Medical research; Neurological and muscular disease

The following program is produced by the University of Michigan broadcasting service under a grant of aid from the National Educational Television and Radio Center in cooperation with the National Association of educational broadcasters neurological and muscular diseases a program from the series human behavior social and medical research produced by the University of Michigan Broadcasting Service. These programs have been developed from interviews with men and women who have the too often unglamorous job of basic research. Research in medicine the physical sciences social sciences and the behavioral sciences locational you will hear what may seem like strange or unfamiliar sods. These are the silence of the participants office laboratory or clinic where the interviews were first conducted. The people you will hear today are Dr. Hans Reese professor of neurology and psychiatry at the University of Wisconsin and Dr. Francis M. pore ster head of the Department of Neurology also at the University of Wisconsin. Professor Roger Williams Professor of Chemistry at the

University of Texas and Dr. J Morrison Brady the medical director of the Muscular Dystrophy Association of America Incorporated. And my name is Glenn Philips. This is the first of two programs on neurological and muscular diseases. We have chosen muscular dystrophy epilepsy and multiple sclerosis. As the focal point these are not of course the only diseases in the category but time will only allow these brief glimpses. One of the Pioneer men in the field of research upon neurological diseases is Dr. Hans Reece of the University of Wisconsin. I asked him what the most pressing problems are in neurological and muscular disease. The most pressing problems relating to human health. In the field of neurology. Multiple Sclerosis the various muscular diseases. Such as muscular dystrophy. And then the larger

problem. The various irritations known infections of the peripheral nerves due to metabolic disorders such as diabetes or arterial sclerosis and various blood diseases. These in my opinion need concentrated research not only by the clinical physician but also by the research person who is steeped in biological and bio chemical information. Presently we do not know the problem. It's a start. We do not know what is the cause of multiple sclerosis. Why does it occur in the age group which we might call early or late

adolescents 18 20 25. Why do the Muscular Dystrophy occur in children and again multiple sclerosis is rarely seen in that age group of 10 or be No. These are important factors which are presently investigated by the geneticists and here in Wisconsin. Great at to deal of attention is given to the genetic approach of neurological diseases. Since we feel that the genetic influence on our current hereditary disposition is of utmost importance in most of our neurological cases. So if I take approximately 46 different. And accepted clinical entities in neurology I

am quite positive that we are almost up to around twenty eight have definite genetic relationships. This is established presently. These others are still under investigation. The communication of how to get this pressing problem of research before the public is handled to a certain extent by national organisation. Which are conducted or are supported by lay people and by the American public. As specially search organisations like multiple sclerosis and the Muscular Dystrophy if we want to speak about how we can perhaps get a better understanding about the diseases is handled by a so called public

relationship. Organisation which provides the various chapters as they are in the states with literatura and with information coming from the regional office in New York presently. There are other organisations who are working on the same field. How much. No the information should go to the general public. It's very difficult to say. I believe an informed individual who knows what an illness can do to him or to his total family should be perhaps. One of them finest. Dr. Francis Forster also the University of Wisconsin enumerated some of the other pressing neurological diseases.

There are a number of these in passing earlier had mentioned brain tumors. These are not common but they are devastating to the people who are involved. And this of course is in the area of concern for us. Parkinsonism is increasing with the older age group that we have in the average life of every age of the citizens increasing due to the better medical care that is obtained. And as one gets older the incidence of Parkinsonism is increasing. And there is a search for newer and better drugs in this area. There are evaluations of surgical treatment in this area and there is a tremendous amount of activity and interest in trying to help these people with the. Shaking holes there. Parkinson there are some. Diseases of muscle particularly my senior gravitas is one that's the root of the weakness so exercise. That disease

is one in which there is a search for a newer and better drugs and they have the. This is that important area. Statistically of course the cerebral vascular diseases so that your accidents. Are. Of great importance. I think you have one dose of. Her moron thinks about the important people and there are times in the world who have been afflicted with the illness rather well known that Sir Winston Churchill had the direction of our own president and cerebral thrombosis two years ago. Pope Pius the 12th either the best or artery from both the reprint Bundy rolls who died of a cerebral haemorrhage and Joseph darling is reported to have died of a cerebral hemorrhage. Well that this is a roster of important people in recent years who have had cerebral vascular disease

either some of them have beautifully recovered or Winston and President Eisenhower and others this is been a devastating and fatal disease. The infections of the nervous system are less of the threat with the control of infectious diseases such as meningitis or bacterial meningitis and so on. However there is always the threat of an outbreak and so the lightest and are on the road to sick concern whenever there is a new influenza epidemic. The Asiatic influenza A few years ago did not produce any appreciable increase in the incidence of and stuff like that. The outbreak for example in New Jersey just last month corn and stuff. Those are these kind of infections that always break loose and push disturbing and I think that some of these diseases that are. Occurring rather episodically and less commonly and require little require considerable

