CHAPTER XIV. Degeneracy of the Body

DEGENERACY OF THE BODY

As degeneracy checks the natural course of embryonic development it necessarily finds expression in the body as well as in the skull. One most striking condition is that by which development of the bones enclosing the spinal cord is checked. The spinal cord is at first essentially a notochord as in the lowest types of vertebrates. The structures surrounding the cord are not divided into vertebræ. This condition is permanent in the lancelet. Around the notochord is later formed a species of membrane which protects it, called the perichord. This condition is the second stage of development of the cord and is the permanent condition in the lampreys. Later still the cartilaginous vertebræ develop, and then these ossify at the point in the perichord which is to form a vertebra, bows of dense tissue form which unite behind. In front similar bows form to constitute the bodies of the vertebræ. These bows remain ununited in some of the lower fish and at certain stages in the human embryo. As degeneracy checks the union of the bows of the vertebræ, imperfection, and even absence, of the union occur, which is called spina-bifida (Fig. 96). This condition when complete is rarely compatible with life. In a partial state it is often found among degenerates. The seat of the trouble is frequently covered by an excessive development of hair (hypertrichosis), especially in the small of the back; this, which occurs very frequently in degenerates, resembles the tail which the ancients represented as that of the fauns.

As the vertebræ unite irregularly, deviations or bends of the spine occur very frequently among degenerates. These may be of any of the types known to surgeons. In man the spinal column terminates in two bones. One of those (composed of five vertebræ) begins at eighteen years to unite slowly into a single bone called the sacrum. The bones of the sacral vertebræ form processes similar to those which are formed from the vertebræ of the chest region. These serve to cover the nerves of the sacral region. The bone immediately below the sacrum, called the coccyx, is essentially the representative of the tail in man. At a certain stage of human development, as in the tadpole, the tail disappears, the nine vertebræ forming the coccyx unite together and become a very diminutive bone which loses nearly all vertebral characteristics. Sometimes this bone retains its embryonic peculiarities to such an extent that it simulates in some degenerates a rudimentary tail. Of this many instances are on record. A greater degree of this condition has been found to occur, with comparative frequency, amongst the lowest negro races. In this respect these are below the anthropoid apes, where the tail, considered from the tail standpoint, has degenerated as in man for the benefit of the organism as a whole.

