such as age. QMG provides a precise quantitative muscle weakness score. as used in the current study. & Hall. Phillips. eye movement.properties. with alpha coefficient between . Demographic and Clinical Data Form.90 (Radloff). CFS.906..

872. followed by prednisone and azathioprine (Imuran) or another immunosuppressive drug. The most common health problem was hypertension (44. as measured by MGFS. were overweight or obese as indicated by BMI (77.9%).5%). stamped envelope. Data Analysis The Statistical Package for the Social Science (SPSS) version 10. along with a self-addressed. About half of the participants had undergone thymectomy. 58.934. pyridostigmine (Mestinon) was the most commonly prescribed drug. and Cronbach's alpha calculation was employed to examine internal consistency of the MGFS and other appropriate instruments used in the study. Psychometric Properties of the MGFS The initial administration of the MGFS for 67 participants was used to examine the internal consistency of the instrument. Therefore. Post hoc power analysis for multiple regression was performed by using the method of Cohen and Cohen (1977). XV. Result of the study Sample Description Participants ranged in age from 24 to 67 years (M = 48.2% reported that they were able to do self-care. A telephone call to remind the participant to return his or her questionnaire was made on the fifth day after the initial visit.6%).850 to . Sixty-seven patients participated over an 8-month period. test-retest reliability was examined by comparing scores from 49 participants on initial testing and repeat testing 5 to 7 days later.001).8%). ranging from .1%). and 2 returned the questionnaire too late for it to be analyzed. Pearson's product-moment correlation coefficient was calculated to determine test-retest reliability of the MGFS. p < .Three days after the clinic visit. and 9.4%). was mailed to each participant to allow retest within 5 to 7 days of initial testing. followed by diabetes mellitus (19. . a new copy of the MGFS. The coefficient alpha scores for MGFS and its three subscales were excellent. About a third were fully active. Approximately 69% reported other health conditions. and respiratory problems (14. As part of MG treatment. Multiple regression analysis was used to examine the relationship between fatigue score. and had generalized MG (92. as shown in Table 2. or office work but were restricted in strenuous physical activity. controlling for appropriate covariables. Almost half of the participants were diagnosed with MG within the past 5 years.0% were restricted even in the ability to walk. light housework.4 years) and were approximately evenly divided by sex (Table 1). The majority were Caucasian (70. For the 1-week retest of the MGFS. 16 did not return the questionnaire. 49 of the 67 participants mailed the questionnaire back within 7 days of the initial test. and selected demographic and clinical variables. The result showed high test-retest reliability (r = .0 was used to analyze the data.

