Case Report TWO NEW CASES OF XYY CHROMOSOME COMPLEMENT And a Review of the Literature*
BRIAN
Introduction The significance of an XYY chromosome complement is controversial and uncertain at the present time (3,15). On the one hand, approximately 1:500-1:900 live male newborns have this complement (3, 9, 24, 39) but very few seem to come to clinical attention; on the other hand, surveys of institutions for the retarded (6, 18), the mentally ill (2, 12, 16, 22), the criminally insane (5, 9, 18, 28, 30) and the aggressive offender (11, 21, 23, 26, 38) have all shown a higher incidence of the XYY complement than would be expected in the general population. Some investigators (4, 15) have tried to explain these individuals, in mental institutions and prisons, on the basis of their being tall and therefore more likely to be considered as dangerous by judges and psychiatrists. Other authors have described them as having more immaturity, emotional lability, and criminality than the normal population (44), and even more emotional instability and impulsivity than other inmates (30). Clinical case histories in the literature generally focus on the unusual and pathological, and one cannot overlook the need for genetic population studies to assess to what extent various sex chromatin abnormalities occur without manifest psychopathology. It was not until 1961 that Sandberg (35) described an XYY man. This was followed in 1965 by Jacobs (18) who reported seven XYY cases among 197 institutionalized 'Manuscript received December 1976; revised September 1977. lLecturer, Dept. of Psychiatry, University of Toronto; Staff Psychiatrist, Clarke Institute of Psychiatry, Toronto, Ont. Can. Psychlatr, Assoc. J. Vol. 22 (1977)
F.
HOFFMAN,
M.D.1
hard-to-manage retarded inmates of a Scottish maximum security hospital (3.6%). All seven were significantly taller than average but had no other physical abnormalities, and none were psychotic. Price and Whatmore (30) added two more cases from the 119 "mentally diseased" dangerous patients from the same hospital (1.9%) for a total of nine XYY patients among 340 criminal, mentally retarded or diseased patients. All of these cases were unusually tall and in fact 30 percent of the men who were 6 feet tall, or more, had double Y chromosomes. Eight were mentally retarded. Comparing all nine cases to a control group of other inmates with severe personality disorders, the authors found the XYY patients were more emotionally unstable, and unable to tolerate even the mildest frustration. They were first convicted at age 13, compared to the control age of 18. Two of the nine had committed sexual assaults against young children; one of the patients showed some schizophrenic features. Jacobs also estimated a population incidence of 0.14 percent by examining 3,500 consecutive male births and pointed to the much higher rate of the XYY chromosome complement in the institutional population. Because of Jacobs' initial finding of an association between the XYY syndrome and aggressive, impulsive, tall men, other investigators began to screen institutional populations and to identify more and more cases. Some authors have described cases which were discovered by screening groups of the criminally insane. In 1966, Casey (5) screened patients over six feet tall in a variety of settings for the criminally insane and found 12 XYY men among 50 tall,
447
448
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
mentally retarded men retained for antisocial behaviour (24%), 4 XYY men among 50 tall, mentally ill patients detained because of antisocial behaviour (8%), and 2 XYY cases among 24 tall criminals (8%). He concluded that, "an extra Y chromosome has a part to play in antisocial behaviour even in the absence of mental subnormality. " In an institution for sex offenders and mentally ill offenders with a total population of 1,400 inmates, Melnyk (22) found 9 XYY individuals (4.5%) among the 200 patients who were six feet, or more, in height. Of 79 mentally disordered sexual offenders he found seven XYY men (8.9%), and of 121 mentally ill, criminally insane, tall men he found two XYY men (1.6%). The seven sexual offenders had committed female pedophilia, aggressive voyeurism, aggressive homosexuality, rape sadism and masochism, male pedophilia and attempted rape, forcible rape, and child molesting. The two patients who were criminally insane had committed vagrancy and car theft, and homosexuality with aggressive acting out. Melnyk points out that "the incidence of 47, XYY in tall, mentally disordered sexual offenders (8.9%) is approximately eight times that of a comparable group of mentally deficient males, and approximately eightynine times the incidence (0.1%) in the general population." Then in 1969, Daly (9, 10) examined all men who were over six feet tall in four maximum security hospitals in Wisconsin, Michigan, Indiana and Ohio and found 10 XYY patients by chromosome analysis, Of these, three were mentally retarded, three were sociopathic, three were schizophrenic and one was "difficult to diagnose because of evidence for a progressive organic disease of the central nervous system." Because of Jacobs' finding that most of the XYY men in a maximum security hospital were mentally retarded, other researchers screened institutions of the mentally retarded, as well as prisons and other institutions. In 1968, Court Brown (7) added another 15 cases who were detected by chromosome studies of newborn babies and of the populations in prisons and hospitals for the mentally subnormal and for mental
Vol. 22, No.8
disease. Three of the cases were discovered in a criminal and delinquent population; two were in mental hospitals, as a result of minor crimes and a lifelong pattern of chronic drifting vagrancy; eight were located in hospitals for the mentally subnormal; one was a newborn baby and the last was a 34 year-old man with a normal psychosocial development who had suffered a stroke. Ricci (33) also described one XYY retarded boy who was tall. Kelly (19) reported a 24 year-old mentally retarded male with a history of seizures, emotional disturbances and suspected arson. Rainer (32) investigated a pair of monozygotic twins with an XYY complement who were both impulsive destructive and yet docile and unassertive when not stressed. In 1974, Clarke (6) described five XYY individuals at the special school in Aberdeen. Their range in I.Q. was from 52-70 and all showed poor motor control and impulsivity. Thus, whether investigators found the XYY subjects in institutions for the criminally insane or for the mentally retarded, the men were most commonly described as impulsive aggressive and antisocial. Meanwhile other investigators screened tall patients in mental hospitals. Forsmann (12, 13) described 12 XYY men from a university psychiatric hospital and research centre and found five with a criminal record. All five criminals were slightly retarded, while three of the seven XYY men without a criminal record were retarded and the other four were normally gifted. Three of the five institutionalized males without a criminal record had a "chronic psychosis", one a chronic obsessional state, and one chronic hypomania and addiction to alcohol. Forsmann concluded that "an abnormal sex chromosome complement may have an unfavourable effect on mental health. It may contribute to the development of the functional psychoses and may even influence such a complex variable of behaviour as our ability to adapt ourselves to the laws of the community. " On the other hand he acknowledged that the XYY complement is "compatible with complete mental health and normal intelligence like the case published by Wiener and Sutherland in 1968." Similar