The ribs and breastbone develop from the processes of the vertebræ. It is probable that every vertebra originally had ribs. Traces of these exist in most vertebræ in the human embryo. In man, as a rule, there are but twelve vertebræ which develop true ribs. In degenerates, as in the gibbon, a thirteenth pair sometimes appears. Normally, the two lowest ribs are, however, very imperfectly developed as compared with the other ten, and one is sometimes absent. The rib develops from the ends of the vertebral bows, which, coming in contact with the muscle plates of their own segment of the body, are by the resulting bulging forced to expand, and later come together through the formation of the breastbone. Checking of these conditions produces various deformities of the chest which have been divided into "funnel-shaped" and "dropper" deformities. Frequently the entire chest wall is arrested early in life (Fig. 97). The relation between the muscle plates and the course of development of the chest is illustrated by the fact that an arrest of development of important muscles often coexists with deformities of the chest. The human limbs are developments from the fin-folds as found in fishes and the human embryo. In one of these the fins are divided into four segments. The upper segment contains one long bone, the humerus (or arm bone), or the femur (or thigh bone). The second segment contains two long bones, the radius and ulna (or arm bones), or the tibia and fibula (or leg bones). The third segment consists of nine small bones, the carpals of the wrist or the tarsals of the ankle. The fourth segment consists of five separate digits. These limbs pass through three stages in embryonic development as to their position, which may be designated as amphibian, reptilian, and mammalian. Many of these bones fuse together (carpals and tarsals). The digits have long before the late fish stage been formed of more than one bone. At times this condition persists even after the completion of human embryonic development. Limb anomalies resulting from checks of development causing either excess or arrest of development are far from uncommon among degenerates, but are not so common as anomalies of form and proportion. Among such anomalies may be mentioned joined limbs (symelia), or the more or less complete absence of limbs (ectromelia), or the absence of a peripheric segment (hemimelia), or the complete or partial absence of a central segment (phocomelia). Among the other important degeneracies of the limbs are supernumerary digits. These Annadale classifies as: First, a deficient digit loosely attached to the hand or foot or to another digit. Second, a more or less developed digit free at its extremity and articulating with other bones. Thirdly, a fully developed separate digit. Fourth, a digit united along its whole length with another digit. The first three types have been called polydactylia. The last has been called syndactylia. There is finally a condition in which union between the digits results in the disappearance of some of the fingers and toes (Fig. 98). This condition is called ectrodactylia. Supernumerary digits to the extent of six fingers and six toes are exceedingly common in the families of degenerates. The influence of heredity in this particular has been well demonstrated. The Kelleia family of Malta was one of the earliest reported. The condition may last for five generations, but often disappears on marriage with normal persons outside the community or family. A family of the Arabian Hyabites, named Boldi, confined marriages to their own tribe. They all have twenty-four digits. Children born with a normal number are killed as being the offspring of adultery. The inhabitants of Cycaux, France, till the end of the eighteenth century, had nearly all supernumerary digits either on the hands or feet. Isolated in a mountainous region, they for years intermarried. On communication being opened, they emigrated or married strangers, and sexdigitism vanished. Maupertius reports the case of a German family whose members had twenty-four digits for many generations. One of them refused to acknowledge a normal child. In one instance in the United States supernumerary digits lasted through five generations. A case reported some years ago was the following: The first instance of the appearance of the deformity was in a man, born of a degenerate family in 1752, who had six toes on one foot. His son was born with six toes on one foot, but the daughter was normal. This daughter had five children; among them were a son and daughter, each of whom had six fingers on one hand. The granddaughter had eight children, including one son with six toes on one foot. Another son had two daughters, each having six fingers on each hand, and one daughter having twenty-four digits. This last girl had three children; the son was doubly deformed like his mother, while a second son had six fingers on each hand, the toes being normal. One of the two daughters of the fourth generation (with only the hands affected) had eight children, several of whom were normally developed, but the rest were deformed as follows: One daughter had an osseous thickening at the end of the digits, one son had twenty-four digits, another had twelve fingers, the toes being normal in number.

I have elsewhere cited an instance from Kiernan in which unilateral sexdigitism was found in four generations of Norwegian degenerates.

Not unfrequently polydactylia is associated with the absence or union or decrease in size of bones of the limbs. It happens that the upper or lower extremities may be increased or diminished disproportionately through the body. This disproportion in size of the fingers and toes is exceedingly frequent. Big digits (macrodactylia, Fig. 99) are comparatively rare, and may only involve a supernumerary bone in the thumb. Short digits (microdactylia) are much more frequent. This state may be constituted by the absence of one bone or the union of two bones, or the shortening of metacarpal or metatarsal bones, or the shortness of several phalangeal bones. There may be increased disproportion between the different fingers. The method of determining this is by comparison with the middle finger. This disproportion may vary greatly. Féré is of opinion that shortening of all the fingers constitutes a grave mark of degeneracy. Relative shortness is exceedingly common.

Under the conditions of development of the limbs from the fin-fold, it follows that these may be checked completely so that the condition approximates the earlier development from the fish. On the other hand the large bones of the arm and thigh may be checked while the digits and the two lower bones (radius and ulna, tibia and fibula) go on to full development as do the digits. Sometimes the arms develop completely while the lower extremity remains in the fin-fold state. On the other hand the arms may be checked and remain in the fin-fold state while the legs go on to full development. Sometimes the bones of the arm and forearm are checked while the digits go on to full development. The lower extremities are sometimes fused together. This condition, from its resemblance to the like state in the seal, is called phocomelia, or seal limbs. They are also called sirens, on account of the resemblance to the sirens of mythology.