research attention and funds spent on them because they may break loose at any moment and become much more serious proportion than we are lulled into a little sense of security because they're not happening at the moment with Dr. Reese and Dr. Forster setting the stage for these two programs. We move now to the subject for this program muscular dystrophy Professor Roger Williams of the University of Texas chemistry department said this when I asked what the greatest recent advance has been concerning muscular dystrophy. Well I think that's. Hard question to answer because actually we are almost. Where we were a number of years ago. Guard to actual treatment of muscular dystrophy which is commonly regarded as being more or less and durable. Of course if we thought it was incurable and would be no point

in. Trying to do anything about it. One of the very interesting findings in this area however is the fact that some mice have been. Bred. Which have a disease somewhat similar to human dystrophy and this makes it possible to experiment with these ones. I think in this area actually what we need. Is. Research. Devoted to nutrition. And by this I mean very sophisticated sense realizing that nutrition is not just for the body as the whole body is nutrition for the cells in the body or nutrition if you will. And that taking into account the genetic differences between people

and the fact that for example muscle cells may be malnourished not only because they. Are. Not because the body is not fed the right things possibly but because the right things do not get to the desired destination in the body. And also we need to bear in mind the fact that. Cells can be nourished at various levels of excellence. They may be perfectly. Normal morphologically. And yet by a proper nutrition they may be tremendously stimulated and by my modification the. Nutrition. Of course nutrition is one of the most important and bar mental things that we can do to anybody.

Human beings require among other things they require oxygen. They require water. There require suitable ambient temperature. They require nutrition. There's only one kind of water they need only one kind of temperature and one kind of oxygen. But the tradition this is very very complicated and is one of the most important and bar metal things that we can control. What is a dystrophy. With Doctor aid team Miller director of the Institute on muscle diseases and Dr. J Morrison Brady discussing this subject with me while I visited the Institute for muscle diseases in New York. This answer was given. Dr Brady said this is a group of muscular disorders that have certain common characteristics as a common denominator for all of them. These characteristic consist primarily of an involvement of the

muscle tissue. Only these diseases this group of diseases primarily affects children but may and does affect adults. A basic characteristic that is not at all is a progressive wasting of the muscle tissue itself in the child there is a replacement of a muscle tissue by fat which of course has no value in terms of the function of the child and is called a pseudo hypertrophy type of muscular dystrophy. However the muscle tissue itself. Gradually deleted by virtue of rather complex chemical phenomenon that we do not know too much about these diseases are progressive and terminate over varying periods of time depending upon the rate of progression in the individual patient. In many of the diseases that we know we know them by one name

and there are different forms or kinds of those diseases. Is this true of muscular dystrophy. Again Dr. Brady No it just appears a cost in context applies to muscle and muscle only in contradistinction to atrophy and in terms of education and become one of the difficult differential diagnostic areas which there is a misunderstanding often times even Acadian the medical profession know what the difference between atrophy and a dystrophy. When the dystrophy is a progressive loss of the basic protein in the muscle cell itself. Bone atrophy and practically all instances is due to a failure of transmission of nerve as in Paul's results. Definitively in the contraction or relaxation of the muscle. And in atrophy then the muscle

cell itself just gradually dries up and become a duckling with a fibrous card and dystrophy the loss of the tissue is in the protein of the muscle cell itself only in the muscle cell and the nervous mechanism. That is responsible for the normal behavior of a muscle is entirely intact. So the difference then would be the basic singular difference between a dystrophy and atrophy. Then as a fact that in the dystrophy the failure is only in the last of the basic code genes in the muscle cell to atrophy. All of the mechanisms are. The other mechanisms are involved. So the atrophy of the muscle loss is a secondary manifestation in the dystrophy is a primary phenomenon.

Dr Williams said a moment ago that a tremendous amount of research is being conducted everywhere on this disease. Dr Brady who is also the medical director of the Muscular Dystrophy Association of America Incorporated told me this about what is needed in research. Due to untie or lack of knowledge of all of the even of the normal mechanisms by which the physiology of the individual muscle cell or in which the individual muscle cell is involved. Some of the reason the research has been done even in the field of what happens to the normal muscle cell in the distraught fake patient. This singular common observed phenomena is that of the loss of certain essential proteins in within the muscle cell. This being true then they greatest individual or single challenge in basic research. As to bend efforts

to determine. What actually happens in the normal cell and what does not happen in the despotic cell. And if we knew the mechanism of why there was this deviation occurred there would then be an answer to actually what caused the difference or what was responsible for the difference in the dystopic cell. With all of the research and the needs of research still to be met is it possible to say what might be the most fruitful or promising approach. Dr Brady. The problem of what is the most fruitful and promising approach. Basic research efforts in muscular dystrophy. Of course I think it's an obvious observation. It's an observation that's quite obvious to all of us. One reason why there is so much effort being spent and so much. Research time required is that we don't know the answers. And since we don't know the answers then the efforts along all