December, 1977
Xr'Y CHROMOSOME COMPLEMENT
449
cases were more recently described by Berg sexual assault. Welch (41) also described a and Lowy (3), and Stenchever and MacIn- tall "defective delinquent" who had this tyre (36). same chromosome complement. In 1969, In 1969, Abdullah (1) reported a 26 Wiener and Sutherland (43) described four year-old man, who had been a behaviour XYY men that they found by screening tall problem since the age of 14 months, men in a Melbourne prison - all four were characterized by hyperactivity and destruc- diagnosed as psychopaths and only one was tive violent behaviour. At age 13 he had retarded. In 1972, Borgaonker and colbegun to hallucinate and have delusions and leagues reviewed the charts of 20 XYY continuously rambling speech. Since that males at the Chromosome Laboratory of time he had spent most of his life in mental Johns Hopkins. Eleven of these were tall hospitals or institutions for the criminally men, ten of whom were inmates of a penal insane. His full scale I.Q. was 83. The institution; the remaining nine cases were diagnosis of chronic schizophrenia was juveniles, four of whom were above the 90th made. percentile range for height. Three of the nine Akesson (2), in 1969, reported screening juveniles had been institutionalized - two as 117 tall men (over 6 feet) admitted to delinquent and one as emotionally or menordinary mental hospitals in Sweden and tally disturbed. The remaining six were found four XYY men. One of these patients referred because of behavioural or emotional had a "chronic psychosis", one had mental disturbances although the authors did not deficiency (borderline) and two had a adequately describe or diagnose these. Also, "prolonged neurotic syndrome." Parker no information was given as to the I.Q. (27) surveyed 10 tall patients with aggressive levels of these juveniles. Marinello (21), in 1969, screened 86 tall behaviour from a state hospital and found one patient with an XYY constitution. He men over 163 em (6 feet) in Attica State was tall, obese and mentally retarded. In Prison and found two cases of XYY 1970, Nielson (23,25) reported 12 XYY complement, and 76 tall men from a state patients, three were in a psychiatric hospital, hospital where he found one XYY complefour in a forensic psychiatric ward, four in ment. He also discovered one XYY cominstitutions for criminal psychopaths and one plement in testing 57 tall, male juvenile in a medical ward. Eleven of these patients delinquents. The first XYY case from the (91%) had "major criminality with violation prison is summarized as a schizoid, mentally of the penal code" and 36 percent were retarded giant who had been convicted of violent. The frequency of sexual criminality child molesting. The second was a 24 towards children was 27 percent and of year-old with an aggressive personality and a arson, 27 percent. The average I.Q. was history from age five of being difficult, 96.0. Richards (34) examined a 47 year-old hitting and making sexual advances toward man in a mental hospital who was diagnosed siblings, fighting with peers and teachers, as an aggressive psychopath. He had shown and eventually committing petty larceny at homosexual tendencies and had made ag- age 15, and armed robbery at age 23. The gressive attacks unrelated to his sexual case in the mental hospital was a 13 year-old orientation. Thus, many of the cases found in with a diagnosis of undifferentiated schizothe mental hospital are described as impul- phrenia characterized by auditory hallucinasive, destructive and antisocial and some tions, and flat affect. It is again interesting that this patient had tried to bum down a were also mentally retarded. There are several reports on XYY men house prior to his confinement. In ;970, among the criminal and juvenile delinquent Falek (11) was screening a prison populapopulations. Telfer (37, 38) describes five tion, either over six feet tall or with acne, or XYY men who were found among a criminal both, and found one man with the XYY population - two were adult criminals, two syndrome. It is interesting that his crime was were criminally insane and one was a arson and that he had also undoubtedly been juvenile delinquent who was being held for schizophrenic with blunted affect, "loose
450
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
associative linkages" and "paranoid" ideation since the age of 14. Cowie (8), in 1968, described an eight year-old boy with a history of antisocial behaviour, truancy and conflict with the police and school. He was tall for his age and when investigated was found to have an XYY chromosome complement. Hunter (17), in the same year, found three XYY boys from a delinquent population screened for height over the 90th percentile. The last group of XYY men who have been described in the literature have been discovered by screening normal populations. Zeuthen (44) reported in 1975 that he had found five XYY men among 3,840 men being examined for military service in Denmark. Only those who were very tall (181 em. or above) or who had small testes (12 ml) were chosen for chromosome analysis. The intellectual level was within the normal range in all but one; the mean full scale I.Q. was, however, lower than expected. These men showed poor attention and were "concrete and unreflective." Their educational level was significantly lower than expected; four of the five had had difficulties at school and three had been in special classes for children with behaviour disorders or slight retardation, or both. Three of them were hyperactive, restless, hot tempered and impulsive during childhood. All five grew up in "good homes" and were considered different from their siblings. Two of the five had criminal records, in one case, for petty larceny and theft, and in the other, for theft and wanton destruction. On psychological testing, all five men displayed "immaturity in cognitive style and in emotional adaptation. Emotional lability was present in all cases, resulting in lack of control of aggressive impulses, and all the men also showed unfulfilled need for contact and passivity. " The only other survey of a normal population was undertaken by Valentine, McClelland and Sergovich (39) who found four XYY infants by testing 1,066 unselected newborns. They followed these children for 2 1/2 to 3 1/2 years and reported that three of them appeared to have normal intellect and personality, but one was of
