Small Head Size Associated with Mental Defect
The Psychological Cl inic Copyright, 1932, by Lightner Witmer, Editor Vol. XX, No. 7 December, 1931 :Author: R. L. Jenkins, M.D.
Institute for Juvenile Research, Chicago
In indexing the data of over five thousand consecutive case records from the Institute for Juvenile Research, Dr Luton Ackerson 1 notes that the question of microcephalus appears thirty-one times. The case records of these thirty-one children were selected for study and it was found that all the children were mentally defective. This paper concerns itself with these thirty-one mentally defective children who approach the microcephalic type in varying degree. Perhaps only five of them justify the diagnosis of microcephalus in a rigid sense. In the consideration of any deviation from the norm, such as small head size, one is interested in the degree of deviation. In order to determine the degree of deviation, it is necssary to determine first the norm, and in this particular instance the selection of a norm presents considerable difficulty.
The simplest method of classification of head size is an absolute one. Thus, Tredgold 2 and others have used seventeen inches as the limit of microcephalus. An individual whose head size, on completion of development, falls below this circumference is classified as microcephalic. In dealing with children, however, one is immediately confronted with the fact that their heads have not yet completed * Studies from the Institute for Juvenile Research, Chicago. Series C, No. 196. 1 -Luton Ackerson, Children’s Behavior Problems (Chicago: The University of Chicago Press, 1931). Microcephalia was not treated statistically here because of paucity of cases. 2 A. F. Tredgold, Mental Deficiency (Wm. Wood & Co., 1929), p. 204. 193 development. Absolute head size therefore will not serve as a criterion of deviation from the norm. A second possibility of determining degree of deviation which presents itself is that of comparing the head-size of a given individual with the norm for his age. If it were known that the growth curve for the head circumference of microcephalic defectives paralleled the normal growth curve of head circumference, this method would be ideal, but, inasmuch as the fontanelles and sutures of the microcephalic sometimes close early in childhood, it seems reasonably certain that this is not always the case. The microcephalic may possibly have his head growth arrested at an earlier age than the normal. By this method of classification, therefore, such a microcephalic would become more widely deviate with approach to maturity.
Tredgold and others have stated that the intelligence of microcephalics is roughly proportional to the head size. In twenty-five cases of our children with small heads upon whom a measurement of head circumference was available,3 the correlation between intelligence quotient and head circumference was -f- .220 ? .128. The correlation between head size expressed in percentage of mean for age 4 and intelligence quotient was -f- .155 ? .131. The exact value of the intelligence quotient was in doubt in several of these cases, and the measurement of head circumference is somewhat influenced by the amount of hair. The size of the correlation coefficients indicates, despite these sources of error, that any relationship between head size and intelligence in this group of cases is very slight if indeed it exists at all.
For purposes of convenience in this paper, all cases with a head circumference of 44 cm. or less, who are also more than 10 per cent under the mean head circumference for their age, will be considered in one group as the extreme deviates. The remainder will be referred to as moderate deviates. There are eight extreme deviates among our cases, ranging in age from 3 years, 7 months to 11 years, 9 months. Four of these are idiots and four are imbeciles. There are eighteen moderate deviates and five unclassified (head circumference not recorded), s One case was discovered and added to the series after these calculations were made. It is not. included among these twenty-five. 4 Mean head circumference for age taken from Ales Hrdlicka, Anthropological Investigation on One Thousand White and Colored Children of Both Sexes. (New York: Wynkoop Hallenbeck Crawford Co.) ranging in age from 1 year, 9 months to 11 years, 9 months. Fourteen are idiots, four are imbeciles and five are morons.
Physical Defects
Of the extreme deviates, two had a left divergent strabismus. One of these was blind, emaciated, and helpless with a diplegia. Another had a marked visual defect and a history suggestive of epilepsy. Still another had a diplegia, emaciation, visual defect, and marked auditory defect. One was very obese and had a marked scoliosis. One was a Mongolian defective with total deafness, a congenital dislocation of the hip, a large congenital depressed skull fracture, and congenital heart disease. The remaining two, a pair of microcephalic brothers, displayed adherent ear lobules, and short incurving little fingers. One of these had almond-shaped eyes.
