Volume 9 / Part 2 — B.

NOTES CONCERNING LACKING, DEFECTIVE, AND ARRESTED MENTAL DEVELOPMENT IN THE FIRST YEARS OF LIFE.

The data we have concerning the behavior of children born, living, without head or without brain, and of microcephalous children, as well as of idiots and cretins more advanced in age, are of great interest, as helping us to a knowledge of the dependence of the first psychical processes upon the development of the brain, especially of the cerebral cortex. Unfortunately, these data are scanty and scattered.

Very important, too, for psychogenesis, are reports concerning the physiological condition and activity of children whose mental development has seemed to be stopped for months, or to be made considerably slower, or to be unusually hastened.

Scanty as are the notes I have met with on this matter, after much search, yet I collect and present some of them, in the hope that they will incite to more abundant and more careful observation in the future than has been made up to this time.

A good many data concerning the behavior of cretin children are to be found in the very painstaking book, "Neue Untersuchungen über den Kretinismus oder die Entartung des Menschen in ihren verschiedenen Graden und Formen" ("New Investigations concerning Cretinism, or Human Deterioration, in its Various Forms and Degrees"), by Maffei and Rösch (two vols, Erlangen, 1844). But, in order that these data should be of value, the observed anomalies and defects of the cerebral functions ought to be capable of being referred to careful morphological investigations of the cretin brain. As the authors give no results of post-mortem examinations, I simply refer to their work here.

I once had the opportunity myself of seeing a hemicephalus, living, who was brought to the clinic of my respected colleague, Prof. B. Schultze, in Jena. The child was of the male sex, and was born on the 1st of July, 1883, at noon, along with a perfectly normal twin sister. The parents are of sound condition. I saw the child for the first time on the 3d of July, at two o'clock. I found all the parts of the body, except the head, like those of ordinary children born at the right time. The head had on it a great red lump like a tumor, and came to an end directly over the eyes, going down abruptly behind; but, even if the tumor were supposed to be covered with skin, there would by no means be the natural arched formation of the cranium of a newly-born child. The face, too, absolutely without forehead, was smaller in comparison than the rest of the body. I found now, in the case of this child, already two days old, a remarkably regular breathing, a very cool skin—in the forenoon a specific warmth of 32° C. had been found—and slight mobility. The eyes remained closed. When I opened them, without violence, the pupil was seen to be immobile. It did not react in the least upon the direct light of the sun on either side. The left eye did not move at all, the right made rare, convulsive, lateral movements. The conjunctiva was very much reddened. The child did not react in the least to pricks of a dull needle tried on all parts of the body, and reacted only very feebly to pinches; not at all to sound-stimuli, but regularly to stronger, prolonged cutaneous stimuli; in particular, the child moved its arms after a slap on the back, just like normal new-born children, and uttered very harsh, feeble tones when its back was rubbed. When I put my finger in its mouth vigorous sucking movements began, which induced me to offer the bottle—this had not yet been done. Some cubic centimetres of milk were vigorously swallowed, and soon afterward the breast of a nurse was taken. While this was going on I could feel quite distinctly with my finger, under the chin, the movements of swallowing. It was easy to establish the further fact that my finger, which I laid in the hollow of the child's hand, was frequently clasped firmly by the little fingers, which had well-developed nails. Not unfrequently, sometimes without previous contact, sometimes after it, the tip of the tongue, and even a larger part of the tongue, was thrust out between the lips, and once, when I held the child erect, he plainly gave a prolonged yawn. Finally, the fact seemed to me very noteworthy that, after being taken and held erect, sometimes also without any assignable outward occasion, the child inclined its head forward and turned it vigorously both to the right and to the left. When the child had sucked lustily a few times, it opened both eyes about two millimetres wide, and went on with its nursing. An assistant physician saw the child sneeze.

These observations upon a human child, two days old, unquestionably acephalous, i. e., absolutely without cerebrum, but as to the rest of its body not in the least abnormal, prove what I have already advanced (vol. i, p. 203), that the cerebrum takes no part at all in the first movements of the newly-born. In this respect the extremely rare case of an acephalous child, living for some days, supplies the place of an experiment of vivisection. Unfortunately, the child died so early that I could not carry on further observations and experiments. The report of the post-mortem examination will be published by itself.