Vol. 22, No.8
borderline intelligence (I.Q. = 82) and by age two was exhibiting "aggressive, defiant, and destructive behaviour." The authors discussed the difficulties in doing follow-up studies and the dilemma as to what parents or guardians should be told, if anything. There are also a few isolated case histories of XYY chromosome complement associated with epilepsy (14), multiple congenital abnormalities (20), abnormal electrocardiograms (31) and genital abnormality (40). Thus, of the 153 cases of XYY chromosome complement that were found in the literature, 135 were discovered within institutions: 47 in maximum security hospitals for the criminally insane; 31 in prisons; 24 in psychiatric hospitals; 18 in the delinquent population; and 15 in institutions for the mentally retarded. An additional nine cases were found by surveys of normal populations (39, 44). The remaining nine are isolated case histories. There are only four XYY men described in the literature who were essentially normal in development (3, 35, 36, 42), and in addition there are three XYY children under the age of 3 1/2 years who had remained normal when last evaluated (39). The remainder of the case histories show a predominance of tall men who are either retarded or, if not, often have had difficulties in school that could be called" learning disabilities" (44). The XYY men reported are often impulsive, aggressive and antisocial and have criminal records beginning at an earlier age than other delinquents or criminals (30). Even six of the nine cases of XYY chromosome complement found in general population surveys (39, 44) showed a proclivity to impulsive destructive and antisocial behaviour. Many of the XYY inmates in prisons or maximum security hospitals were also noted to be retarded (5, 9, 18). The nature of the crime and violence tended to be impulsive and destructive and not well thought out. The charges leading to incarceration varied from murder (22) to vagrancy (5). There were a number of case reports that pointed out the incidence of sexual crimes (20 cases), especially against minors, (16, 18, 21-23) and of arson, (six
December, 1977
Xyy CHROMOSOME COMPLEMENT
cases - 11, 19, 21, 23). Of those cases described in detail there are at least 11 with a diagnosis of schizophrenia or "chronic functional psychosis" (1, 2, 9, 11, 12,29), with an additional four cases of very severe schizoid personality (7, 12,21). In addition, some of the 47 patients found in maximum security hospitals were probably schizophrenic, although the psychiatric description or diagnosis was not included in many of the case reports. The current literature does not indicate that there is any physiological or anatomical abnormality of high incidence other than the prolonged P.R. interval on the ECG (31), and tallness. The following case histories describe patients recently discovered who show certain features similar to those surveyed in the literature. Case 1 This unmarried, 22-year-old plumber's helper had been staying in a psychiatric hospital for five months because of fears that he was going to lose control and hit or kill someone for no reason. He felt nervous and very guilty because he could not control these feelings and wept as he talked of these impulses. For the past two years the patient complained of "increasing difficulty in thinking straight, difficulty in getting along and in talking with other people." He stated that he was anxious when around people and that he had frequent anger outbursts during which he is afraid of losing control. For several years, he tended to isolate himself because of his anxiety and so he would not hurt anyone. On the night prior to admission, he had had an argument with someone in the house where he lived and was afraid he would hit or kill this person, although the patient recognized the triviality of the argument. Past Legal and Psychiatric History: At age 14, he was convicted of raping a 9 year-old girl. At age 15, he was convicted of breaking and entering. He was convicted of assault with a deadly weapon, at age 16, when he displayed a knife during a fight. He had numerous convictions for drinking while under age. At age 17, he was admitted to a psychiatric hospital where he was difficult to manage because of fire-setting on the ward. He did not cooperate in follow-up treatment and for the next few years lived a solitary life, while being on welfare and unemployed. Personal History: Birth and development were
451
apparently normal. Beginning at age five he showed a constant behaviour problem, setting fires and fighting with teachers. He did poorly in school, failing examinations several times in the lower grades. He had always been a loner and felt other people were looking down on him. This was aggravated by his frequent failures at school and by his legal problems, which he thought were widely known in the community. He had few friends and a decreasing interest in sex with it being less now compared to when he was a teenager. The patient said the rape incident was motivated by curiosity rather than "passion". He does not enjoy his occasional sexual feelings now. He has always had difficulty in controlling tension and would frequently punch walls or get drunk to relieve the tension. His work record was poor, with most jobs coming quickly to an end because of his anxiety and impulsive aggressive behaviour. Family History: The patient describes his father as an alcoholic and a very rough man, who would beat him with a belt for no reason. He describes his mother as a good person, but he could not confide in her because she told everything to his father. Neither parent was as tall as the patient. He is the third of five siblings - the others are all doing well with no known emotional or psychiatric problems. No one else in the family has legal problems or has been admitted to a psychiatric facility. Mental State: On examination the patient was very muscular, 6 feet, one inch tall, and weighed 180 pounds. He was unshaven but otherwise neatly dressed, appeared nervous and agitated, and frequently changed his position. On the ward, he would have poor impulse control and kick or punch the wall angrily with little or no provocation. He complained of frequent angry feelings which he found difficult to control. He felt depressed and said that life was not worth living but denied any thought of taking his own life. The patient states that his mind wanders and that he has difficulty in concentrating and organizing his thoughts. He showed blocking and tangentiality of thought and had great difficulty in expressing himself. For example, "Last week I ... its been I don't know what building ... I get paranoid is happening. Its ... I'm confused ... my mind is empty . . . they make it that way." The patient said "a force field is hacking up my thoughts." He also felt at times that people were interfering with his thoughts "shoving them aside" or even "crawling inside." At other times he experienced his thoughts as "floating away" , which left his mind empty.
