Rev Neurol. 2007 Apr 1-15;44(7):397-403.
[Article in Spanish]
de Entrambasaguas M, López-Bernabé R, López-Alemany M.
Servicio de Neurofisiología Clínica, Hospital General de Castellón, 12004 Castellón de la Plana, España. entrambasaguas_man@gva.es
Abstract
INTRODUCTION: Myasthenia gravis lacks a diagnostic gold standard, so diagnosis is supported by the findings of several tests. Conversion from ocular myasthenia gravis (OMG) to a more widespread disease is frequently early. AIMS. To describe and compare the clinical data and findings of the diagnostic tests of patients with OMG and those with a different diagnosis, and to know the conversion rate from OMG to generalized myasthenia. PATIENTS AND METHODS: Descriptive and analytic retrospective study of 44 patients referred for neurophysiological testing through 4 years because of suspected OMG. RESULTS: 12 patients (27%) were diagnosed as having OMG. Evolution time prior to diagnosis tended to be shorter in OMG patients. Isolated ptosis or combined with diplopia was more frequent in OMG, while isolated diplopia was so in other diagnoses (p = 0.003). No thymoma was found. SFEMG jitter of facial muscles was abnormal, including blocking, in all OMG patients (8/8) and normal in the rest (30/30). Edrophonium test was positive in all OMG patients (7/7) and doubtful in another one (1/7). Anti-AChR titers were initially positive in 10/11 OMG patients and 0/17 with other diagnoses. Aponeurotic ptosis and strabismus were the most frequent non-myasthenic etiologies. OMG showed an early generalization in two patients who developed dysarthria. CONCLUSIONS: The low ratio of diagnostic confirmation suggests that in the face of ptosis or diplopia diagnostic testing with a high sensibility for OMG is favoured. Jitter showed the best initial diagnostic performance.
Thursday, August 5, 2010
Predictable recovery from myasthenia gravis crisis with plasma exchange: thirty-six cases and review of current management.
J Clin Apher. 1999;14(1):1-8.
Mahalati K, Dawson RB, Collins JO, Mayer RF.
Department of Pathology, The University of Maryland School of Medicine, Baltimore, USA.
Abstract
Adult, acquired, idiopathic, autoimmune myasthenia gravis has a well-characterized IgG anti-acetylcholine striated-muscle receptor antibody. Removal by plasma exchange is effective, established therapy to augment anti-cholinesterase and immunosuppressive therapy and is the treatment of choice for myasthenia gravis crisis. We report 36 consecutive patients referred and accepted for plasma exchange, 32 of whom were in or entering myasthenia crisis, over a 10 year period. An average of 7.8 (range 1 to 16) plasma exchange procedures were done, with uniform, significant improvement, including extubation of 13 in myasthenic crisis and discharge from hospital in all. We conclude that this is the best treatment for myasthenia gravis crisis in hospital. From recent cases, most, if not all, crises can be prevented by IVIgG or plasma exchange as out-patients with use of corticosteroid and or azathioprine.
Mahalati K, Dawson RB, Collins JO, Mayer RF.
Department of Pathology, The University of Maryland School of Medicine, Baltimore, USA.
Abstract
Adult, acquired, idiopathic, autoimmune myasthenia gravis has a well-characterized IgG anti-acetylcholine striated-muscle receptor antibody. Removal by plasma exchange is effective, established therapy to augment anti-cholinesterase and immunosuppressive therapy and is the treatment of choice for myasthenia gravis crisis. We report 36 consecutive patients referred and accepted for plasma exchange, 32 of whom were in or entering myasthenia crisis, over a 10 year period. An average of 7.8 (range 1 to 16) plasma exchange procedures were done, with uniform, significant improvement, including extubation of 13 in myasthenic crisis and discharge from hospital in all. We conclude that this is the best treatment for myasthenia gravis crisis in hospital. From recent cases, most, if not all, crises can be prevented by IVIgG or plasma exchange as out-patients with use of corticosteroid and or azathioprine.
Myasthenia gravis. Diagnostic methods and control measures for a chronic disease.
Postgrad Med. 1993 Oct;94(5):161-4, 167-71, 174.