Every observer of young children knows the great variety in the rapidity of their development, and will agree with me in general that a slow and steady development of the cerebral functions in the first four years, but especially in the first two years, justifies a more favorable prognosis than does a very hasty and unsteady development; but when during that period of time there occurs a complete and prolonged interruption of the mental development, then the danger is always great that the normal course will not be resumed. So much the more instructive, therefore, are the cases in which the children after such a standstill have come back to the normal condition. Four observations of this kind have been published by R. Demme ("19. Bericht über das Jenner'sche Kinderspital in Bern, 1882," S. 31 bis 52). These are of so great interest in their bearing on psychogenesis, and they confirm in so striking a manner some of the propositions laid down by me in this book, that I should like to print them here word for word, especially as the original does not appear to have found a wide circulation; but that would make my book altogether too large. I confine myself, therefore, to this reference, with the request that further cases of partial or total interruption of mental development during the first year of life, with a later progress in it, may be collected and made public.

It is only in rare cases that microcephalous children can be observed, while living, for any considerable length of time continuously. In this respect a case described by Aeby is particularly instructive.

A microcephalous boy was born of healthy parents—he was their first child—about four weeks too soon. His whole body had something of stiffness and awkwardness. The legs were worse off in this respect than the arms; they showed, as they continued to show up to the time of his death, a tendency to become crossed. The boy was never able to stand or walk. He made attempts to seize striking objects, white or party-colored, but never learned actually to hold anything. The play of feature was animated. The dark eyes, shining and rapidly moving, never lingered long upon one and the same object. The child was much inclined to bite, and always bit very sharply. Mentally there was pronounced imbecility. In spite of his four years the boy never got so far as to produce any articulate sounds whatever. Even simple words like "papa" and "mamma" were beyond his ability. His desire for anything was expressed in inarticulate and not specially expressive tones. His sleep was short and light; he often lay whole nights through with open eyes. He seldom shed tears; his discomfort was manifested chiefly by shrill screaming. He died of pulmonary paralysis at the end of the fourth year.

The autopsy showed that the frontal lobes were surprisingly small, and that there was a partial deficiency of the median longitudinal fissure. The fissure did not begin till beyond the crown of the head, in the region of the occiput. The anterior half of the cerebrum consequently lacked the division into lateral hemispheres. It had few convolutions also, and the smoothness of its surface was at once obvious. The corpus callosum and the fornix were undeveloped. "The gray cortical layer attained in general only about a third of the normal thickness, and was especially weakly represented in the frontal region." The cerebellum not being stunted, seemed, by the side of the greatly shrunken cerebrum, surprisingly large.

In this case the microcephalous of four years behaves, as far as the development of will is concerned, like the normal boy of four months. The latter is, in fact, superior to him in seizing, while the former in no way manifests any advantage in a psychical point of view.

Two cases of microcephaly have been described by Fletcher Beach (in the "Transactions of the International Medical Congress," London, 1881, iii, 615-626).

E. R. was, in May, 1875, received into his institution at the age of eleven years. She had at the time of her birth a small head, and had at no time manifested much intelligence. She could not stand or walk, but was able to move her arms and legs. Her sight and hearing were normal. She was quiet and obedient, and sat most of the time in her chair. She paid no attention to her bodily needs. She could not speak and had to be fed with a spoon. After six months she became a little more intelligent, made an attempt to speak, and muttered something indistinctly. She would stretch out her hand when told to give it, and she recognized with a smile her nurse and the physician. Some four months later she would grind her teeth when in a pleasant mood, and would act as if she were shy when spoken to, holding her hand before her eyes. She was fond of her nurse. Thus there was capacity of observation, there were attention, memory, affection, and some power of voluntary movement. She died in January, 1876. Her brain weighed, two days after her death, seven ounces. It is minutely described by the author—but after it had been preserved in alcohol for six years, and it then weighed only two ounces. The author found a number of convolutions not so far developed as in the fœtus of six months, according to Gratiolet, and he is of opinion that the cerebellum was further developed after the cerebrum had ceased to grow, so that there was not an arrest of the development but an irregularity. The cerebral hemispheres were asymmetrical, the frontal lobes, corresponding to the psychical performances in the case, being relatively pretty large, while the posterior portion of the third convolution on the left side, the island of Reil, and the operculum were very small, corresponding to the inability to learn to speak. The author connects the slight mobility with the smallness of the parietal and frontal ascending convolutions.