452
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
The patient states that he heard voices talking to him, calling his name and also commenting on his actions in the third person. He also described hearing high pitched noises from time to time. The patient's memory was intact but his attention and concentration appeared to be somewhat impaired. His ability on tests of similarities, comparisons and proverbs was essentially normal. Investigations: A chromosome karotype showed 47 chromosomes, and the extra "Y' chromosome was confirmed by fluorescence. Tests showed an I.Q. of 106 with little inter-test variability. Despite a normal I.Q., the school reports showed poor progress throughout school. Serial EEGs consistently showed a diffuse dysrhythmia of intermittent 4-7 c.p.s. theta activity of low and medium voltage. Epilepsy, however, and focal abnormalities were ruled out. Course in Hospital: In hospital, his condition responded quickly to Trifluoperazine and Pericyazine. However, the patient remained highly dependent on the security of the social atmosphere of the hospital. He continued to show a very low frustration tolerance, remained explosively angry and would periodically hit walls, windows and furniture. He was preoccupied with fires and on one occasion was found setting a ping-pong ball on fire and watching it bum. He continued to have ruminations, not always unpleasant to him, of raping little girls. With prolonged individual and group psychotherapy, an intensive rehabilitation program and the use of Pericyazine and Thioridazine, the patient's condition improved. Following discharge, he has continued to see the author. He completed a rehabilitation course in the community and has been steadily employed for ten months. The diagnoses are acute paranoid schizophrenia and personality disorder (severe) of the antisocial and passive-aggressive type.
Case 2 This 18 year-old male was referred to a psychiatric hospital by his probation officer. The patient said that for the past 3-4 years he had suffered from extreme nervousness and unhappiness. He felt crowded in by people, felt that they stared at him and talked about him, and that strangers called him "queer" when he walked down the street. The patient tended to isolate himself because of these fears and had no friends. He also complained of lifelong aggressive tendencies over which he had little control. His behaviour became much worse at the age of 12 when his mother left the family. He gave a history of frequent fights in school - he eventually was
Vol. 22, No.8
expelled. In adolescence, fights had occurred in billiard halls, or bars and he quit or lost at least three jobs because of feeling picked on, and because of anger outbursts when he would put his fist through a wall.
Personal History: The patient's birth and early development were normal. From the age of five he has always been a very angry, tough, little boy. In class he was hyperactive and easily distracted. From age 7 or 8, he has hated animals and has often been cruel to cats. He described several incidents where he very much enjoyed setting fire to garbage cans in the neighbourhood, feeling excited while watching the police and firemen arrive. Throughout his school years, he has had difficulties and attended at least five schools before he was 15. When he reached grade 8, at age 15, he was transferred to a vocational school where he continued to get into numerous fights. He had avoided trouble with the police until he was 15 years of age, when he played truant from school and at 16, when he cashed his brother's pay cheque and was put on probation for one year. He had also been charged for shoplifting items which he did not need. He has never had friends, and none of his four jobs has lasted more than a few weeks. When he returned home, he ran off frequently and unexpectedly, and often slept in garages and subway stations. Family History: The patient describes his father as a caretaker, 5 feet 8 inches in height, a quiet man who, however, has serious violent outbursts. The mother is also described by him as being explosive, and he reports, without being able to give details, that she has had "nervous breakdowns". The patient's father told us that his wife deserted the family when the boy was 12 years of age and following that, the patient's behaviour deteriorated with increased truancy and impulsive aggressive behaviour. There is one younger brother who does not have any emotional problems, but who the patient feels deliberately provokes him by always calling him "dummy" . Mental Status: On examination, the patient was 6 feet 4 inches tall and weighed 170 pounds. He appeared much older than his stated age of 18. He was anxious and restless and had difficulty in sitting still. There was a certain grandiosity and bravado in his plans and many discrepancies in his story. There was a lack of logic in his thoughts and his speech was circumferential, almost tangential. However, there was no definite looseness of associations or autistic thinking. The diagnosis was personality disorder with aggressive, antisocial and paranoid features.
