24 страница. We are concerned to know the percentage in any typical colony of our modern civilization that may be placed in the various classifications of normal

FIG. 124. This is a typical mongoloid defective. Note the marked lack of development of the middle third of the face and nose with the upper arch too small for the lower. Individuals of this type look alike and act alike and all have typical speech and behavior defects. These are now associated with definite defects in the brain. Nearly all are either a first or last child. A large percentage are born to mothers over forty years of age.

We are concerned to know the percentage in any typical colony of our modern civilization that may be placed in the various classifications of normal, mildly backward mentally, severely backward mentally, unsocial, delinquent, criminal, idiots, epileptic and insane. Tredgold (2) reports two surveys in England and Wales which give figures on the proportion of the population that could be identified with definite lesions. It would be fortunate if a survey could be made in the United States that would indicate the extent of the increase in delinquents of various types, including racketeers and criminals. It would be very helpful if these data could be related to the degrees of prenatal injury. There are many phases of modern degeneration which lend themselves to study from the standpoint of the probable role of progressive decline in the efficiency of nutrition to the progressive increase in morbidity, mortality, mental deficiency and delinquency. This is discussed in the next chapter on Soil Depletion, Plant and Animal Deterioration.

From the point of view of this problem the differences between the modern white civilizations and many of the primitive groups is interesting. Criminal tendencies in isolated primitives are so slight that no prisons are required. I have referred to the Loetschental Valley in Switzerland, which, until recently, has been physically isolated from the process of modernization. For the two thousand inhabitants in that valley, there is no prison. In Uganda, Africa, the Ruanda tribes estimated to number two and a half millions, had no prisons.

Observation of Nature's normal facial patterns in the primitive racial stocks, establishes types within the limits of normality. The readers of this text by observing the individuals in any given families may see in how large a percentage of white families the progressive narrowing and lengthening of the face in the younger members of the family as compared with the older occurs. Further observations will enable one to recognize rapidly, even without experience and special training in anatomy, these evidences of prenatal injury.

I made a survey in the New England States, Quebec and Eastern Ontario, because the United States death rate from heart disease as reported by the American Heart Association was shown to be highest in Vermont and New Hampshire, followed closely by Massachusetts and New York. I first visited the New York State Hospital for tuberculosis patients at Raybrook, near Saranac. With the assistance of a member of the staff, I examined fifty young men and women in the wards. In that group, only three were found with normal facial and dental arch development. These three individuals were marble cutters who were suffering from silicosis. The forty-seven other individuals examined (94 per cent) were found to have marked evidence of injury in the developmental period. At the state fair at Rutland, Vermont, to which residents came from various communities throughout the state, I was able to count, by observing and recording the individuals who passed, that in each 100 people, three out of four gave evidence of injury in the developmental period. Similar studies were made at the State Farm for delinquent boys and girls, almost all of whom had been before the courts. 1 found that a very high percentage, approximating 100 per cent of those observed, had received injuries in the prenatal period. I then went to Quebec and studied groups of school children in the early teens. I observed groups in which a very high percentage gave marked evidence of prenatal injury. This seemed aggravated in districts where the farms had been abandoned, because the land was not producing as well as in the past. I studied Indians in two Indian Reservations, also, and there again found marked evidence of injury typical of our modernized communities. Similarly, a limestone district in Ontario was visited and critical observations were made of the facial form of the new generation, in regions in which the fertility of the soil had been definitely depleted through exhaustion. These again showed evidence of prenatal injury through faulty nutrition. The prisoners in a jail were examined, and all of them except two habitual drunkards showed marked evidence of prenatal injury.

If space permitted, it would be interesting to include here a discussion and illustrations of the physical characteristics of the racketeers and criminals whose pictures are shown in our newspapers almost daily. It is rare that a normal face is depicted in this group.

As an approach to more detailed study of the available information regarding the processes that are involved in the production of facial deformities, it will be helpful to think of the face as constituting the floor of the anterior part of the brain. The pituitary body is situated on the underside of the brain just back of the eyes. It is the governing body for the activity of growth, and largely controls the functioning of several of the other glands of internal secretion. It is, as it were, the master of the ship. We are, accordingly, primarily concerned with the role that it plays, and the forces which control its own development and function. Its dependence upon vitamin E has been demonstrated by many workers. For example, Dr. M. M. O. Barrie (3) has reported that an inadequate amount of vitamin E produces marked disturbance in the growth of the offspring of rats. He states:

The changes observed are similar in several ways to those produced by hypophysectomy (removal of pituitary gland). Marked degranulation of the anterior pituitary is found in both the abnormal young and the adult sterile animals. Lack of vitamin E therefore produces a virtual nutritional hypophysectomy in the young rat.