Other expressions of degeneracy, albeit sometimes secondary, are club-foot and club-hands (Fig. 100). In many instances these are retentions of positions assumed by the limbs of the foetus in the course of evolution, and are therefore, in the adult, expressions of degeneracy. Club-foot was an expression of degeneracy which appeared in Byron, the poet, as a consequence of the degeneracy present in both the Byrons and the Gordons, as Kiernan has shown. Commenting on this condition as found in Byron, F. S. Coolidge, of Chicago, remarks: "Byron undoubtedly suffered from double congenital club-foot, the deformity being worse on the right." While in Coolidge's opinion congenital club-foot unquestionably arises from different causes, it is, however, so frequently an accompaniment of severe forms of mal-development and of congenital brain defects, that there can be no doubt but that imperfect constitutional development is one of its causes. That the deformity with the many limitations which it involves may tend to create morbidness is very likely to be an additional symptom of the degeneracy which, in certain cases, is the underlying cause for the deformity. Dareste, who has studied the club-foot and the club-hand from the standpoint of experimental teratology, finds that in no small number of cases club-foot and club-hand result from checked development. Absence of the kneecap or patella may, as H. N. Moyer has shown, be an expression of degeneracy.

The conditions resultant on checked development may appear in any of the bony or muscular structures. At times muscles checked in development pass on to conditions present in the lower apes. Sometimes the checked development of bones results in artery courses which are present in some of the lower animals. Just above the bend of the elbow in the embryo is an opening through which an artery passes in many quadrupeds. In adult man, as a rule, this has disappeared, but not rarely in degenerates the opening persists with the artery through it.

Hernias, of all varieties, are noticeably hereditary, but what is hereditary is not the rupture, but the laxity of the orifice of the cavity of the abdomen. As descent of the testicles from the abdomen (where they are embryonically in man and normally in many animals) is often delayed and even does not occur in degenerates, hernia of the groin variety is particularly apt to occur in them. These hernias are often found united with defects of the testicles as well as deficiencies of the chest. Deformities of the nose are also especially apt to coexist with these.

Degenerate women frequently have supernumerary milk glands arranged on the abdomen as in some lemurs, while males may have supernumerary breasts of either male or female type. These breasts may be represented by nipples alone. In either sex arrested development of the face, middle ear, and palate often coexists with these supernumerary breasts.

The degeneracies of the body combine so frequently with those of the skull and the brain as to indicate a common origin. Polydactylia is found with almost all the degeneracies of the body. It occurs with all the degeneracies of the eye, from those which are purely atavistic like coloboma to those like retinitis pigmentosa and amauroses, which are atavistic in origin. Hare-lip, cleft palate, and deformities of the jaws and teeth are often found associated with all the bodily degeneracies and the nutritive, intellectual, and moral degeneracies as well. Phocomelia with brain deformity has been found associated with them. Anomalies of the genital organs are also quite frequently associated with these and with finger anomalies. In the subjects of juvenile obesity are frequently associated unstable mentality and will-power, and delayed or precocious sexual maturity. My own observations have shown this condition to be frequently associated with the jaw and teeth degeneracies.

Aside from their general significance as stigmata of degeneracy, anomalies of the external ear have been found frequently associated with mal-development of lungs, kidneys, liver, and intestines. Hidden spina-bifida is often associated with the same anomalies, and not rarely with irregular development of the genital organs of both sexes. Albinism or deficient pigment in the skin and hair is not only often associated with grave degeneracies, intellectual and moral, but appears combined with mal-development of the spleen, liver, and kidneys. The opposite state (melanoderma, or black skin) is often associated with similar deformities, especially with early precocity in development. It has also been found in connection with hairlessness and irregularities in development of the teeth and jaws.