December, 1977 Discussion
X'YY CHROMOSOME
The similarities between these two cases and some of the cases described in the literature are quite striking. Both young men were over 6 feet tall (7, 30), impulsive, aggressive and antisocial, to a degree that they resemble the criminal populations studied by Falek (1 I), Nielsen (23, 26) Telfer (38) and Wiener (43). This abnormal behaviour began in both patients at a very early age and they therefore resemble the delinquent populations studied by Borgaonkar (4), Cowie (8) and Marinello (21). Both patients were convicted of offences prior to age 18, similar to the patients of Price (30). In addition both were quite suspicious and even paranoid toward the environment, and the patient in Case 1 suffered from an acute paranoid schizophrenia illness that is similar to the cases described by Abdullah (1) and Marinello (21). Whether there is any connection between this type of acute psychotic illness and the chronic psychosis also described in the literature (2, 9, 12) is unknown. The (Case 1) patient had been convicted of rape at the age of 14 and therefore shows a similarity to the 20 cases of sexual crimes among XYY men described in the literature (16, 18, 21, 37). Although he was of normal intelligence, he did not progress well in school (44), and the patient in Case 2 had no better than borderline intelligence similar to the retardates (6, 7, 19, 33) and retarded criminals (5, 7, 12,23,41). Other similarities include the preoccupation with fire (11, 21, 23) and the normal anatomy and physiology except for tallness. It must be recognized that most of the cases described in the literature were found by screening the biased or abnormal populations of prisons, psychiatric hospitals and institutions for the retarded. It is not known yet whether any of this pathology is more common in XYY men than in the normal population. However, from the nine XYY cases found by the general population surveys of Valentine (39) and Zeuthen (44), at least six have had a grossly abnormal psychosocial development with difficulties in school, restlessness and impulsivity, criminal convictions, lack of control of aggres-
453
COMPLEMENT
siveness and emotional lability. The three "normal" XYY infants followed by Valentine were not, in fact, followed past the age of 3 1/2 years and their future development may be in doubt. Because there are a few normal XYY cases reported and because very few of the XYY men come to medical and legal attention, it is unlikely that this chromosome complement is a sufficient and necessary cause for the described psychopathology. It is also apparent that genetic influences in the criminal and mentally ill population may be underestimated. Investigation of tall, impulsive, aggressive men with criminal records beginning at an early age and involving arson and sexual offences, as well as persons with a paranoid and schizoid life style, may well reveal an XYY chromosome complement. Because so many of the cases seem to follow a pattern, it is possible that this chromosome pattern predisposes to an abnormal personality development which becomes manifest only if certain abnormal environmental influences are present. In this context, the impulsive, aggressive parents of these two patients could be important. However, if we are to further elucidate the phenotype of an XYY genotype, we will need to screen and test a very large segment of the normal population for sex chromatin abnormalities or else test newborns and do a follow-up study to assess their personality and behaviour. The medical and ethical problems of such a study has been discussed by Valentine (39). Summary
Two cases with XYY chromosome complement are presented and compared with 153 cases described in the literature. Some patients with abnormalities of personality development and manifest psychopathology may have sex chromosome abnormalities and should be studied further for the interaction of genetic and environmental factors in personality development. In particular, tall, schizoid impulsive men with a history of criminality, arson or sexual offences should be screened for this particular genetic configuration. Because of the biased manner by which most of the cases
454
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
are found, It IS not yet known whether an X'r'Y genotype results in a characteristic phenotype. What is needed is a genetic study of a normal population and a prospective study of newborns with various genotypes which would, unfortunately, create complex research and ethical problems. Acknowledgements
The author is grateful to Drs. F.H. Lowy and E. Persad for bringing the second case to his attention, and to Mrs. Lillian Scarpellino for typing this manuscript.
References
1. Abdullah, S.: Extra Y chromosome and its psychiatric implications. Arch Gen Psychiatry, 21: 497,1969. 2. Akesson, H. O. et al: Gross chromosomal errors in tall men admitted to mental hospitals. Acta Psychiatr Scand, 45: 37, 1969. 3. Berg, J. M., Lowy, F. H.: XYY syndrome: a comment. ModMed Can, 30: 692,1975. 4. Borgaonkar, D. S., Unger, W. M., Moore, S. R., Crofton, T. A.: 47, XYY syndrome, height and institutionalization of juvenile delinquents. Br J Psychiatry, 120: 549, 1972. 5. Casey, M. D., et al: YY chromosomes and antisocial behaviour. Lancet, 2: 859, Oct. 1966. 6. Clarke, D. F., Johnson, A. W.: XYY individuals in a special school. Br J Psychiatry, 125: 390, 1974. 7. Court Brown, W. M., Price, W. H., Jacobs, P. A.: Further information on the identity of 47, XYYmales.BrMedJ, 2: 325,1968. 8. Cowie, J., Kahn, J.: XYY constitution in a prepubertal child. Br Med J, 1: 748, 1968. 9. Daly, R. F.: Mental illness and patterns of behaviour in 10 XYY males. J Nerv Ment Dis, 149: 318, 1969. 10. Daly, R.F.: Neurological abnormalities in XYY males. Nature, 221: 472,1969. 11. Falek, A.: An attempt to identify prisoners with an XYY chromosome complement by psychiatric and psychological means. J Nerv Ment Dis, 150: 165, 1970. 12. Forssman, H.: The mental implications of sex chromosome aberration. Br J Psychiatry, 117, 353, 1970. 13. Forssman, H., Hambert, G.: Chromosomes and antisocial behaviour. Lancet, 282, July 1966.