The independent variables were then allowed to enter the model stepwise.25 as the exclusion criterion. p = . statistical significance was obtained and [R.sup. The nine variables were age. gender. Stepwise multiple regression analysis with depression as a covariable was then used to assess the relationship of nine selected demographic and clinical variables to fatigue score. number of years since diagnosis.sup. Therefore. BMI. with depression as a covariable in the regression model. CFS scores for physical. and depression (CES-D) score.056). it was noted that one item is difficult to interpret. (1998).sup. and the nine independent variables were then allowed to enter the model stepwise using alpha = .2] = . gender. activity restriction. Examination of residual analyses revealed two influential outliers. and total fatigue." This item is scored on a scale with anchors 1 (never) to 5 (always).2] change = . Post hoc power analysis for this multiple regression analysis yielded a power (1-[beta]) of . Discussion The MGFS demonstrated adequate internal consistency.191). mental.025). and history of thymectomy) were performed to examine the relationships between fatigue. The first independent variable to enter was activity restriction ([R. Item 14 contains the statement "Rest relieves my fatigue. number of years since diagnosis. that variable was forced into the regression model at the initial step. followed by number of years since diagnosis ([R. respectively.Fatigue and Selected Demographic and Clinical Variables Multiple regression analyses with five covariables (age. the analyses were repeated with the exclusion of the two respective participants (n = 65).370. As shown in the table. BMI. The resulting bivariate correlations are shown in Table 3. In the overall regression model. To control for the effect of depression. 59% of the variance in the MGFS score was accounted for by depression. as measured by MGFS. The test-retest reliability of the MGFS within a 1-week time period was high (r = .20 as a criterion to enter the equation and alpha = . Activity restriction and number of years since diagnosis explained 19% and 3% of the variance. A score of 5 could be interpreted as indicating either a high or a low level of fatigue. and disease severity (MQMG) score. indicating that the sample size was sufficient to detect the absence of correlation between fatigue and variables that did not enter the model. activity restriction. number of current medications. and number of years since diagnosis. The correlation with disease severity score approached statistical significance (r = . To determine whether differing interpretations of this item would .313 to .872) and was almost identical to the 2-week test-retest results obtained by Grohar-Murray et al. other morbidity. and disease severity.2] change = . Depression accounted for 37% of the variance in fatigue score.237. MGFS fatigue score correlated positively (r = . in the initial step. history of thymectomy.515) with all fatigue and depression scores. The current findings strongly support the previously described results from preliminary psychometric testing published by Grohar-Murray et al. A summary of the stepwise multiple regression analysis for the MGFS with 65 participants is presented in Table 4.94. On inspection of items included in the MGFS before use in the current study.

item 14 was retained and scored forward as in the original instrument for the remaining analyses. the muscle weakness score was the actual score for muscle weakness at the moment of testing.463. fatigue. MGP (fatigue perception) and MGT (task avoidance) subscales of the MGFS seem to be most comparable to the physical fatigue subscale of the CFS. Cronbach's alpha calculation was performed for total MGFS and the MGT subscale (which included item 14) using forward and reverse scoring of item 14. the correlation results were improved. and . and total fatigue (using the 4-point CFS responses) were . respectively.518. The results showed minimal differences in Cronbach's alpha. Low positive correlations were found between the MGFS score and the CFS scores for physical. The improvement in correlation results indicated that the bimodal rating system of the CFS and inclusion of the MGM (motor sign or symptoms) subscale of MGFS may have been factors . Also. there was a significant correlation between these two scores (r = . and in the majority of reported studies.003 for the total MGFS and . There may be several reasons for the fact that the correlations for these fatigue scales were low. p < . and total fatigue (r = . On inspection of specific items. morning or afternoon) and duration of action of the common drug pyridostigmine could affect the degree of muscle weakness.237.05). . a 5-point Likert scale rating is used for responses on the MGFS. To determine whether use of the bimodal rating system affected the results. The MGFS contains three subscales and the CFS. Correlation coefficients between the combined MGP and MGT score and the CFS scores for physical. mental. In addition. Therefore. On the other hand. respectively). mental. without the inclusion of the five covariables. because the MGFS score indicates physical fatigue and the MQMG score indicates muscle weakness. the MGFS is designed for MG patients.390. p = . the correlation analysis was rerun using the 4point Likert scale for the CFS. 457. a difference of . and participants responded to the MGFS by considering their overall.006 for the MGT subscale. It was surprising that the correlation between the fatigue (MGFS) and disease severity (MQMG) scores was low and only approached statistical significance (r = . The results showed only a small increase in the correlation coefficients between the MGFS score and the CFS scores for physical. and total fatigue. two subscales. the time that muscle weakness was tested (i. whereas participants respond to CFS items on a 4-point Likert scale. . these responses are recoded to produce a bimodal rating system (yes or no).459.495. Low correlation between the MGFS and CFS may also be due to differences in emphasis.affect the internal consistency of the instrument. When the analyses were rerun using the combined score of the MGP and MGT subscales rather than the total MGFS score.056). and . mental. whereas the CFS is not designed for persons with any specific disease. Although the MGFS and CFS are both designed to measure severity of fatigue.. Thus.296. Note that when simple correlation between the MGFS score and MQMG was run. Another explanation for the lack of significant correlation is that the MGFS is a self-report questionnaire. it was concluded that the interpretation of this item did not substantially alter the results of the MGFS. The fact that the correlation approached significance indicated that it most likely would become significant if the number of participants were increased. rather than momentary.e.