The other case is that of a girl of six years (E. H.), who came to the institution in January, 1879, and died in July of the same year. She could walk about, and she had complete control of her limbs. She was cheerful, easy to be amused, and greatly attached to her nurse. She associated with other children, but could not speak a word. Her hearing was good, her habits bad. Although she could pick up objects and play with them, it did not occur to her to feed herself. She could take notice and observe, and could remember certain persons. Her brain weighed, two days after death, 20-1/2 ounces, and was, in many respects, as simple as that of an infant; but, in regard to the convolutions, it was far superior to the brain of a monkey—was superior also to that of E. R. The ascending frontal and parietal convolutions were larger, corresponding to the greater mobility. The third frontal convolution and the island of Reil were small on both sides, corresponding to the alalia. The author is of opinion that the ganglionic cells in this brain lacked processes, so that the intercentral connections did not attain development.

A more accurate description of two brains of microcephali is given by Julius Sander in the "Archiv für Psychiatrie und Nerven-Krankheiten" (i, 299-307; Berlin, 1868), accompanied by good plates. One of these cases is that of which an account is given by Johannes Müller (in the "Medicinische Zeitung des Vereins für Heilkunde in Preussen," 1836, Nr. 2 und 3).

In the full and detailed treatises concerning microcephali by Karl Vogt ("Archiv für Anthropologie," ii, 2, 228) and Von Flesch ("Würzburger Festschrift," ii, 95, 1882) may be found further data in regard to more recent cases.

Many questions of physiological and psychological importance in respect to the capacity of development in cases of imperfectly developed brain are discussed in the "Zeitschrift für das Idioten-Wesen" by W. Schröter (Dresden) and E. Reichelt (Hubertusburg).

But thus far the methods of microscopical investigation of the brain are still so little developed that we can not yet with certainty establish a causal connection, in individual cases, between the deviations of microcephalic brains from the normal brain and the defects of the psychical functions. The number of brains of microcephali that have been examined with reference to this point is very small, although their scientific value, after thorough-going observation of the possessors of them during life, is immense. For microcephalous children of some years of age are a substitute for imaginary, because never practicable, vivisectory experiments, concerning the connection of body and mind.

To conclude these fragments, let me add here some observations concerning a case of rare interest, that of the microcephalous child, Margarethe Becker (born 1869), very well known in Germany. These observations I recorded on the 9th of July, 1877, in Jena, while the child was left free to do what she pleased.

The girl, eight years of age, born, according to the testimony of her father, with the frontal fontanelle (fonticulus anterior) closed and solid, had a smaller head than a child of one year. The notes follow the same order as that of the observations.

Time, 8.15 A. M.—The child yawns. She grasps with animation at some human skulls that she sees on a table near her, and directs her look to charts on the walls. She puts her fingers into her nostrils, brushes her apron with both hands, polishes my watch, which I have offered her and she has seized, holds it to one ear, then to one of her father's ears, draws her mouth into a smile, seems to be pleased by the ticking, holds the watch to her father's other ear, then to her own other ear, laughs, and repeats the experiment several times. Her head is very mobile.

The child now folds a bit of paper that I have given her, rolls it up awkwardly, wrinkling her forehead the while, chews up the paper and laughs aloud. Saliva flows from her mouth almost incessantly. Then the child begins to eat a biscuit, giving some of it, however, to her father and the attendant, putting her biscuit to their lips, and this with accuracy at once, whereas in the former case the watch was held at first near the ear, to the temple, and not till afterward to the ear itself.

The girl is very lively; she strikes about her in a lively manner with her hands, sees charts hanging high on the walls, points to them with her finger, throws her head back upon her neck to see them better, and moves her fingers in the direction of the lines of the diagrams. At last weariness seems to come on. The child puts an arm around the neck of her father, sits on his lap, but is more and more restless.

8.50.—Quiet. To appearance, the child has fallen asleep.

8.55.—Awake again. The child sees well, hears well, smells well; obeys some few commands, e. g., she gives her hand. But with this her intellectual accomplishments are exhausted. She does not utter a word.

