Myasthenia Gravis Support Group
Myasthenia gravis (MG) is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. Although myasthenia gravis may affect any voluntary muscle, muscles that control eye and eyelid movement, facial expression, and...
Act II lit a fire under me regarding my highly opinionated (and of course correct :-) ) views of the state of diagnosis and reporting of MG testing and treatment. So I responded to the article in the Cleveland Journal. It was published this month without revision. I will post my letter here. There was a reply which essentially said they believed what they reported and are afraid to treat patients without serological or positive muscle testing. It's up to us essentially to take the initiative, point out the fallibility and assume the risk of taking the drugs we need. Not something that surprises any of us, especially those of us who are seronegative to their testing. Let's hope this will elicit some response.
This is the link to the journal (thanks Sherry):
http://m.ccjm.org/content/current
TO THE EDITOR: Dr. Li and colleages provide a well-written article about what is generally believed regarding myasthenia gravis (MG). However, like most reviews, it perpetuates the myths surrounding current medical practice, resulting in delays in diagnosis, treatment initiation, and insurance approval and reimbursement, and therefore increased morbidity and mortality. Stricter statistical and editorial review is needed and what is known and unknown clearly stated. Patients, in particular those of us who are physicians ourselves, recognize that this is no academic quibble.
Myasthenia gravis was a clinical diagnosis until blood tests began to pick up antibodies. If the blood tests have to be positive to diagnose MG, then everyone diagnosed with MG will have positive blood tests. If the muscle studies have to show particular abnormalities to diagnose MG, then everyone diagnosed with MG will have those abnormalities. It makes doctors more comfortable to have these evidences of their understanding verified, but it does not help any of the patients who do not meet the testing criteria but have the clinical findings.
We know, in contrast to what was thought a number of years ago, that there are seronegative patients with clinical evidence of myasthenia who are antibody-positive. For those who are MuSK-positive, their problem is now well described, and although it affects a different part of the neuromuscular junction, it remains under the MG umbrella. We also know there are other antibodies, for which we have no commercial tests, in patients with symptoms of MG who respond to treatment for autoimmune problems. This article is relatively dismissive of the clinical validity of those antibodies, and certainly a degree of skepticism is a good thing as long as the patients remain diagnosed and treated.
It is of more than academic interest that these misconceptions and prejudices be recognized. At the very least, editorial boards should insist that statistics in papers reflect the diagnostic skills of the authors. If over 95% of an authors diagnosed patients are seropositive, then one can suspect there is heavy reliance on blood studies for diagnosis, and rejection of those who do not meet those criteria. The statistics should read over 95% of patients we diagnose with MG have positive blood studies rather than over 95% of patients with MG have positive blood studies. Dismissing a significant portion of a patient population will also affect treatment statistics, which then should read that for those who meet this criteria, __% will respond to
If patients meet clinical criteria for the diagnosis of MG and a large percentage do not have positive serology, then more research needs to be done into their particular autoimmune problems, and better testing may become commercially viable. Recognizing the problem will lead to better clinical diagnosis and treatment, and strict diagnostic criteria would permit their inclusion in studies. For many of us this would create a more open and questioning atmosphere as to our understanding of the spectrum of autoimmune myasthenia and the ability and willingness to diagnose and treat seronegative autoimmune myasthenia when we see it.
Copyright 2014 The Cleveland Clinic Foundation
doi: 10.3949/ccjm.81c.03003 Cleveland Clinic Journal of Medicine March 2014 vol. 81 3 144-145
This is the link to the journal (thanks Sherry):
http://m.ccjm.org/content/current
TO THE EDITOR: Dr. Li and colleages provide a well-written article about what is generally believed regarding myasthenia gravis (MG). However, like most reviews, it perpetuates the myths surrounding current medical practice, resulting in delays in diagnosis, treatment initiation, and insurance approval and reimbursement, and therefore increased morbidity and mortality. Stricter statistical and editorial review is needed and what is known and unknown clearly stated. Patients, in particular those of us who are physicians ourselves, recognize that this is no academic quibble.
Myasthenia gravis was a clinical diagnosis until blood tests began to pick up antibodies. If the blood tests have to be positive to diagnose MG, then everyone diagnosed with MG will have positive blood tests. If the muscle studies have to show particular abnormalities to diagnose MG, then everyone diagnosed with MG will have those abnormalities. It makes doctors more comfortable to have these evidences of their understanding verified, but it does not help any of the patients who do not meet the testing criteria but have the clinical findings.
We know, in contrast to what was thought a number of years ago, that there are seronegative patients with clinical evidence of myasthenia who are antibody-positive. For those who are MuSK-positive, their problem is now well described, and although it affects a different part of the neuromuscular junction, it remains under the MG umbrella. We also know there are other antibodies, for which we have no commercial tests, in patients with symptoms of MG who respond to treatment for autoimmune problems. This article is relatively dismissive of the clinical validity of those antibodies, and certainly a degree of skepticism is a good thing as long as the patients remain diagnosed and treated.
It is of more than academic interest that these misconceptions and prejudices be recognized. At the very least, editorial boards should insist that statistics in papers reflect the diagnostic skills of the authors. If over 95% of an authors diagnosed patients are seropositive, then one can suspect there is heavy reliance on blood studies for diagnosis, and rejection of those who do not meet those criteria. The statistics should read over 95% of patients we diagnose with MG have positive blood studies rather than over 95% of patients with MG have positive blood studies. Dismissing a significant portion of a patient population will also affect treatment statistics, which then should read that for those who meet this criteria, __% will respond to
If patients meet clinical criteria for the diagnosis of MG and a large percentage do not have positive serology, then more research needs to be done into their particular autoimmune problems, and better testing may become commercially viable. Recognizing the problem will lead to better clinical diagnosis and treatment, and strict diagnostic criteria would permit their inclusion in studies. For many of us this would create a more open and questioning atmosphere as to our understanding of the spectrum of autoimmune myasthenia and the ability and willingness to diagnose and treat seronegative autoimmune myasthenia when we see it.
Copyright 2014 The Cleveland Clinic Foundation
doi: 10.3949/ccjm.81c.03003 Cleveland Clinic Journal of Medicine March 2014 vol. 81 3 144-145
Still, a MD at a teaching hospital doubts my MG status due to a normal SFEMG and RNS test. Sigh. I'm so thankful for my original neuro who
understood that not all MG is seropositive and looked at the whole picture. I would love to have more evidence for MG before taking the immunosuppressant drugs. I am holding off for now and just doing the Mestinon (which helps). Thank you for writing these folks on our behalf! Way to go!!
Carly
Until they test to uncover, not disprove MG, the results are going to be nothing like 99%. b.
Thank you so much!
What a great service to the MG Community!
- Ross
(another MG patient - who passed all EMG/SFEMG tests.)