2] = . which was consistent with the study of Grohar-Murray. Schwartz et al.. et al. 60 had generalized MG and 5 had ocular MG. Hoover. 1994. 1997). yielded correlations between . The current findings showed a moderate correlation between depression score and the MGFS score (r = . 1996. Therefore. Depression has been reported to be correlated with fatigue in both healthy people and patients. The regression analysis results showed that. the greatest correlation coefficient seen between the two fatigue assessments was only moderate (r = . Grohar-Murray.61). 2002. However. Becker. When nine other variables that might affect fatigue were examined. & Fields. It is not surprising that.. 515). Thus. as fatigue scores increased.sup. Walker et al. after controlling for the effect of five covariables (Table 3). reported a moderate correlation between fatigue severity score and their variable called level of functional status (r = .580. Libbus et al. 001). The resulting regression model was similar to the regression model for all 65 participants ([R. because activity restriction might be different between these 5 and the 60 generalized MG patients. it can be concluded that type of MG did not confound the regression analysis results.001. the severity of fatigue increased also.576.17 and . 22% of the variance in fatigue score was explained by two variables. MG patients were less physically active. Dzurec. as the duration of disease increased.. 1998. after controlling for the effect of depression. Similarly.. Authors conclusion and recommendation . 1995. the stepwise regression analysis was rerun with the 60 generalized MG patients only. even with deletion of these two factors. Of the 65 participants in the current study. p < .that decreased the correlation between scores on the two fatigue scales. 1996. This is not surprising because other studies that compared fatigue and depression in various populations.. this level of functional status variable comprised the same items as activity restriction in the current study. The best predictor of fatigue severity was activity restriction. Lee et al.518). depression score was used as a covariable because of its moderate correlation with fatigue score. XVI. & Lightner. 1998.. p < . O'Dell et al. Ford et al. r = . For 250 MG patients. In the current study. et al. (1994). followed by number of years since diagnosis. the zero-order correlation between MGFS score and activity restriction was moderate. Becker. Curran.82 (Andrykowski. It is possible that the 5 ocular MG patients may have confounded the regression results.

In this study regardless of the age group MS could occur. e. XIX.it is effective to elderly. XVII. Competent. and somehow effective to pregnant.The best predictor of fatigue severity was activity restriction. Patients as well as medical team should surely have the knowledge to take the responsibility to explain to our patients the best possible health care especially for those who are suffering from myasthenia gravis. Applicability The nurses. Most probably it will help the patients and the medical team to alleviate suffering and pain not only to those myasthenia gravis patient but as well as for the other people might have it. It said to be the best predictor of fatigue severity was activity restriction. Effectiveness. XVIII. Appropriateness-appropriate treatment for the disease . It also will lay the groundwork for further investigations. resulting in improving patients' quality of life. Evaluating nursing care practice a. Knowledge of the relationship between fatigue severity and physiological/psychological factors such as activity restriction and depression will help healthcare providers provide comprehensive care to their MG patients. c. Basically to determine the Participants' muscle weakness and the triggering factors that causes fatigue. Acceptability-it is acceptable for it is effective especially to the elderly patients d. Safe-the study is safe b. Since this is a rare neurological disorder as studied and prove by research we could give the best quality care for our patient especially for those who are suffering from myasthenia gravis. In addition depression was also one of the triggering factors that causes fatigue. as well as the medical team would benefit the most.only competent doctors should do the study for it to be safe to the client. Reviewers conclusion/commentary The study “fatigue in myasthenia gravis patients” implies about the study of patients who’s suffering from myasthenia gravis.

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