The deformities of the chest and their resulting interferences with respiration are not only associated with conditions like narrowing of the pulmonary artery, of the aorta, and with cardiac deformities, but also lead to diminution of the respiratory power and, in consequence, to conditions predisposing to pneumonia and consumption. They are also, as my own observation has shown, associated with deformities of the face, of the nose, of the mouth cavity, of the palate, and of the jaws. These conditions result in mouth breathing and in other conditions which predispose to the attacks of microbes. The deformities of the arterial system coexisting with checked development of the chest are apt to extend to the blood vessels of the kidneys, and therefore to retain these in the embryonic condition, thus predisposing them to disease. To a somewhat lesser degree this arterial condition coexisting with checked development of the chest or with the other arrested conditions of development associated with it, may extend to the liver in such a degree as to prevent it destroying the toxins of typhoid fever and allied diseases, thereby increasing the dangers from these disorders. The same influence may be exerted on the spleen and suprarenal capsules, thus interfering with the physiologic guards these organs furnish against disease and its results. Not infrequently do these last conditions tend to give an epileptic character to degeneracies which would otherwise be destitute of it. With the interference with the proper blood supply, due to checked conditions of the organs named, may occur gout, diabetes, and many forms of rheumatism with their secondary consequences.

The imperfect and irregular action of the lungs associated with the arrests in development of the face, nose, palate, jaws, and chest may produce an irregular blood supply to the brain, which will exaggerate the mental instability of the degenerate.

Among the conditions which are expressions of degeneracy of the body, combined with degeneracy of the head and face, are three conditions known as infantilism, masculinism, and feminism. Practically all three are arrests of development of the promise of the child type. Owing to the struggle for existence which occurs at puberty between the old type of the chondrocranium and its new type as supplemented by the dermal bones, the nervous system takes a distorted ply which arrests both the bodily, nervous, and mental development at certain points. In infantilism the arrest is of the future promise of the child, so that the body and face remain at the childish point, or the body and nervous system are checked, or finally the nervous system or certain organs alone are checked while the body goes on to full development. Not infrequently the face is arrested at any period from birth to puberty (Fig. 101). Hence the reason many persons retain their youthful appearance throughout life. These people are often vain and egotistic. The mental stamina is weak, and they are frequently unreliable, while the females are often prostitutes or prurient prudes, hysteric reformers or gossip mongers.

The female type, from the standpoint of bodily and nervous development, most nearly approximates the promise of the child type, and checks of development will result in masculinism and feminism. In the first the female has proceeded so far in development as to have female organs and their functions while retaining traces of a predominant character of the lower male type. In the second, the male has proceeded along the line of evolution toward the female type, but ere sex has been fixed, further development has been checked and the male type is finally assumed as the predominant one. Both sexes proceed embryologically from an indifferent type nearly resembling the hermaphroditic type found in the lower vertebrates. The arrest of development may therefore take place at any point in the embryonic evolution. The male may preserve only the female breasts (Fig. 102), while normal in other respects; or, again (Fig. 103), present cryptorchidism or sloping shoulders, and be otherwise masculine. On the other hand, his nervous system may have taken such a ply that at the period of puberty the sexual instincts may be female in type. In some instances, this may extend merely to an extreme modesty toward males, to an intense liking for female occupations and disgust for male occupations. In the female precisely analogous conditions may occur. In certain cases the sex side is entirely dormant until awakened at puberty. Education of these cases of arrested development may give the sex direction rather than any in-born tendency. In one case, a male who had undergone arrest of development in his evolution towards the female type was brought up as a girl, had unusually pleasing womanly qualities; as a result was married twice to intensely devoted husbands, and the real sex was never even suspected until post-mortem examination revealed that the supposed woman was a male. In other cases where the nervous system has taken one sexual ply, while the body has taken another, an exceedingly unfortunate class of beings results. This class of beings needs especially careful training during puberty and adolescence. In some instances in addition to the sexual distortion there exist in these beings conditions of mental defect and moral obliquity. In the last case they approximate the criminal type. In the first case residence in an insane hospital protects the community and these beings against themselves. In some instances no mental defect nor, in a strict sense, moral obliquity occurs. Here the patient requires very careful study from every standpoint. There is in the higher and lower races a tendency in different directions as to the predominance of sex. The woman of the lower races more nearly resembles the male of the race. The male of the higher races more nearly resembles in structure the female of his race than does this female the females of the lower race. This is in part due, in the course of evolution, to the intrusion of the male on female occupations, since all occupations, other than war, hunting, and fishing, were created by women. The male, however, in the higher races, while thus taking on the female intellectual and æsthetic qualities, retains the male sexual characteristics, mentally and physically, but in accordance with the law of evolution, these manifest themselves less explosively as in the case of the female. As the victim of masculinism or feminism is a degenerate the explosive manifestations are more marked. Every one of the stigmata of degeneracy already described may coexist with any one of the three conditions named: infantilism, masculinism, and feminism.