Vol. 22, No.8
14. Forssman, H.: Epilepsy in an XYY man. Lancet, 1389, June 1967. 15. Hook, E. B.: Behavioural implications of the human XYY genotype. Science, 179: 139, 1973. 16. Hope, K.: Psychological characteristics associated with XYY sex-chromosome complement in a state mental hospital. Br J Psychiatry, 113: 495-8, 1967. 17. Hunter, H.: Chromatin-positive and XYY boys in approved schools. Lancet, 816, April 1968. 18. Jacobs, P. A., Bronton, M., Melville, M. M.: Aggressive behaviour, mental subnormality and the XYY male. Nature, 208: 1351,1965. 19. Kelly, S., Almy, R.: Another XYY phenotype. Nature, 215: 405,1967. 20. Kosenow, W., Pfeiffer, R. A.: YY Syndrome with multiple malformations. Lancet, 1375, June 1966. 21. Marinello, M. J., Berkson, R. P.,: A study of the XYY syndrome in tall men and juvenile delinquents. JAMA, 208, 321, 1969. 22. Melnyk, 1.: Sex chromosome XYY phenomenon occurrence examined in mental patients. Nature, 224: 369,1969. 23. Nielsen, J.: Criminality among patients with Klinefelter's syndrome and the XYY syndrome.BrJ Psychiatry, 117: 365,1970. 24. Nielsen, J.: Klinefelter's syndrome and the XYY syndrome. Acta Psychiatr Scand, Supplement, 209, 1969. 25. Nielsen, J.: Prevalence and a 2 1/2 years incidence of chromosome abnormalities among all males in a forensic psychiatric clinic.BrJ Psychiatry, 119: 503-12,1971. 26. Nielsen, J., Tsuboi, T., Sturup, G., Romano, D.: XYY chromosomal constitution in criminal psychopaths. Lancet, 576, Sept. 1968. 27. Parker, C. E., Melnyk, J., Fish, C. H.: The XYYsyndrome.AmJMed, 47: 801, 1969. 28. Polani, P. E.: Abnormal sex chromosomes and mental disorder Nature, 223: 680, 1969. 29. Price, W. H., Strong, J. A., Whatmore, P. B., McClemont, W. F.: Criminal patients with XYY sex-chromosome complement. Lancet, 565, March 1966. 30. Price, W. H. Whatmore, P. B.: Behaviour disorders and patterns of crime among XYY males identified at a maximum security hospital. Br Med J, 1: 533, 1967. 31. Price, W. H.: The electrocardiogram in males with extra Y chromosomes. Lancet, 1106, May 1968. 32. Rainer, J. D., Abdullah, S., Jarvik, L. F.:
December, 1977
33. 34. 35. 36. 37. 38.
39.
40. 41.
42. 43.
455
Xyy CHROMOSOME COMPLEMENT
XYY karyotype in a pair of monozygotic twins: a 17 year life history study. Br J Psychiatry, 120: 543, 1972. Ricci, N., Malacame, P.: An XYY human male. Lancet, 721, March 1964. Richards, B. W., Stewart, A.: The YY syndrome. Lancet, 984, April 1966. Sandberg, A. A., Koepf, G. F., Ishihara, T., Hauschka, T. S.: An XYY human male. Lancet, 488, Aug. 1961. Stenchever, M. A. MacIntyre, M. N.: A. normal XXY man. Lancet, 680, 1969. Telfer, M. A., Baker, D., Longtin, L.: YY syndrome in an American Negro. Lancet, 95, Jan. 1968. Telfer, M. A., Baker, D., Clark, G. R., Richardson, C. E.: Incidence of gross chromosomal errors among tall criminal American males. Science, 159: 1249, 1967. Valentine, G.H., McClelland, M. A., Sergovich, F. R.: The growth and development of four XYY infants. Pediatrics, 48: 583, 1971. Vignetti, P., Capotorti, L., Ferrante, E.: XYY chromosomal constitution with genital abnormality. Lancet, 588, Sept. 1964. Welch, J. P., Borgaonkar, D. S., Herr, H. M.: Psychopathy, mental deficiency, aggressiveness and the XYY syndrome. Nature, 214: 500, 1967. Wiener, S., Sutherland, G.: A normal XYY man. Lancet, 1352, 1968. Wiener, S., Sutherland, G. et al: XYY males in a Melbourne prison. Lancet, 150, Jan. 1968.
44. Zeuthen, E., Nielsen, J., Hansen, M., Christensen, A.: A psychiatric-psychological study of XYY males found in a general male population. Acta Psychiatr Scand, 51: 3-18, 1975.
Resume On presente deux cas de complement chromosomique XYY et on les compare avec 153 cas decrits dans le litterature. Certains patients montrant des anomalies du developpement de la personnalite avec psychopathologie evidente peuvent avoir des anomalies des chromosomes sexuels, ce qui demande une etude plus en profondeur de I' interaction des facteurs genetiques et de I' environnement sur le developpement de la personnalite. Plus particulierernent, on devrait faire le depistage de cette configuration genetique chez des hommes grands, irnpulsifs et schizoides, ayant une histoire d'offenses criminelles, sexuelles ou incendiaires, Cependant, vu la facon selective dont la plupart des cas ont ete detectes, on ne sait pas encore si un genotype XYY correspond a un phenotype caracteristique. Il serait done necessaire de proceder a une etude de genotype chez la population normale et aune etude prospective chez les nouveaux-nes lesquels presentent des genotypes varies. Toutefois, ceci exigerait des recherches complexes et poserait des problemes d'ethique.