Kollmann, who saw this microcephalous subject in September, 1877, writes, among other things, of her ("Correspondenzblatt der Deutschengesellschaft für Anthropologie," Nr. 11, S. 132):

"Her gait is tottering, the movements of the head and extremities jerky, not always co-ordinated, hence unsteady, inappropriate and spasmodic; her look is restless, objects are not definitely fixated. The normal functions of her mind are far inferior to those of a child of four years. The eight-year-old Margaret speaks only the word Mama; no other articulate sound has been learned by her. She makes known her need of food by plaints, by sounds of weeping, and by distortion of countenance; she laughs when presented with something to eat or with toys. It is only within the last two years that she has become cleanly; since then her appetite has improved. Her nutrition has gained, in comparison with the first years of life, and with it her comprehension also; she helps her mother set the table, and brings plates and knives, when requested to do so, from the place where they are kept. Further, she shows a tender sympathy with her microcephalous brother; she takes bread from the table, goes to her brother's bedside and feeds him, as he is not of himself capable of putting food into his mouth. She shows a very manifest liking for her relatives and a fear of strangers. When taken into the parlor she gave the most decided evidences of fear; being placed upon the table she hid her head in her father's coat, and did not become quiet until her mother took her in her arms. This awakening of mental activity shows that, notwithstanding the extremely small quantity of brain-substance, there exists a certain degree of intellectual development with advancing years. With the fourth year, in the case of M., independent movements began; up to that time she lay, as her five-year-old brother still lies, immovable in body and limbs, with the exception of slight bendings and stretchings."

Richard Pott, who (1879) likewise observed this microcephalous subject, found that she wandered about aimlessly, restlessly, and nimbly, from corner to corner [as if], groping and seeking; yet objects held before her were only momentarily fixated, scarcely holding her attention; often she did not once grasp at them. "The girl goes alone, without tottering or staggering, but her locomotive movements are absolutely without motive, having no end or aim, frequently changing their direction. Notwithstanding her size, the child gives the impression of the most extreme helplessness." She was fed, but was not indifferent as to food, seeming to prefer sour to sweet. She would come, indeed, when she was called, but seemed not to understand the words spoken to her; she spoke no word herself, but uttered shrill, inarticulate sounds; she felt shame when she was undressed, hiding her face in her sister's lap. The expression of her countenance was harmless, changeable, manifesting no definite psychical processes.

The statements contradictory to those of Kollmann are probably to be explained by the brevity of the observations.

Virchow ("Correspondenzblatt," S. 135), in his remarks upon this case, says: "I am convinced that every one who observes the microcephalic child will find that psychologically it has nothing whatever of the ape. All the positive faculties and qualities of the ape are wanting here; there is nothing of the psychology of the ape, but only the psychology of an imperfectly developed and deficient little child. Every characteristic is human; every single trait. I had the girl in my room a few months since, for hours together, and occupied myself with her; I never observed anything in her that reminds me even remotely of the psychological conditions of apes. She is a human being, in a low stage of development, but in no way deviating from the nature of humanity."

From these reports it is plain to be seen that for all mental development an hereditary physical growth of the cerebrum is indispensable. If the sensuous impressions experienced anew in each case by each human being, and the original movements, were sufficient without the development of the cerebral convolutions and of the gray cortex, then these microcephalous beings, upon whom the same impressions operated as upon other new-born children, must have had better brains and must have learned more. But the brain, notwithstanding the peripheral impressions received in seeing, hearing, and feeling, could not grow, and so the rudimentary human child could not learn anything, and could not even form the ideas requisite for articulate voluntary movement, or combine these ideas. Only the motor centers of lower rank could be developed.

In peculiar contrast with these cases of genuine microcephaly stands the exceedingly remarkable case, observed by Dr. Rudolf Krause (Hamburg), of a boy whose brain is not at all morbidly affected or abnormally small, but exhibits decidedly the type of the brain of the ape. The discoverer reported upon it to the Anthropological Society ("Correspondenzblatt a.a. O., S. 132-135) the following facts among others:

"The skull and brain belonged to a boy who was born on the 4th of October, 1869, the last of four children. Paul was scrofulous from his youth. He did not get his teeth until the end of his second year, and they were quite brown in color and were soon lost. According to the statement of Paul's mother, he had several successive sets of teeth. It was not until the fifth year that he learned to walk. He was cleanly from the third year, but not when he felt ill. His appetite was always good up to his last sickness of four weeks. His sleep was habitually undisturbed. He was of a cheerful temperament, and inclined to play; as soon as he heard music he would dance, and sing to the music in rather unmelodious tones. When teased he could be very violent; he would throw anything he could lay his hands on at the head of the offender. He liked the company of others, especially of men. By the time he was four years old he had learned to eat without help. Paul was very supple, was fond of climbing, and had great strength in his arms and hands especially; these had actually a horny appearance, and thus reminded one of the hands of the chimpanzee. He could sit on the ground with his legs wide apart. His gait was uncertain, and he was apt to tumble; he ran with knees bent forward and legs crooked; he was fond of hopping, and seemed particularly ape-like when doing so. The great-toe of each foot stood off at an angle from the foot, and thus gave the impression of a prehensile toe. I thought at first that this deviation had its origin in the fact that the child, on account of his uncertainty in walking, wanted to get a broader basis of support; but I afterward gave up that opinion, because I have never found an instance of a similar habit in other children with diseased heads, e. g., hydrocephalous children. Paul could speak but little, could say hardly any words except Papa and Mama, and even these he did not until late learn to pronounce in two syllables; he uttered for the most part only sounds that resembled a grunt. He imitated the barking of a dog by the sound rrrrrr. He frequently stamped with feet and hands, clapped his hands together, and ejaculated a sort of grunting sound, just as I have observed in the case of gorillas and chimpanzees.

