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 vocational you will hear what may seem like strange or unfamiliar sounds. These are the sounds of the participants office laboratory or clinic where the interviews were first conducted. The people you will hear today are Dr. Hans H. 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 of 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. Let's start. We do not know what is the cause of multiple sclerosis. Why does it occur. In the age group which should be might call early 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. RB 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 on or are supported by lay people and by the American public. As specially such 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 literature sure 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 is 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 and 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 do 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 hosier Parkinsonism. Diseases of muscle particularly my senior Gravis is one that to me the weakness on exercise. And disease is one in

which there is a search for newer and better drugs and they have the. This is that an important area. Statistically of course the cerebral vascular disease is so that your accidents. Are. Of great importance. I think I have one just a. Moment thinks about the important people and there are times in the world who have been afflicted with these things rather well known that Sir Winston Churchill had the correction and our own president had several bills from both of those two years ago. The Pope Pius the 12th either the best or artery from both of the refrain when the Roosevelt died of a cerebral haemorrhage and Joseph Stalin was reported to have died of a cerebral haemorrhage so that I think this is a roster of important people in recent years who have had cerebral vascular disease in

either Some of them have beautifully recovered his 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 a bacterial meningitis and so on. However there is always the threat of an outbreak and so the lightest and on the road to sear 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 encephalitis. The outbreak for example in New Jersey did you last month going and so full of life and those of these kinds of infections can always break loose. And these are of course disturbing and I think that some of these diseases that are occurring rather episodically and less commonly and require little we 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 as been concerning muscular dystrophy. Well I think that's. Hard question to answer. Because actually we're almost. Where we were a number of years ago or guard to actual treatment of muscular dystrophy which is commonly regarded as being. More or less and curable disease. Of course if we thought it was incurable and would always remain so there 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. Found and 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 in the very sophisticated sense realizing that nutrition is not just for the body as a whole but it is the nutrition for all the cells in the body. Cellular 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 they 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 for 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 only 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 art 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 back to 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 disease primarily effects childrens but may and does affect

adults. The. 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 fatally 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 atrophies and in terms of education of this become one of our difficult differential diagnostic areas in which there is a misunderstanding often times even occasion in the medical profession what the difference between atrophy and a dystrophy. When the dystrophy is a progressive loss of the basic protein in the muscle cell it's cell. Phone atrophy and practically all instances is due to a failure of transmission of nervous impulse results. Definitively in the contraction or relaxation of the muscle. Sunday and the admin atrophy then the muscle cell itself just gradually dries up and becoming nothing but a fibrous cord and dystrophy the loss of the tissue. Is in the.

Protein of the muscle cell itself and 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 factor in the dystrophy the failure is only in the last of the basic proteins 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 in 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 my. Entire. 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 mid 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 cells 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 are the 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 dystrophy Excel. 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 despotic 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. That's one reason why there is so much effort being spent and so much. Research time requires 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 denominators but the single. Greatest problem is a knowledge of what happens precisely to. A muscle protein itself. There are certain basic enzymatic processes that are involved in the since this is another protein that is utilized by the muscle cell itself. And there are sort of a mom of the cur. As far as a breakdown of the end products of the metabolism of the that occurs within the muscle cell. Every just don't know what the answers are in reference to the difference between. Those processes that occur in the normal muscle and in this graphic 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. We 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. There's of course as this is a very very grave problem you're dealing of course with a. Disease is progressive. And life is obviously shortened considerably. By the progress of it is 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 appear among adults the ratio approximately three quarters of the total patient known and observed are among the

children about one fourth among the adult. Now when you're dealing with. A problem has a very poor outlook as far as along generally 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 the patient itself. 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 what 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 as well as the parent problem that had to be taken into consideration. The day to day problems of. Getting the child. Out of bed and play 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 comes almost obvious that. Your child Amol be to be involved in a serious and progressive fatal disease. There are many questions that you would 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 the 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 is that 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 why is this have to happen to me number one. Number two there's the ego. Centric. Support 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 What is this or

that disease hereditary. What about muscular dystrophy. Yes there is a very definite relationship here. They. 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. Vary of children. This is manifested as a recess of characteristic. Affecting. Male children that she might bear according to the men Day in law. Dominance recesses in the appliance 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. Be. A. Fourth. I. Probably. Would carry or Force One. Might. I actually develop the disease. And of course there are exceptions to this. This does not apply as 100 percent but we have definite case records of families where the. Portion of male children who have. Mostly dystrophy are in excess of a recessive formula. It'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 NEA E.B. Radio Network.