Association des Psychiatres du Canada Canadian Psychiatric Association DEMANDE D'ADMISSION Monsieur Ie Secretaire, Association des psychiatres du Canada, Bureau 103, 225, rue Lisgar, Ottawa, Canada K2P OC6 Je demande mon admission comme membre actif, membre associe, membre correspondant, membre affilie, membre en formation ou membre inactif de l'Association des psychiatres du Canada. (Biffer les mots qui ne s'appliquent pas). Je suis autorise exercer dans la province de . _
a
Signature
Date
Nom et prenoms: (lettres moulees) . .
.
Adresse de bureau
.
Domicile:
.
...._.
.
. .
_ .
Date du diplome en medecine et endroit:
.
Certificat du College Royal des medecins et chirurgiens du Canada:
Qui
Certificat d'un bureau provincial: Nom du bureau provincial:
_ _
.
Date de naissance:
.
. Non
0 0
. ....
_
0 0
.
_ Date
---.-.-.--------------------------------
.
.__.
_ _ _
Bref expose de la formation et de l'experience en psychiatrie: Genre de travail actuel en psychiatrie: Si vous etes inscrit l'universite
aun cours de specialisation, mention de
.__._.
_
a
Je m'engage me conformer aux Statuts et Reglements ..__... de l'Association des psychiatres du Canada
_
Signature du candidat
Recommandation: (A etre signee par deux MEMBRES ACfIFS de I'Association)* (1) _ Nom en lettres moulees
Signature
(2)
Nom en lettres moulees Signature ·Aucune recommandation exigee pour un membre correspondant,
,
REGLEMENTS
I. 2. 3. 4. 5. 6.
Les membres actifs versent une cotisation annuelle de $125.00. Les membres associes versent une cotisation annuelle de $30.00. Les membres correspondants versent une cotisation annuelle de $30.00. Les membres en formation versent une cotisation annuelle de $20.00. Les membres affilies versent une cotisation annuelle de $25.00 Un mari et sa femme qui sont tous deux membres actifs versent une cotisation annuelle reunie de $240.00. 7. La cotisation comprend I' abonnement a la Revue de I' Association des psychiatres du Canada et a la Lettre-nouvelles de I'A.P.C. 456
BRIAN
Introduction The significance of an XYY chromosome complement is controversial and uncertain at the present time (3,15). On the one hand, approximately 1:500-1:900 live male newborns have this complement (3, 9, 24, 39) but very few seem to come to clinical attention; on the other hand, surveys of institutions for the retarded (6, 18), the mentally ill (2, 12, 16, 22), the criminally insane (5, 9, 18, 28, 30) and the aggressive offender (11, 21, 23, 26, 38) have all shown a higher incidence of the XYY complement than would be expected in the general population. Some investigators (4, 15) have tried to explain these individuals, in mental institutions and prisons, on the basis of their being tall and therefore more likely to be considered as dangerous by judges and psychiatrists. Other authors have described them as having more immaturity, emotional lability, and criminality than the normal population (44), and even more emotional instability and impulsivity than other inmates (30). Clinical case histories in the literature generally focus on the unusual and pathological, and one cannot overlook the need for genetic population studies to assess to what extent various sex chromatin abnormalities occur without manifest psychopathology. It was not until 1961 that Sandberg (35) described an XYY man. This was followed in 1965 by Jacobs (18) who reported seven XYY cases among 197 institutionalized 'Manuscript received December 1976; revised September 1977. lLecturer, Dept. of Psychiatry, University of Toronto; Staff Psychiatrist, Clarke Institute of Psychiatry, Toronto, Ont. Can. Psychlatr, Assoc. J. Vol. 22 (1977)
F.
HOFFMAN,
M.D.1
hard-to-manage retarded inmates of a Scottish maximum security hospital (3.6%). All seven were significantly taller than average but had no other physical abnormalities, and none were psychotic. Price and Whatmore (30) added two more cases from the 119 "mentally diseased" dangerous patients from the same hospital (1.9%) for a total of nine XYY patients among 340 criminal, mentally retarded or diseased patients. All of these cases were unusually tall and in fact 30 percent of the men who were 6 feet tall, or more, had double Y chromosomes. Eight were mentally retarded. Comparing all nine cases to a control group of other inmates with severe personality disorders, the authors found the XYY patients were more emotionally unstable, and unable to tolerate even the mildest frustration. They were first convicted at age 13, compared to the control age of 18. Two of the nine had committed sexual assaults against young children; one of the patients showed some schizophrenic features. Jacobs also estimated a population incidence of 0.14 percent by examining 3,500 consecutive male births and pointed to the much higher rate of the XYY chromosome complement in the institutional population. Because of Jacobs' initial finding of an association between the XYY syndrome and aggressive, impulsive, tall men, other investigators began to screen institutional populations and to identify more and more cases. Some authors have described cases which were discovered by screening groups of the criminally insane. In 1966, Casey (5) screened patients over six feet tall in a variety of settings for the criminally insane and found 12 XYY men among 50 tall,
447
448
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