The work recently done in this field by Dr. Hector Mortimer and his associates in McGill University, Montreal, has included studies of skull development of rats. He has shown that the surgical removal of the pituitary body at the base of the brain in very young rats produces regularly a certain type of defect in skull development. This has been characterized by a lack of development forward of the muzzle or face, with a narrowing of the nose and dental arches. He found that by the addition of extracts made from the pituitary glands, which he had removed surgically, he entirely prevented the development of these defects, thereby establishing the relation of the injury to deficiencies of the hormones developed by that organ. Another approach to the problem on which he has expended much fruitful effort, has been in connection with the study of the skulls of individuals who are known to have disturbances in the functioning of the pituitary gland through the interference caused by tumors. Common illustrations are the cases of acromegaly or giantism. By associating these physical changes in bodily form with each x-ray, data obtained from skiagraphs, together with the history and the nature of the tumor, considerable information has been developed. Another important series of studies has included the correlation, by means of the x-rays, of the skulls of individuals suffering from certain types of physical and mental disturbances, with certain abnormalities in the skull as shown by the x-rays. By these various means Dr. Mortimer has been able to divide the various types of skull defects and developmental and growth defects into distinct classifications. With this yardstick he is able to classify individuals from their Roentgenograms. It is of interest that in his work, in association with Dr. G. Levine, Dr. A. W. Rowe and others at the Evans Memorial for Clinical Research and Neuro-Endocrine Research in Boston, important relationships have been established through the examination of over three thousand case histories. X-ray records of the skull are included in the studies. They report that independent and previous physiological investigations gave evidence at the time of the examination of disturbed pituitary function. Dr. Mortimer's excellent investigations seem to indicate clearly that facial and dental arch form are directly related to and controlled by the functioning of the pituitary body in the base of the brain. Dr. Barrie (3) reports that partial deficiency of vitamin E, as shown in the case of the female rat, results in the prolongation of gestation which may be continued as long as ten days beyond the normal period. The offspring under these conditions are abnormal. Further, animals deficient in vitamin E, occasionally give birth to a litter, but fail to lactate.

When we realize that one of the best sources of vitamin E is wheat germ, most of which is removed from white flour, usually along with four-fifths of the mineral, we see one cause of the tragedy that is overwhelming so many individuals in our modern civilization. In many individuals it may be wise to reinforce our modern white bread and starchy dietary with wheat germ, which can be obtained in package form from the manufacturers of flour. As this is put up in cans, all air is displaced with an inert gas when the cans are sealed. While in this way oxidation of the embryo which is very fragile, is prevented, as soon as the seal is broken, oxidation sets in and progresses rapidly, producing a product that is not comparable to the wheat embryo of freshly cracked whole wheat. My investigations indicate that Nature has put just the right amount of embryo in each grain of wheat to accompany that quantity of food. If the whole wheat is prepared and eaten promptly after grinding and exposing the embryo to oxidation, the effect desired by Nature is adequately provided.

It is important to emphasize in connection with the development of the deformities of the face that other skeletal deficiencies or abnormalities result from the same disturbing factors. One of these is the narrowing of the entire body, with a tendency to increase in height. This is shown in many of the family groups of modernized primitives. The effect of this narrowing of the body, which in girls results in the boyish type of figure due to the narrowing of the hips, introduces an entirely new and serious problem in the experience of our modern civilization when confronted with the problems of childbirth.

Among primitive races living in a primitive state childbirth was a very simple and rapid process, accompanied by little fear or apprehension; whereas, in the modernized descendants, even in the first and second generations of those individuals born to parents after they had adopted the foods of modern white civilizations, serious trouble was often experienced.

We have been considering the changes which take place in the skeletal growth as a result of the disturbances in the functioning of the pituitary body of the individual after birth, or of the mother during the prenatal period. We are also concerned with changes in the soft tissues, particularly the brain. I have presented data indicating that a very large percentage of mentally backward children have disturbances in facial development. The available data also indicate that a large percentage of those who are seriously injured in facial form have some disturbance in their mental or moral character. Whether there is relationship between the processes which develop these physical abnormalities in brain growth and mental efficiency, including emotional states and character traits is now to be considered.

An important contribution has been made to this phase of the problem by Dr. James Papez, Professor of Anatomy at Cornell University, (4) who concludes his report:

Is emotion a magic product, or is it a physiologic process which depends on an anatomic mechanism?... The evidence presented is... suggestive of such a mechanism as a unit within the larger architectural mosaic of the brain.

