Dr. Virgil Richard Zarling, of St. Mary's Hospital in Minneapolis, discusses the causes and diagnoses of muscular dystrophy with reporter Dan Olson.
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No, I primarily designation is one where the the significant disease is confined to the muscle. We do treat about 10 or 12 other sub species of neurologic disease which by virtue of the involving the nervous system end up with atrophy our weakness of muscles wasting a message. And so that there is a broader coverage. We also cover the to get is quite well known in my atrophic lateral sclerosis the Lou Gehrig disease. We also cover a disorder called myasthenia gravis, which some spectacular strides have been made him recently. And so that we see a holeRainbow of disease is actually the muscular dystrophy is very much before the public side. Because of course the telephones have been one of the most effective publicity devices perhaps for bringing the problem of muscular dystrophy to people's minds the use of the use of celebrities to ask people to donate money. And of course, I suppose the clinic is one of the groups that gets money from the muscular dystrophy telethons. Is that right? Oh, yes, we benefit from that. We raised a tremendous amount of money over the past years the people in this area been particularly generous and I think we were up around either 8 to 900,000 possibly last year. I don't know the exact figures and now that a certain person goes in our general fund that is defended is used for researching for clinical work taking care of patients directly andThat has amazingly for all those totally see how to be towed boards at the time of the telephone is collected at least 100% I know last year there was at 103 percent of a total actually collected of the party was on the board now getting back to the disease muscular dystrophy. I understand from what I have read about it that it is not one disease, but in fact a family of diseases or a collection of muscle deterioration problems, is that right? Yes, it is fundamentally and hereditary disorder of the the membrane surrounding the muscle cell.And it is Manifest in different forms, and we divide them up and call him the dystrophies because they are all disturbances of growth of muscles these disturbances in the growth of muscles. It's it's hereditary, but how does it manifest itself? In other words? What are the symptoms or are there symptoms that people would notice right away. Yes. The commonest kind is one called duchenne dystrophy after that fellow the first described it back in about a hundred years ago in 1850. And this is a disorder that occurs in boys before the age of 2 and while they have normal birth delivery.99% of them then after about the time they're walking they have begin have difficulty running difficulty climbing steps. They sometimes look very muscular because there's a hypertrophy an overgrowth of the neural muscle cells. So they look very muscular at times and look like very healthy young man Juggernaut and yet they run very awkwardly get you to where they generally recognized that they have a weakness know how many people are affected by these this group of disease is called muscular dystrophy are the dystrophies country. There are I believe in Minnesota about a hundred cases.We generally figure that there are other dacentec the most severe and round tab. I've decided there about 20 to 30 per hundred thousand people dress United States. The interesting thing in this state is it we've kept Google map of where we get our patients from we've had a patient may be recounted some patients from every county in the state except one so that it is a general widespread disorder hearing that there's something more in the Twin Cities because of the population does the reporting of the disease though fault her because it is difficult to identify or is not identified for a long time because people may have a Dystrophy and yet it is it is not known to them. Oh, yes. We see very variations in the form of dystrophy the most severe this to shintech, but there are at least three other types, which is a major categories and which may have varying degrees of severity and maybe just some weakness about the hips are about the shoulders but not a great deal ofOther manifestation and this has been recognized by physicians and we've been functioning for about 15 years now, I believe this is our 16th here and we still find it since command completely undiagnosed and it is a rare thing after all I'm going to say they probably between 4 and 800 cases and there is no cure. There's just therapy is that right? There is no specific cure for the dystrophies themselves at the present time. In fact, we just found out today and got came to agreement among the people around the world that the basic disorder was in the cell membrane before they've been arguing that something came down the nerve and disturb the South now everyone's cell membrane. There has been progress for Francis now in myasthenia gravis, what you going to subsections, we now are curing it as opposed to just helping it along as we did for the previous 100 years.Now found it. It's an immune disorder and that in the transmission from the nurse out to the muscle that there's a there's no receptor of the impulse on the muscle surface from the nerve and it did its immune disorder that is its insert an allergy the wipes out the receptors and now we can either wash the plasma or give them anti allergy types of medication and the patient gets well so that there has been progress as far as a specific thing is happening right now. Is there being split off into various X doctor and Durango as down to Mayo Clinic on the Grant from these funds has identified at least two new types of muscle. What should I do to an enzyme and which is in diminished amounts in the muscle and by feeding last two young children?That's not a 100% cure but at least the disorder can be modified. Why has muscular dystrophy. Why is it taking so long to diagnose MD while essentially to get to its roots? Why is it such a mysterious disease? It's been mysterious because we haven't had the tools. It was only since 1950 that there was a Revival of interest and muscle diseases. Most better than men at the National Institutes of Health and they did develop a technique of staining of muscle fibers so that we could not recognize the different types within the muscle and do what a comment about Mike Staines so they could different colors a little slide and tell which group of muscle fibers are Disturbed and make the diagnosis more accurately than two. We do have to test where we can pick up an enzyme in the bloodstream called creatinine phosphokinase CPK do identify who has the disorderSome of the rate that it's progressing and also developed the other identify the other members of the family who have this disorder and who might be carriers of this disorder. So now we can pick up the youngster some forms to decide who would not be suspected and this is just become available positions within the last 10 years and a very widespread basis, but let's talk about family histories. How much can they tell us about who may be prone to muscle deterioration the a great deal about this and we constantly use genetic histories The Descent type of dystrophy is the probably the most interesting it's what's called a sex linked disorder. That is it is transmitted to boys from their mothers.And only in that pattern but half of that boy's sisters will be carriers know they will be carriers. They will not contract muscle deterioration dystrophy. That's right. They will not show any evidence of the disease though. They may have occasionally a little enlarged from the Cavs. All right. What is the percentage chance of those sisters were carriers passing on dystrophy to their male children? Well, it will be a 50-50 chance with each birth and each one of her daughters will also have a 50-50 chance. But again the woman the daughter will be a carrier the dye will be a carrier. What are the series dr. Zarling as to why this genetic change has taken place in some people so that they are transmitters of muscular dystrophy.Well, they serious that they I guess the best way to explain it would be that they simply hand down from one family to another. A stencil of the way proteins are going to be formed and somehow others are other that stands one of those stencil pictures. You got a hole in it. And when the muscle is made it's nearly complete, but it's got a leak and essential enzymes leak out energy chemicals. What is a picket fence? That's the fence around the muscle cell and their few slots missing so that whatever is inside can get out.