Among the moderate deviates there were six cases of spastic paralysis ? diplegia or paraplegia. Two were blind and six showed visual defect to less degree. Eleven had strabismus. One suffered a disseminated chorio-retinitis and one displayed nystagmus. In one case the left eye and left pupil were smaller than the right and in this and three others the pupillary response to light is described as sluggish. One child had irregular pupils. One child displayed a right facial paresis and another a right facial weakness. Three were classified as Mongoloid. In four cases the thyroid was enlarged but in only one case is the enlargement recorded as marked. There was a doubtful classification of “hypopituitary type” in one case. Cardiac disease, congenital or acquired, was diagnosed in five cases. Four cases were recorded as having cyanosis of the extremities. Enlarged tonsils and carious teeth were the rule. One child had a webbing of the hands and feet. One had undescended testes. One was obese.
We see a striking number and variety of major defects exhibited by this group. Most of these children fell short of normal height and weight. Kyphosis and scoliosis were frequent. Two moderate deviates were left-handed.
A definite history of convulsions was obtained in two of the extreme deviates and five of the moderate deviates. It is interesting to note that of twenty-six Wassermann reactions recorded, all were negative. Wassermann reactions were available on five extreme deviates (two tests in one case), thirteen moderate deviates (two tests in one case), and on five parents and one brother of moderate deviates. All the serological evidence regarding lues as a factor in these cases is therefore negative.
Prenatal and Natal Factors
There were no significant differences between the ages of the parents of these children and those of parents of Institute children in general.
The age of both parents was available in twenty-three of the microcephalic cases. The mean difference in the ages of the parents of the twenty-three microcephalics was 3.1 years, while the difference in ages of parents in 870 control cases was 4.4 years. Of the eight extreme deviates, four were boys and four were girls, while of the moderate deviates and unclassified, eleven were boys and twelve were girls. No sex difference was apparent. The average chronological age of the group was 8.3 years. This is somewhat younger than the age of Institute cases in general. The average size of family in the thirty-one cases was 3.5 children while that in a control series of 1,854 cases was 4.1 children. Five, or 16 per cent of the series studied, were only children while 12 per cent of the control series were only children. Fourteen, or 45 per cent, were first-born, while 37 per cent of 1,354 control cases were first-born. The mean birth position was 2.3 while that for the controls was 2.6. These discrepancies are probably explained by the younger age of the group with small heads.
The average time intervening between the birth of the preceding child and the child of this series was 3.3 years while that for 761 control cases was 2.9 years. Of course none of these differences are statistically significant with so few cases in the group of children with small heads. In five cases the pregnancy was described as difficult or uncomfortable, or the mother was stated to have been ill during pregnancy. In one additional case the mother had pneumonia less than one month before the birth of the child. In eleven cases the pregnancy was described as comfortable or normal. There were two cases of prolonged or difficult labor and one forceps delivery recorded. One mother fell on the sidewalk a month before delivery. Three infants were resuscitated with difficulty at birth.
The reported birth weights in eleven cases range from 2y2 lbs. to 12 lbs., and average 7 lbs., 6 oz. Three cases perhaps deserve individual mention. One extremely deviate idiot girl was the second to be born of a pair of twins. The other twin, a girl, was (according to the family) normal (age 3 years, 11 months). At birth the patient weighed 2y2 lbs. and the normal twin 5 lbs. The pregnancy went to term, according to the mother, but was uncomfortable. Delivery was normal. An idiot boy (unclassified group) was born in an asphyxiated condition (described as “blue baby”) after a two-hour labor. Delivery was spontaneous. The child was worked over by the physician for an hour and a half. His birth weight was reported to be 12 lbs. The head was described as “swollen at the back.” The child was unable to nurse for two weeks. This was the case in which the mother had pneumonia less than one month before the birth of the child.
In no case is there record of the treatment of the uterus with x-ray or radium during pregnancy.
Heredity
The investigation, from the case histories, of the heredity of this group of patients was unsatisfactory. In some cases few data were obtained. Most of the parents of these patients were foreign-born. Some spoke English poorly. Most had lost touch, to a greater or less degree, with their relatives in Europe. Furthermore, people are proverbially hesitant to reveal the existence of defectives in their family. The histories on this point reveal only a few alcoholics, a few unstable personalities, an aunt with amenorrhea and sterility, another affected with a tic, an uncle who was an epileptic, and a sibling with epilepsy. One child had a sibling who died at nine weeks of hydrocephalus.