Juhn MS.
Garden City Hospital, Michigan.
Abstract
Myasthenia gravis should be entertained as a possible diagnosis in any patient who presents with muscle weakness or visual disturbance. The most common symptom is exacerbation of muscle fatigue with repeated use of the muscle and improvement with rest. Several inexpensive diagnostic tests for myasthenia gravis that can be performed by primary care physicians are available. Standard therapy in most cases is early thymectomy, followed by a highly individualized medication program that usually includes the anticholinesterase drug pyridostigmine bromide (Mestinon, Regonol). Corticosteroids and other immunosuppressive agents (usually azathioprine [Imuran]) may be added or substituted if response to anticholinesterase therapy is inadequate. Although myasthenia gravis is a chronic disease, it can be well controlled in most patients, provided they comply with treatment. Patient education is therefore essential.
Juhn MS.
Garden City Hospital, Michigan.
Abstract
Myasthenia gravis should be entertained as a possible diagnosis in any patient who presents with muscle weakness or visual disturbance. The most common symptom is exacerbation of muscle fatigue with repeated use of the muscle and improvement with rest. Several inexpensive diagnostic tests for myasthenia gravis that can be performed by primary care physicians are available. Standard therapy in most cases is early thymectomy, followed by a highly individualized medication program that usually includes the anticholinesterase drug pyridostigmine bromide (Mestinon, Regonol). Corticosteroids and other immunosuppressive agents (usually azathioprine [Imuran]) may be added or substituted if response to anticholinesterase therapy is inadequate. Although myasthenia gravis is a chronic disease, it can be well controlled in most patients, provided they comply with treatment. Patient education is therefore essential.
Diagnostic problems in patients with myasthenia gravis
Srp Arh Celok Lek. 2000 Jul-Aug;128(7-8):247-52.
[Article in Serbian]
Lavrnić D, Rakocević-Stojanović V, Tripković I, Pavlović S, Stević Z, Trikić R, Nesković V, Apostolski S.
Institute of Neurology, Clinical Centre of Serbia, Belgrade.
Abstract
Myasthenia gravis (MG) is an antigen-specific autoimmune disease in which antibodies directed against nicotinic acetylcholine receptors of the postsynaptic muscle membrane (nAChR) impair neuromuscular transmission. MG is clinically characterized by abnormal muscle fatigue and weakness. The initial symptoms and signs are often unrecognized. Therefore, we analyzed the diagnostic errors and duration of diagnostic delay in patients affected with MG (n = 444) in a ten-year period (January 1, 1983-December 31, 1992) in Yugoslavia. The initial diagnosis was correct in 44.4% of patients and erroneous in 38.4%; 17.2% of patients were admitted without an initial diagnosis. The average duration of diagnostic delay was 11 months. We present the differential diagnostic difficulties in MG and discuss the principles of diagnostic strategy which may reduce the risk of diagnostic errors in MG.
[Article in Serbian]
Lavrnić D, Rakocević-Stojanović V, Tripković I, Pavlović S, Stević Z, Trikić R, Nesković V, Apostolski S.
Institute of Neurology, Clinical Centre of Serbia, Belgrade.
Abstract
Myasthenia gravis (MG) is an antigen-specific autoimmune disease in which antibodies directed against nicotinic acetylcholine receptors of the postsynaptic muscle membrane (nAChR) impair neuromuscular transmission. MG is clinically characterized by abnormal muscle fatigue and weakness. The initial symptoms and signs are often unrecognized. Therefore, we analyzed the diagnostic errors and duration of diagnostic delay in patients affected with MG (n = 444) in a ten-year period (January 1, 1983-December 31, 1992) in Yugoslavia. The initial diagnosis was correct in 44.4% of patients and erroneous in 38.4%; 17.2% of patients were admitted without an initial diagnosis. The average duration of diagnostic delay was 11 months. We present the differential diagnostic difficulties in MG and discuss the principles of diagnostic strategy which may reduce the risk of diagnostic errors in MG.
Misdiagnosis of Myasthenia Gravis
J Natl Med Assoc. 1987 April; 79(4): 425–429.