Research data have been presented which deal with the anatomical defects of the brain of individuals suffering from the typical mental and physical patterns of the so-called Mongolian idiot. In these cases the gyrus cinguli of the brain were found to be absent, which indicates the impossibility that these individuals function normally either physically or mentally.

Modern civilization has provided a large group of the defectives known as Mongolian idiots. They have very definite characteristics both physical and mental. Among the former, one of the most universal expressions is a vacant stare associated with a face that is markedly underdeveloped in the middle third, usually accompanied by narrow nostrils and a narrow upper dental arch.

One of the outstanding characteristics of the group is their inability to develop mentally beyond three to eight years of age. Because of the difficulty of building a character and intelligence level beyond infancy, these unfortunates are housed largely in state institutions for feebleminded. Since the physical picture is similar to that which occurs in a much less severe form in a large number of individuals in our modern civilization, it is important that we study this group in the light of the information that is available with regard to their physical, mental and moral characteristics, and in the light of such information as is available regarding their origin.

The surveys that have been made reveal the fact that nearly all of them are born to mothers more than forty years of age, and apparently at a period of very low efficiency in reproductive capacity. While most of the discussion and literature stress the importance of the age of the mother, some data are now available which throw responsibility also on the paternal side.

Korosi, as reported by Tredgold in Mental Deficiency, came to the conclusion as a result of the investigation of 24,000 unselected individuals that the children of fathers below twenty or above forty years of age are weaker than the children of fathers between these ages. Also, the children of mothers over forty years of age are weaker than those born to mothers below this age. Tredgold presents data connecting defective structures in the brain with certain phases of physical behavior and mental deficiencies. He quotes many authors whose data correspond with his own. Much of this material relates to accounts of incomplete prenatal development of nerve structures in the brain.

We are particularly interested in the origin and the nature of the brain lesions. Penrose, (5) in analyzing the relative etiologic importance of birth order and maternal age in Mongolism presents data obtained from an examination of 224 defectives in which the total number of children in all the families involved was 1,013. Accordingly, in these families approximately 20 per cent were so affected. The average number of children per family was five and one-half. He states:

Mongolian imbeciles are very often born last in a long family. This fact, which was pointed out many years ago by Shuttleworth, has led clinicians to believe that Mongolism is to some extent a product of the exhaustion of maternal reproductive powers due to frequent child bearing.... The conclusion is widely accepted with the reservation that the affected child is not necessarily born at the end of the family. Several cases are first-born, in fact, and it is sometimes stated that the conditions occur more frequently in first and last children than in other ordinal positions. There is, however, ample evidence that Mongolian imbeciles have a significantly later birth rank than normal children.

G. Ordahl (6) has reported on a study of ninety-one cases in which he found that fifty-six or 60 per cent were the last born. The families averaged five children. He states that "uterine exhaustion is the most commonly advanced reason for Mongolism." Madge T. Macklin (7) says, "It is usually stated that it (Mongolism) occurs more frequently in the later pregnancies owing either to reproductive exhaustion or to too advanced age of the mother." We are concerned at this point for evidence that will throw light on the relationship between the functioning of the pituitary body in the base of the brain and the development of this type of facial and mental deformity.

A striking case is that of a boy sixteen years of age who was a typical Mongolian idiot. He had two sisters who were much older than he. His mother was a partial invalid when he was born late in her life. We have no data relative to the details of the children who may have been lost. His father was living and well except for a railroad injury. This boy at the age of sixteen was infantile in many of his characteristics and developments. The genitals were those of a boy eight years old. The facial expression was that of the typical Mongolian idiot. By the Binet test he had a mentality of about four years. Roentgenograms of his hands showed that the epiphyseal bones had not united. He played on the floor with blocks and with rattles like a child. His interest was in children's activities. (Fig. 125.) The outstanding physical characteristic was his maxillary arch which was so much smaller than the mandibular arch that it went entirely inside it. In order to give him a masticating surface, and with the hope of helping him both physically and mentally, since several cases had greatly benefited by such an operation, I determined to widen his arch by moving the maxillary bones apart about one-half inch. The position of his teeth before the moving of the bones is shown in Fig. 126. Roentgenograms showing the opening of the median suture with increase of pressure are also shown in Fig. 126. An important phase of this case was that the left nostril was entirely occluded, and probably had been all his life. A rhinologist spent half an hour trying to shrink the tissue with adrenalin and cocain sufficiently to get air or water through, and was not able to do so. The quantity of air that he was able to inhale through his right nostril was so scant that he continually breathed with his mouth open. At night he was forced to lie with something like his coat rolled into a hard ridge and placed under the back of his neck and his head pushed far back to a position that would open his mouth and retain it so, or he would awaken, strangling because of the closing of his mouth.