There were six Jewish families among the group?not a disproportionate number for the Institute population. In four cases one or more siblings of the patient had been examined at the Institute. These cases seemed to offer better promise of study of small head size and mental defect with relation to their possible familial character. They are, therefore, reported.
Case Studies I A. A. and B. A., microcephalic brothers, were examined at ages 11 years, 9 months and 7 years, 0 months, respectively. They were referred by their school for lack of progress.
The biparietal diameter recorded for A. A. is 13 cm., that for B. A. is 12 cm. The occipitofrontal diameter for A. A. was 14.5, that for B. A. 14. The circumference of A. A.’s head was 43.7, that of B. A. 42. A. A. ‘s head circumference is 14 per cent below the average for his age, B. A.’s 19 per cent.
A. A. had an intelligence quotient of 33, B. A. an intelligence quotient of 41.
A. A. was 5.6 inches under the average height, B. A. 4.3 inches. A. A. was 6 lbs. underweight for his height and age, B. A. 1.5 lb. Both displayed Harrison’s groove, submerged, cryptic (A. A.), or scarred (B. A.) tonsils, with enlargement of the cervical lymph glands, adherent ear lobules, short incurving little fingers and evidences of nail-biting. A. A. had mild cyanosis of extremities and B. A. is recorded as having clammy extremities. Both had negative Wassermann reactions. Neither child was a behavior problem. A. A. had been observed to masturbate when he was small. He was thought to have stopped. He was given to protective lying. B. A. told lies both protective and fantastic.
The parents of A. A. and B. A. are described as dull, hardworking Polish folk of peasant background. The father was a railroad laborer, the mother worked out three hours daily, cleaning a library. The mother, 36 years of age at time of examination of the children, stated she had seven years of education in Poland. The father, 40 years of age, had “several years” schooling in Poland. Both were “strong and well.”
A. A. and B. A. were the first and third children, respectively, in a family of four children. The second child was a boy in the fourth grade (one year retarded). He was described as a fine boy and no trouble. This was confirmed by a neighbor woman who acted as interpreter. The fourth child was a boy 17^ months old, who was walking and beginning to talk.
All the relatives were in Poland and little was known about them. The grandparents were all dead. Two maternal aunts, four maternal uncles and one paternal uncle in Poland were described as “strong and well.”
Both children were born easily at term and breast fed (A. A., 11 months, B. A., 1 year). A. A. weighed 9 lbs. at birth, B. A., 8 lbs. A. A. walked at 13 months and talked at 5 years. B. A. walked at 1 year and talked at 4 or 5 years. Both had their first tooth at 6 months.
The parents planned to put these boys into a circus. Here we have two microcephalic defectives of strikingly similar development, born of dull parents with a sibling evidently of much more nearly normal, if not normal, intelligence between them.
II D. E., F. E., and G. E., sisters, were examined at different times. D. E., the eldest, was examined in 1923 at the age of 11 years, 6 months. She was referred by an orphan asylum for placement in an institution for the feeble-minded. F. E. was referred by the Juvenile Court for advice regarding disposition, because her home was unfit. She was examined in 1927 at the age of 12 years and 9 months. G. E., the youngest, was referred by the Juvenile Court in 1927, because a child-placing agency desired advice regarding placement. She was 11 years, 0 months of age. The mother of these children died in 1918. In 1919 they were placed in an orphan asylum. In 1923 D. E. was committed to a state institution for the feeble-minded. The father remarried the following year and took the remaining children home. The records for F. E. and G.
E. are more full than that for D. E. D. E. had a biparietal diameter of 13 cm., F. E. a diameter of 13.5 cm., and G. E. a diameter of 12.6 cm. The occipito-frontal was 16 cm. in the case of D. E., 16.2 cm. for F. E., and 15 cm. for G. E. The circumference was 48.1 cm. in the case of D. E., 49.6 for F. E., and 46.2 for G. E. D. E.’s head circumference was 6 per cent below the average for her age, F. E.’s, 9 per cent and G. E.’s, 10 per cent. The notation of microcephalic appearance occurs on the records for D. E. and G. E.
The intelligence quotient of D. E. was 57 (54 after several years at a state school for the feeble-minded), that for F. E. was 69 (63 on point scale performance) and that for G. E. 67 (67 also on point scale performance).