Among the most striking manifestations arising from arrest of development in certain directions with possible hypertrophy in others, are the conditions known as giantism and dwarfism (see Frontispiece). Both these conditions may be expressions of atavism to no very remote ancestors and present little if any evidences of degeneracy. In other cases degeneracy may be well marked, and the condition be due to imperfect gland action, such as disorder of the pituitary body, which causes very frequently an enlargement of many of the bones of the body, and very often a uniform enlargement of all the bones. Indeed, as Marie has said, giantism is acromegaly occurring during the period of adolescence. In many instances the opposite condition, dwarfism, occurs during infancy from causes which check the further growth of the body, although the general functions remain unchanged. Dwarfism is very apt to be attended by preservation of the intellectual faculties without evidence of degeneracy, other than the egotism shown in extreme vanity. Moral defects are, however, more apt to occur in dwarfism than in giantism, in which last condition mental defect is apt to occur, varying from a simple good-humoured stupidity to feeble-mindedness. In proportion as the central nervous system has been affected will the stigmata of degeneracy appear in both conditions. As the line between disease and disordered function is not thinly drawn in these cases, disorders like rickets or local bony tendencies to extensive growth may coexist with both conditions. Infantilism is peculiarly apt to occur with giantism, and while less frequent in dwarfism it also occurs, but is then especially apt to be associated with rickets.

Closely akin to these conditions are leontiasis ossium and acromegaly, both of which are characterised by similar trophoneurotic defects. The first of these conditions may occur precedent to puberty and cease in its completion. Kiernan has observed this in the case of an imbecile on Ward's Island, who lived until the age of 75, after spending more than sixty years in the charitable institutions of New York. His ancestry was of the criminal and defective classes. Acromegaly is characterised by abnormal growth, chiefly in the bones of the head, face, and extremities. As a rule, the disorder begins at the completion of puberty, although it occasionally occurs at the onset of the climacteric. The illustration given (Fig. 104) presents the characteristic features of the disease. In this case there are local evidences of congenital defect. The prevailing trend of opinion is that this condition is due to irregular action of the pituitary body which controls osseous development.

As already pointed out in the chapter on Heredity and Atavism, the arrests of development may affect one side of the body, while the other pursues the direct course of development. This may show itself in over-growth, as well as in under-growth, on the affected side. The person may appear, even from the centre of the forehead down, as if the halves of the bodies of two different persons had been joined in one. Conditions may vary from this extreme type to a state in which a lack of proper nerve and blood supply on the undeveloped side predisposes to attacks of disease, as already pointed out.

Not rarely does it happen that acromegaly attacks the side most deficient in nerve supply. The same is true of allied disorders affecting the growth of the muscles.

The conditions of development may be such that both sides are equally defective in nerve supply, so that when acromegaly occurs it may attack both sides equally. This is particularly apt to be the case with the lower extremities, and enlarged feet (Fig. 105) are not an uncommon result.

The feet, in addition to the condition already described as being common in both extremities, exhibit special stigmata of their own in consonance with the evolutionary advance which the foot has shown as compared with the hand in man's evolution. In the lower races the hollow of the foot does not exist, and the condition known as flat-foot occurs (Fig. 106). This is usually associated with low instep. It occurs among the stigmata of degeneracy, and is not rarely associated with grave moral defects and intellectual distortions. It and other feet degeneracies have been found frequently among paranoiacs, moral imbeciles, and prostitutes. This is particularly true of the prehensile power of the foot.