FIG. 125. These views show physical changes in the mongoloid type due to movement of maxillary bones to stimulate the pituitary gland in base of brain. Left, front and side view before, center, front and side view in thirty days. Right, front and side view six months after. Aged sixteen, infantile before, adolescent after operation. While he improved mentally he was harmless before and a sex pervert after operation and had to be placed in an institution.

FIG. 126. These x-ray pictures show the position of the teeth before operation to move maxillary bones; and progessively, by the dates shown, the widening of the upper arch. In twelve weeks' time the boy passed through adolescence. New bone rapidly filled in the space between the separated bones. The space was retained with a fixed bridge carrying two additional teeth. His mother was nearly fifty when he was born.

With the movement of the maxillary bones laterally, as shown progressively in Fig. 126, there was a very great change in his physical development and mentality. He grew three inches in about four months. His moustache started to grow immediately; and in twelve weeks' time the genitals developed from those of a child to those of a man, and with it a sense of modesty. His mental change was even more marked. The space between the maxillary bones was widened about one-half inch in about thirty days. This lateral pressure on the maxillary bones was accomplished by rigid attachments to the teeth of the two sides of the upper arch. The outward movement of the maxillary bones (which form the roof of the mouth and sides of the nose) by pressure on the temporal bones produced a tension downward on the floor of the anterior part of the brain, thus stimulating the pituitary gland in the base of the brain. In a few weeks' time he passed through stages that usually take several years. At first, he got behind the door to frighten us; later, he put bent pins on chairs to see us jump when we sat down, and finally he became the cause of a policeman's coming to the office from where he was conducting traffic on the corner below to find who it was squirting water on him when his back was turned. He developed a great fondness for calling people over the telephone, wanted to borrow my automobile to take his mother for a drive, and with his arm caressingly about the shoulders of one of the secretaries, invited her to go with him to a dance. All this change developed in about twelve weeks.

A most remarkable event happened in connection with this procedure. He lived in another city, and so, while with me, stayed in a boarding house at a little distance from my office in order that he might have frequent, and almost constant attention. On his return to his home town, his efficiency had increased to such an extent that his mother could send him with the money to the grocery store with the order for the day's groceries, and he could bring back the right change and could tell when it was correct. He could also come alone to me ninety miles by railroad and make two changes of trains and the various transfers on the street cars of the city with accuracy and safety.

He wore an appliance in his mouth to keep the bones in position. This appliance became dislodged; the maxillary bones settled together; immediately, or in a day or two, he lapsed into his old condition of lethargy accompanied by an old trouble, which had frequently been distressing, namely, nausea, this sometimes lasting for twenty-four hours. With the readaptation of the separating appliance and the reconstruction of the retaining appliance, he returned rapidly to his improved condition.

But a new problem had developed. We had changed an infant to a potential man with the impulses of a man, but with the mind of a child. With the change in his physical condition he became a menace in his community as a sex pervert. His mother died and his sister married. It became necessary to have him placed in a state institution for defectives. During the period he was in my care, he had learned to read child stories and newspaper headings, and had spent much time doing so. The changes in his physical appearance are shown in Fig. 125, above, front view, and below, side view. The first picture at the left shows his appearance before the operation; the second, thirty days after; and the last, six months later. The opening produced in the upper arch in front of half an inch was filled by supplying two teeth on a restoration, which at the same time held the maxillary bones in their new position. In six months he had developed whiskers and moustache. The progressive changes in the position of the maxillary bones with the opening of the median suture are shown in Fig. 126, together with the mechanical appliance. In the last view, the restoration carrying the porcelain teeth to fill the space is shown.

A very important contribution to our knowledge of the cause of Mongolism has recently been published by Dr. Clemens E. Benda, (8) Clinical Director of the Wrentham State School, Wrentham, Massachusetts, in association with the Harvard Medical School of Boston. He and his group have approached the problem of Mongolism from two different angles; first, as to determine whether it is accidental, and second, whether it is a unit of symptoms which can be related to more essential alteration. Their studies including careful anatomical studies have been made on the basis of an examination of 125 Mongoloids. He states:

Summarizing our investigations, the pituitary in mongoloids reveals a peculiar and definite pathology. On the basis of fourteen cases we feel justified in emphasizing that in mongolism definite failure of the pituitary development is to be found. Mongolism appears as a hypopituitarism of a specific type, in which the absence or deficiency of basophiles seems to be essential.