D. E. was 3 inches below the average height for her age and 1 lb. above the mean weight for her age and height. She had an opacity of the pupil of the left eye, due to an old injury, enlargement of the cervical lymph glands and perspiration of the hands and feet. The tonsils had been excised. The hemoglobin was 75 per cent and the red cell count 4,750,000. The leucocytes numbered 9,000 and there was a slight lymphocytosis (41%).
F. E. measured 4.9 inches below the average height for her age and 11 lbs. over the standard weight. She is recorded as having had carious teeth, high palate, infected tonsils and adenoids enlargement of cervical lymph glands, bilaterally enlarged epitrochlear glands and cyanosis of extremities. Her vision was defective (right eye 3/10, left eye 4/10). There was evidence of nailbiting. The thyroid was enlarged (1+) and the eyes are described as of Mongoloid type. The red cell count was 4,400,000. G. E. was one inch below average height and 8 lbs. underweight for her height. She had a right facial paresis, a convergent strabismus, defective vision (right eye 5/10, left eye 6/10), carious teeth with enamel defect, and poor dental hygiene. There was evidence of an old fracture of the left ulna. There was marked cyanosis of the extremities. Her face was somewhat asymmetric, with a high frontal region. There was a shallow Harrison groove, the right supraclavicular fossa was deeper than the left, and the resonance of the right apex was slightly impaired. The red cell count was 3,920,000. All three girls had negative Wassermann reactions. D. E. had been in an orphan asylum four years at the time of examination. There were no behavior difficulties and she was reported to work very well under supervision. She was committed to a state institution for the feeble-minded.
F. E. had a language deficiency. She was in the fifth grade (two years retarded). Her scholarship was poor. She had remained out till midnight four nights during the week preceding the examination and refused to tell where she had been. During the psychiatric interview she admitted soliciting boys and men on the streets for sex intercourse to secure money. She was committed to a state institution for the feeble-minded.
G. E. was in a special room in school. She ran away from home at every opportunity. She had no playmates but dogs. She also was a sex delinquent. She Avas placed in a fine boarding home. In a short time it was necessary to move her because of her sullenness, inattention, laziness, dirty and disorderly habits, roughness, interest in boys, disobedience, and general incorrigibility. She was then placed in a home of lower standards but did not improve. She was placed in a receiving home, but it was necessary to remove her as she was a detriment to the other children. She was then tried in two more foster homes. After re-examination at the end of the year, a commitment to a state institution for the feeble-minded was advised and carried out.
The parents of these children were born in Czecho-Slovakia. The father was a steel clipper who had held the same job for 21 years. His health was good. The mother was in good health (except for excessive drinking) until the time of lier death from pneumonia. This drinking was taken up subsequent to the birth of D. E. Very little information was obtained about the family. The father spoke English poorly, and the step-mother not at all. In the cases of F. E. and G. E. there is record of a normal birth and of infancy and childhood free from illness. D. E., F. E. and G. E. were the first, third and fourth of five children. The second, a boy, was reported by an orphan asylum to be dull and backward and later reported by his father to be “a good boy” in the “fifth or sixth grade.” The youngest child, a boy, was adopted.
Here we have three children in a family in whom small head size is associated with mental deficiency.
Ill J. L. and K. L., sister and brother, were examined in 1924 and 1926, respectively. J. L. at the age of 16 years, 1 month was referred by the Board of Education in order to find a suitable institution. K. L. was examined at the age of 3 years, 3 months. He was referred by his father because he could not walk or talk. J. L. had a biparietal head diameter of 15.3 cm., occipito-frontal diameter of 17.6 cm. and a head circumference of 54.5 cm. This was average for her age. Skull measurements are not recorded for K. L. The head was small. He has been described as microcephalic by a dispensary. J. L. lias an intelligence quotient of 51. K. L. was an idiot (intelligence quotient probably below 10).
J. L. was 5 inches below average height for her age and 42*4 lbs. over the average weight for her height and age. She had carious teeth, separation of the upper and lower incisors, asymmetry of the ears, hypertrophied remnants of tonsils and a slight divergent strabismus. She wore glasses. There was cyanosis of the hands and wrists, and mottling of the body. Her basal metabolism (one determination) was -f-39%. K. L. was an undersized infant of 3 years, 3 months, unable to walk or talk. He had scurvy and was thought to be suffering from hypothyroidism. J. L. was uncontrollable in her home. Her father feared she would become a sex delinquent. The child was placed in a private school for defective children. She was later removed to a state institution for defectives, where she was paroled to her home. She worked in a candy factory at $12.00 a week.