"Paul was smaller than children of his age; on his right eye he had from his youth a large leucoma; the eyelids had generally a catarrhal affection, and were in a state of suppuration. The head looked sore; the forehead was small. Paul had a strongly marked tendency to imitation. His whole being, his movements, were strikingly ape-like. He was decidedly neglected by his parents, was generally dirty in appearance, and I really think the early death of the child was induced by the slight care taken of him. Paul was taken sick at the beginning of December, 1876, with an acute bronchial catarrh, and died on the 5th of January, 1877, at the age of seven and a quarter years.

"If you look at the cranium and the brain here, which belonged to the child just described, there are lacking in the first place all the characteristics of microcephaly. The cranium possesses a capacity of 1,022 cubic centimetres, and the brain weighs 950 grammes; they do not deviate, therefore, from the normal condition. But let the cranium, where it is laid open by the saw, be observed from within, and we notice an asymmetry of the two hemispheres of the brain; the cranium is pushed somewhat forward and to the right. The partes orbitales of the frontal bone are higher and more arched than is usual, in consequence of which the lamina cribrosa of the ethmoid bone lies deeper, and room is given for the well-known conformation of the ethmoidal process in the brain. The cerebral convolutions are plainly marked upon the inner surface of the cranium. The facial cranium shows no deviations. There is no prognathism. The formation of the teeth alone is irregular; one pre-molar tooth is lacking above and below in the jaw, and, in fact, there is no place for it. The incisors and the pre-molar teeth are undergoing change.

"The two cerebral hemispheres are asymmetrical; in the region where the parieto-occipital fissure is situated on the left hemisphere, the two hemispheres diverge from each other and form an edge which curves outward and backward, so that the cerebellum remains uncovered. On the lower surface of the frontal lobes there exists a strongly marked ethmoidal prominence. Neither of the fissures of Sylvius is quite closed, the left less so than the right; the operculum is but slightly developed, and the island of Reil lies with its fissures almost entirely uncovered. This conformation reminds us throughout of the brain of the anthropoid apes. The two sulci centrales sive fissuræ Rolandi run straight to the border of the hemisphere, less deeply impressed than is normally the case, without forming an angle with each other. Very strongly and deeply impressed sulci præcentrales seem to serve as substitutes for them. The sulcus interparietalis, which begins farther outward than in the ordinary human being, receives the sulcus parieto-occipitalis—a structure in conformity with the typical brain of the ape. The sulcus occipitalis transversus, which is generally lightly stamped in man, extends here as a deep fissure across over the occipital lobe, thus producing a so-called simian fissure, and the posterior part of the occipital lobe has the appearance of an operculum. The fissura calcarina has its origin directly on the surface of the occipital lobe, does not receive until late the fissura parieto-occipitalis, and goes directly, on the right side, into the fissura hippocampi. This abnormal structure also is typical for the brain of the ape.

"The gyrus occipitalis primus is separated from the upper parietal lobe by the sulcus parieto-occipitalis, a formation that, according to Gratiolet, exists in many apes. The gyrus temporalis superior is greatly reduced on both sides, and has an average breadth of only five millimetres; it is the one peculiarity that recalls emphatically the brain of the chimpanzee, which always has this reduced upper temporal convolution.

"We have here, then, a brain that scarcely deviates from the normal brain in volume, that possesses all the convolutions and fissures, seeming, perhaps, richer than the average brain in convolutions, and that is in every respect differentiated; and notwithstanding all this it approximates, in its whole structure, to the simian rather than to the human type. Had the brain been placed before me without my knowing its origin, I should have been perfectly justified in assigning this brain to an anthropoid ape standing somewhat nearer to man than does the chimpanzee."

No second case of this sort has thus far been observed.