The evidence indicates that this severe type of facial and brain injury is related directly to a lowered reproductive capacity of the mother associated with age, since the majority are born to mothers beyond forty years of age, and to an inadequate nutrition of the mother, particularly in vitamin E since this vitamin plays so important a role in the nutrition of the pituitary body.

Important new data have been provided in an analysis of births in the United States in connection with the development of the Mongolian group. Bleyer (9) has reported a study of 2,822 cases. He reports that of the total births in the United States in 1934, of 1,095,939, there were 1,822 reported as Mongoloids. The average age of the mothers of these individuals was forty-one years. He reports data indicating that in the age group of mothers forty to forty-four the chances of the development of a Mongoloid would be seventy-five times as high as normal expectancy, and in the age forty-five to forty-nine the chances are 125 times normal expectancy. In a group of 1,942 Mongoloids, 1,100 or 57 per cent were last children. These data are in keeping with those of several other investigators, and emphasize the problem of depleted reproductive capacity.

The interesting problems involved in the birth of identical twins throw light on the origin of both physical and mental characters. It is a matter of great significance in connection with these studies that anomalies which we can associate with parental deficient nutrition are reproduced in both twins. Important additional light has been thrown on this phase by a family of six pairs of fraternal twins born to the same parents. These are reported by Dr. William W. Greulich, of New Haven. (10) It is significant that nine of these individuals (one of the oldest pair is deceased, and the youngest twins are yet babes in arms) show marked narrowing of the nostrils and lack of development of the middle third of the face, narrowing of the face and tendency to be mouth breathers. Further, the severity of this condition appears to be progressively more severe in the younger pairs of twins, sufficiently grown to show facial development. There is accordingly, evidence here of progressive lowering of reproductive efficiency, and the fact that both individuals are involved similarly has great significance, since they are fraternal twins arising from a single ovum. This seems clearly to relate this disturbance with a deficient germ plasm. Factors that are reproduced in identical twins would include both hereditary characters and those that are produced by a disturbance in environment resulting in an interference with normal hereditary processes. In a case of twins that are not identical, there is significance in the development of similar deformities which are likely to be of acquired origin rather than of hereditary origin. In Fig. 127 is seen a pair of twins. Note that they have similar disturbance in the development of the dental arches with the upper laterals depressed and the cuspids crowded outward in the arch.

FIG. 127. These boys are twins, not identical. Note, however, both have same type of deformity of the dental arch, apparently due to the same cause.

A very important source of information which deals with the relation of disturbances of the physical development of the head and mentality is provided by a study of the members of the teen-age group who are classed as mentally backward. In an examination of a Cleveland school in a colored district that has been set aside largely for boys and girls who are distinctly deficient in their ability to learn, it was disclosed that a very large percentage suffered from gross facial deformities when judged by these standards. Typical individuals in this group are shown in Fig. 128, one is white, lower left. It is clear that these boys were all physically injured in the formative period. Their clinical history indicates that the brain was involved in this disturbed development.

FIG. 128. These four boys are typical of a group of several hundred in a special school for backward children. Practically all showed some evidence of incomplete facial and dental arch development. The range of defects is wide. Blacks are similarly injured as whites. This is inhibited heredity.

One of the problems involved in the development of the group of disturbances having physical and mental expressions, is associated with the sensitiveness of the body during the period of adolescence. Many students of degenerative problems have emphasized various phases of this large problem. Burt" commented: "It is almost as though crime were some contagious disease, to which the constitutionally susceptible were suddenly exposed at puberty, or to which puberty left them peculiarly prone." The age of adolescence is also the period of greatest susceptibility to dental caries. Data derived from chemical studies of the blood and saliva show that in this period of susceptibility to dental caries the supplies of minerals and vitamins are inadequate to meet Nature's demands, and the system borrows minerals from the skeleton to maintain vital processes. Lichtenstein and Brown (12) report data which reveal that educational quotients, like intelligence quotients, fall with increase in age during the years of developing puberty. They show that the educational quotient at nine years of age for the group studied was 100; at eleven years of age, 89; at twelve years, 83; and at thirteen years, 74. The changes in facial and dental arch form, which I have described at length in this volume, develop in this age period also, not as a result of faulty nutrition of the individual but as the result of distortions in the architectural design in the very early part of the formative period. Apparently, they are directly related to qualities in the germ plasm of one or both parents, which result from nutritional defects in the parent before the conception took place, or deficient nutrition of the mother in the early part of the formative period. Case records show that the first signs of delinquency generally make their appearance during these years. The age reported most frequently was that of thirteen.

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