The parents were Jews born in Russia. There was no history of mental disease or defect in the family, according to the parents. The father seemed a bright, intelligent man.
No developmental history of J. L. was obtained. The mother had pneumonia less than one month before the birth of K. L. This was the 12 lb. (?) “blue” baby born spontaneously, in a badly asphyxiated condition after a two-hour labor. The physician worked resuscitating the infant one and one-half hours. The head was “swollen at back” and the infant could not nurse for two weeks. Convulsions set in at 2 months and lasted 2 years, sometimes as many as 10 or 15 attacks occurring in one day.
J. L. and K. L. were the first and third children, respectively, in a family of three children. The second child, a boy, was in the third grade before nine years of age and was described by the father as strong, lively, and “smart.” IV X. Z. and Y. Z., brothers, were examined at different times in 1920. X. Z. was referred by his father for convulsive attacks and examined at the age of 14 years, 4 months. Y. Z. was referred by the Juvenile Court because of feeble-mindedness.
X. Z. had a biparietal head diameter of 14.5 cm., an occipitofrontal diameter of 17.8 cm. and a circumference of 52.9 cm. This is 2y2% below the average for his age. The measurements for Y. Z. were not recorded, but he was described as microcephalic. The intelligence quotient of X. Z. was 87, that of Y. Z. was about 15. X. Z. had carious teeth and peg-shaped widely separated upper incisors. Y. Z. displayed a spastic paralysis of all extremities with contractures and atrophy, Harrison’s groove and Hutchinson teeth. X. Z. was not a behavior problem. Both children had epileptic attacks. Y. Z. was born spontaneously after a difficult labor.
The mother was sexually promiscuous before and after marriage and was divorced for extra-marital sex relations. She had two miscarriages, one before and one after the birth of these two children. She was addicted to the use of alcohol. The parents were both native-born, the supposed father apparently a normal individual. A paternal uncle was epileptic.
Discussion
It will have been noted that the preceding case reports fall into two categories. First, we have the pair of truly microcephalic brothers. When only eight children of truly microcephalic head dimensions were discovered by a search of the records of over 5,000 Institute cases and two of these children proved to be brothers, the likelihood of this being merely coincidence is extremely small. Next, we have a pair of sisters in whom small head size is associated with mental defect. These girls are both among the group of moderate deviates. Search of the index revealed a third sister in this family who had been examined at the Institute. This child also displayed comparably small head size and comparable mental defect. The last two families cited obviously belong in a different category. In each case we have a child of small head size and search of the cross index has revealed a sibling of normal head size. One of these small headed children displayed idiocy and a spastic diplegia with contractures and atrophy. The other was an idiot with a history of severe birth injury and convulsions. The first two families are considered as instances of small head size and mental defect associated as factors of hereditary tendency. It seems probable that the primary character in these cases is the familial tendency to cerebral hypoplasia and that the small head size is the secondary concomitant character.
Each of the second two families contains an idiot sibling with small head size and another sibling of normal head size. One of these was an imbecile. The other fell a little below average intelligence, but had convulsive seizures. A condition of infantile cerebral palsv seems probable in the case of each idiot. Here the primary factor was acquired cerebral damage and we see no evidence of familial tendency of the small head size.
Summary
Records of thirty-one children of small head size were selected from the files of the Institute for Juvenile Research. Study of these records revealed all the children to be mentally defective. There was a striking frequency of severe physical and neurological defect among this group. It was not possible to demonstrate any statistically significant differences between this group and the general Institute population in regard to birth position, age of parents at birth, or other items of this character. The Wassermann reaction was negative in the cases of nineteen of these children and six of their relatives who were tested. Four of the series were Mongolian defectives and should be considered generically different from the remainder.
Two families are cited in which there seems to have been a primary cerebral hypoplasia with associated small head size on an hereditary basis.
Two families are cited in which infantile cerebral palsy was apparently responsible for small head size in one sibling while the unaffected child had a head of normal size.
Conclusions
Small head size may be associated with mental defect apparently due to a primary cerebral hypoplasia, with mental defect due to infantile cerebral palsy or with mental defect due to Mongolism. There is indication that small head size associated with mental defect due to cerebral hypoplasia may be hereditary.
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