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DEPARTMENTS OF LABOR AND HEALTH,EDUCATION,
SUBCOMMITTEE OF THE
SUBCOMMITTEE ON DEPARTMENTS OF LABOR AND HEALTH, EDUCATION, AND
JOHN E. FOGARTY, Rhode Island, Chairman
FRED MARSHALL, Minnesota JOHN TABER, New York
COMMITTEE ON APPROPRIATIONS CLARENCE CANNON, Missouri, Chairman
GEORGE H. MAHON, Texas
JOHN TABER, New York
KENNETH SPRANKLE, Clerk and Staff Director
DEPARTMENTS OF LABOR AND HEALTH, EDUCATION,
TUESDAY, FEBRUARY 25, 1958.
RESEARCH on NEUROLOGICAL DISEASES AND BLINDNESS
DR. H. HOUSTON MERRITT, PROFESSOR OF NEUROLOGY AT COLUM-
Just for the purposes of the record, tell us who you are and what
STATEMENT OF DR. H. HOUSTON MERRITT
Dr. MERRITT. Yes, sir. Mr. Fogarty and members of the committee,
of these lay organizations, and I am happy to come here to give you
National Association for Retarded Children, American Academy of Neurology, American Neurological Association. I am here to speak in behalf of the 1959 appropriations for the National Institute of Neurological Diseases and Blindness. As you know, the National Committee for Research in Neurological Disorders was organized in 1952 to assist the director of the National Institute of Neurological Diseases and Blindness in formulating and coordinating a national research program in this field. The membership of the national committee consists of 2 professional societies, and 9 voluntary agencies, each of which is dedicated to research in a neurological or sensory disorder. I have already listed the constituent members of the national committee. As you know, the national committee each year studies carefully the research and training needs of the Neurology and Blindness Institute and develops a citizens budget which it believes reflects accurately the requirements of the Institute for the coming fiscal year. I know that Dr. Pearce Bailey has appeared before you and has given you a careful review of program developments and research achievements made possible by the Institute during the current year. I shall, therefore, confine my remarks to a few of the key research trends, critical areas in the Institute's program, and the Institute's needs for the coming fiscal year. I will, of course, be only to glad to answer any questions you may
ask on any part of the program. First, T would like to speak about some of the advances in basic
research on the brain. At this time there is a unique opportunity for the study of abnormalities of the composition of the brain which exists in association with some of the neurological and sensory disorders of infancy and childhood. I would like to introduce this by telling how one of our brain disorders was discovered: a disorder of the brain chemistry and how it was discovered and show the importance of this discovery with regard to basic research on the brain. The discovery of phenylketonuria provides an interesting example of the way in which important scientific advances have developed. This story goes back to about 1930. It concerns a family in which two of the children, apparently normal at birth, had developed a severe disturbance of brain function associated with mental retardation. Their distraught mother had become aware of the fact that the urine had a peculiar odor, and that the children themselves seemed to carry this same condition. She went to a number of physicians throughout the continent of Europe asking for help for her children, and begged them to try to find out what the peculiar odor meant. As is so often the case with the parent of a brain damaged child, she went from one physician to another but nowhere could she find help for her children. One of her consultants, when besought by her to do something about the peculiar odor which she had noticed, suggested that this might simply be imaginary and that the strain of her worries was getting to be too much for her.
After many months of fruitless search, she finally went to Professor Folling, a biochemist, and prevailed on him to make a careful analysis of the urine. Dr. Folling carried out the usual tests, and, in addition, tried the use of ferric chloride, a reagent which had been found useful in testing for the presence of a number of unusual urinary compounds. Much to his surprise, he discovered that with the addition of this compound, a bright green color formed. Referring to the work of previous investigators who had studied certain abnormal body compounds, he revealed that this indicated the presence of phenylpyruvic acid in the urine. With the publication of Folling's report in 1934, a number of other families, in which this form of inborn error of body chemistry existed, were discovered. Dr. Jervis in this country, at the Letchworth Village in New York State, discovered that by modifying the diet, one could modify the amount of this abnormal compound which was excreted. These observations led to the suggestion that possibly the brain damage present in these cases was attributable to a toxic effect of the abnormal chemicals present, and that if one could modify their presence, the brain damage might be prevented. Within the past few years, a special diet has been developed which is free of the chemical which these individuals are unable to utilize properly within their bodies. When this chemical is eliminated from the diet, the toxic products do not accumulate, and the intoxication is prevented. The results with this therapy are extremely encouraging. Until this important discovery, children with defects such as these described were considered to be hopeless. They were cared for in isolated State training schools or chronic disease hospitals. The emphasis of our research effort has been toward the evaluation of their mental ability or I. Q., and the psychological or psychiatric aspects of management. The discovery of phenylketonuria has had three important effects: It has led to a hopeful form of therapy for the disease. Even more important, it has provided an important lead to the understanding of some of the basic chemical reactions of the nervous system. Finally, it has encouraged the search for other similar forms of biochemical defects among children and adults with neurological deficits. Already 3 or 4 other similar forms of chemical defect have been discovered. So far, such discoveries have depended upon almost chance observations. The time has come, however, when a systematic and concerted attack on the problem is feasible. Such an attack is possible now because of the discovery of new chemical techniques which make possible the analysis of body fluids for the presence of large numbers of compounds simultaneously. Along with these new techniques, the investigator can screen carefully selected individuals in the search for aberrations of the chemical reactions within their body. With the discovery of such aberrations, there is then opened up a new avenue for the investigation of the bodily reactions which occur in normal and abnormal individuals, and for the discovery of ways of modifying these reactions to provide the most favorable environment for nervous system function in both the normal and abnormal brain.