lines of approach to the basic cause of this set of diseases. Certainly must have some common denominator but the. Greatest problem is a knowledge of what happens precisely to. A muscle protein itself. There are certain basic enzymatic processes that are involved and they say this is another protein that is utilized by the muscle cell itself. And there are sort of Naam of the curve as far as a breakdown of the products of the metabolism of the that occurs within the muscle cell. And we just don't know what the answers are in reference to the difference between. Those posses that occur in the normal muscle and in the muscle. Therefore as far as a single pinpointing a precise area of interest and effort

it is along the line of what happens to the muscle protein itself until we know more about this behavior will not be in a position actually to provide a precise answer or a really an intelligent lead on the treatment of the disease because of the progressive nature of this disease. And the outlook for cure presently DMN the psychological problems of the patient and the family must be great. Dr. Brady mentions a few of the problems and how they could be handled. This is of course as this is a very very grave problem you're getting. Of course with a disease as progressive and life is obviously shortened considerably by the progress of this disease the most of these patients occur in the pediatric group at least the onset occurs in the pediatric group. There is a certain

number of peer among adults the ratio approximately three quarters of the total patient known and observed are among the children. One fourth among the adult. Now when you're dealing with a problem has a very poor outlook as far as along the patient is concerned and with increasing disability. You have problems as far as a child in the family setting is concerned the. Increased amount of. Attention That must be directed in the proper care of a patient at selves. Or themselves. There has to be a very careful. Evaluation of the effect of the patient on other children in the family. There has to be a very careful. Analysis an explanation to the parents as to what the disease is.

What the picture is going to be when and what the parental responsibilities are. One catastrophe of any kind. Hits any family situation. This involves a patient the parents and it's obvious that there are many imply a parent problem that had to be taken into consideration. The day to day problems of. Gaining a child. Out of bed and playing and answering questions as the whole phenomenon of childhood behavior problems are involved. Since you have a disability of progressive severity. And I mean you place this type of a movie. Problem for the parents. There is a tendency for an immediate sense of guilt for the parents and until the. Disease is

much better understood by the parents. Many many problems arise. I think it becomes almost obvious that your child Amol be to be involved in a serious and progressive fatal disease. There are many questions that you want answered. As Dr. Brady indicated the family does enter into this area of concern. Do they however become a hindrance to the actual treatment of the patient. They can be however. The way this has been worked out which makes I believe an awful lot of sense. Once a family understands their problem and their obligation of the care of the patient within the constellation of the family pattern. Certain mistakes are obvious. For one thing the child is treated. As he should be treated now this is rather a dyke dichotomous statement. I mean with this. There is a tendency sometimes in a disability

to overprotect. The disabled child in a family situation. And it is this can be unfair can be unfair to other siblings as the brothers and sisters may suffer from that as well the attempt as far as the orientation of the patient with other members of the family is concerned as they are they receive that degree and amount of care both qualitatively and quantitatively that they need to. Get along as well as it's possible for them to in terms of the degree of disability they possess at the time of their first scene and this has to be adjusted as they progress. The parents must understand this. The parents must accept this type of. Problem. And without their cooperation and understanding the parents themselves can become very upset.

I think the one thing that happens here that is true of each and every disability. Occurs in a child. As with the first general reaction of the parents is that of hostility to the child. Who is. Obviously ill with a disability that in this instance is related to. Genetic factors. And they feel it. Why is this have to happen to me number one. Number two there's the eagle. Centric. It's a part of the individual that has. Been badly buffeted by having an obviously disabled offspring in the family. In preparing for these programs preliminary discussions were had with Lay individuals in an attempt to find out what questions they might ask of medical

authorities if given the chance. One question that was often asked quads is this or that disease hereditary. What about muscular dystrophy. Yes there is a very definite relationship here. They. Are. This particularly applies. To the pseudo hyper cofee muscular dystrophy. In which. The mother carries the genetic. Weakness. Which does not affect the mother in any way shape or form however. And they. Are bearing of children. This is manifested as a recess of characteristic. Affecting. The male children that she might bear according to the M&A and dominance recesses and applying it as it was such recessive factors. Then supposing the mother for example bears for

child of four boys. One boy will definitely have muscular dystrophy. One boy may not carry or not be involved at all. One boy may or may not. Pay for. Probably I would carry or Force One might actually develop the disease. And of course there are exceptions to this. This does not apply as 100 percent. We have case records of families where the portion of male children who have. Must be dystrophy are in excess of a recessive formula that's applied as a means of projecting what the possibilities of future children might be. U.S. Dr. Han Reese Dr. Francis Forester

Professor Roger Williams and Dr. J Morrison Brady have been discussing neurological and muscular diseases. The first of two programs on this subject. Next week you will hear Dr. Forrester as he discusses epilepsy and multiple sclerosis. On the next program from the series human behavior social and medical research consultant for this program was Dr. Russell diong of the University of Michigan Medical School. Glenn Phillips speaking asking that you join us next week and thanking you for being with us at this time. This program has been produced by the University of Michigan broadcasting service under a grant in aid from the National Educational Television and Radio Center in cooperation with the National Association of educational broadcasters. This is the NRA E.B. Radio Network.