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).