Steve D. Wheeler, MD
Cleveland, Ohio
Patients with myasthenia gravis (MG) are too often misdiagnosed as having another disorder. Three patients are presented who were thought to have amyotrophic lateral sclerosis, velopharyngeal incompetence, and no diagnosis of MG, but actually each had myasthenia gravis. Their histories illustrate how the diagnosis of myasthenia gravis can be easily missed.
There is no typical case of myasthenia gravis but, rather, this entity remains a clinical diagnosis that relies on a well-taken history, adequate examination, and appropriate interpretation of laboratory tests. To miss the diagnosis of myasthenia gravis is to cause the patient social, psychological, medical, and economic suffering.
Printable PDF of the complete article:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2625490/pdf/jnma00919-0097.pdf
Steve D. Wheeler, MD
Cleveland, Ohio
Patients with myasthenia gravis (MG) are too often misdiagnosed as having another disorder. Three patients are presented who were thought to have amyotrophic lateral sclerosis, velopharyngeal incompetence, and no diagnosis of MG, but actually each had myasthenia gravis. Their histories illustrate how the diagnosis of myasthenia gravis can be easily missed.
There is no typical case of myasthenia gravis but, rather, this entity remains a clinical diagnosis that relies on a well-taken history, adequate examination, and appropriate interpretation of laboratory tests. To miss the diagnosis of myasthenia gravis is to cause the patient social, psychological, medical, and economic suffering.
Printable PDF of the complete article:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2625490/pdf/jnma00919-0097.pdf
Does This Patient Have Myasthenia Gravis?
Katalin Scherer, Richard S Bedlack, David L Simel. JAMA. Chicago: Apr 20, 2005. Vol. 293, Iss. 15; pg. 1906, 9 pgs
Abstract (Summary)
As Schrerer et al stress, clinicians must be able to diagnose myasthenia gravis, since delays in establishing the diagnosis may put patients at risk for complications from this treatable disease. Here, Schrerer et al determine if items in the history and examination or results of simple tests change the likelihood of myasthenia gravis as a diagnosis. Results of their study are presented.
Section: THE RATIONAL CLINICAL EXAMINATION
Publication title: JAMA. Chicago: Apr 20, 2005. Vol. 293, Iss. 15; pg. 1906, 9 pgs
Source type: Periodical
ISSN: 00987484
ProQuest document ID: 827173981
Text Word Count 5609
Abstract (Summary)
As Schrerer et al stress, clinicians must be able to diagnose myasthenia gravis, since delays in establishing the diagnosis may put patients at risk for complications from this treatable disease. Here, Schrerer et al determine if items in the history and examination or results of simple tests change the likelihood of myasthenia gravis as a diagnosis. Results of their study are presented.
Section: THE RATIONAL CLINICAL EXAMINATION
Publication title: JAMA. Chicago: Apr 20, 2005. Vol. 293, Iss. 15; pg. 1906, 9 pgs
Source type: Periodical
ISSN: 00987484
ProQuest document ID: 827173981
Text Word Count 5609
Myasthenia gravis: the problem of a "psychiatric" misdiagnosis.
Med J Aust. 1986 Jun 9;144(12):632-8.
Nicholson GA, Wilby J, Tennant C.
Abstract
Patients with myasthenia gravis are commonly misdiagnosed as having a psychiatric disorder; this leads to considerable delay in reaching the correct diagnosis and in instituting appropriate and effective treatment. Patients who are most at risk are those with somatic presentations (limb weakness, fatigue) in contrast to ocular or bulbar presentations. Similarly, those who manifest anger or anxiety traits are more likely to be misdiagnosed.
Nicholson GA, Wilby J, Tennant C.
Abstract
Patients with myasthenia gravis are commonly misdiagnosed as having a psychiatric disorder; this leads to considerable delay in reaching the correct diagnosis and in instituting appropriate and effective treatment. Patients who are most at risk are those with somatic presentations (limb weakness, fatigue) in contrast to ocular or bulbar presentations. Similarly, those who manifest anger or anxiety traits are more likely to be misdiagnosed.
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