mentally retarded men retained for antisocial behaviour (24%), 4 XYY men among 50 tall, mentally ill patients detained because of antisocial behaviour (8%), and 2 XYY cases among 24 tall criminals (8%). He concluded that, "an extra Y chromosome has a part to play in antisocial behaviour even in the absence of mental subnormality. " In an institution for sex offenders and mentally ill offenders with a total population of 1,400 inmates, Melnyk (22) found 9 XYY individuals (4.5%) among the 200 patients who were six feet, or more, in height. Of 79 mentally disordered sexual offenders he found seven XYY men (8.9%), and of 121 mentally ill, criminally insane, tall men he found two XYY men (1.6%). The seven sexual offenders had committed female pedophilia, aggressive voyeurism, aggressive homosexuality, rape sadism and masochism, male pedophilia and attempted rape, forcible rape, and child molesting. The two patients who were criminally insane had committed vagrancy and car theft, and homosexuality with aggressive acting out. Melnyk points out that "the incidence of 47, XYY in tall, mentally disordered sexual offenders (8.9%) is approximately eight times that of a comparable group of mentally deficient males, and approximately eightynine times the incidence (0.1%) in the general population." Then in 1969, Daly (9, 10) examined all men who were over six feet tall in four maximum security hospitals in Wisconsin, Michigan, Indiana and Ohio and found 10 XYY patients by chromosome analysis, Of these, three were mentally retarded, three were sociopathic, three were schizophrenic and one was "difficult to diagnose because of evidence for a progressive organic disease of the central nervous system." Because of Jacobs' finding that most of the XYY men in a maximum security hospital were mentally retarded, other researchers screened institutions of the mentally retarded, as well as prisons and other institutions. In 1968, Court Brown (7) added another 15 cases who were detected by chromosome studies of newborn babies and of the populations in prisons and hospitals for the mentally subnormal and for mental
Vol. 22, No.8
disease. Three of the cases were discovered in a criminal and delinquent population; two were in mental hospitals, as a result of minor crimes and a lifelong pattern of chronic drifting vagrancy; eight were located in hospitals for the mentally subnormal; one was a newborn baby and the last was a 34 year-old man with a normal psychosocial development who had suffered a stroke. Ricci (33) also described one XYY retarded boy who was tall. Kelly (19) reported a 24 year-old mentally retarded male with a history of seizures, emotional disturbances and suspected arson. Rainer (32) investigated a pair of monozygotic twins with an XYY complement who were both impulsive destructive and yet docile and unassertive when not stressed. In 1974, Clarke (6) described five XYY individuals at the special school in Aberdeen. Their range in I.Q. was from 52-70 and all showed poor motor control and impulsivity. Thus, whether investigators found the XYY subjects in institutions for the criminally insane or for the mentally retarded, the men were most commonly described as impulsive aggressive and antisocial. Meanwhile other investigators screened tall patients in mental hospitals. Forsmann (12, 13) described 12 XYY men from a university psychiatric hospital and research centre and found five with a criminal record. All five criminals were slightly retarded, while three of the seven XYY men without a criminal record were retarded and the other four were normally gifted. Three of the five institutionalized males without a criminal record had a "chronic psychosis", one a chronic obsessional state, and one chronic hypomania and addiction to alcohol. Forsmann concluded that "an abnormal sex chromosome complement may have an unfavourable effect on mental health. It may contribute to the development of the functional psychoses and may even influence such a complex variable of behaviour as our ability to adapt ourselves to the laws of the community. " On the other hand he acknowledged that the XYY complement is "compatible with complete mental health and normal intelligence like the case published by Wiener and Sutherland in 1968." Similar
December, 1977
Xr'Y CHROMOSOME COMPLEMENT
449
cases were more recently described by Berg sexual assault. Welch (41) also described a and Lowy (3), and Stenchever and MacIn- tall "defective delinquent" who had this tyre (36). same chromosome complement. In 1969, In 1969, Abdullah (1) reported a 26 Wiener and Sutherland (43) described four year-old man, who had been a behaviour XYY men that they found by screening tall problem since the age of 14 months, men in a Melbourne prison - all four were characterized by hyperactivity and destruc- diagnosed as psychopaths and only one was tive violent behaviour. At age 13 he had retarded. In 1972, Borgaonker and colbegun to hallucinate and have delusions and leagues reviewed the charts of 20 XYY continuously rambling speech. Since that males at the Chromosome Laboratory of time he had spent most of his life in mental Johns Hopkins. Eleven of these were tall hospitals or institutions for the criminally men, ten of whom were inmates of a penal insane. His full scale I.Q. was 83. The institution; the remaining nine cases were diagnosis of chronic schizophrenia was juveniles, four of whom were above the 90th made. percentile range for height. Three of the nine Akesson (2), in 1969, reported screening juveniles had been institutionalized - two as 117 tall men (over 6 feet) admitted to delinquent and one as emotionally or menordinary mental hospitals in Sweden and tally disturbed. The remaining six were found four XYY men. One of these patients referred because of behavioural or emotional had a "chronic psychosis", one had mental disturbances although the authors did not deficiency (borderline) and two had a adequately describe or diagnose these. Also, "prolonged neurotic syndrome." Parker no information was given as to the I.Q. (27) surveyed 10 tall patients with aggressive levels of these juveniles. Marinello (21), in 1969, screened 86 tall behaviour from a state hospital and found one patient with an XYY constitution. He men over 163 em (6 feet) in Attica State was tall, obese and mentally retarded. In Prison and found two cases of XYY 1970, Nielson (23,25) reported 12 XYY complement, and 76 tall men from a state patients, three were in a psychiatric hospital, hospital where he found one XYY complefour in a forensic psychiatric ward, four in ment. He also discovered one XYY cominstitutions for criminal psychopaths and one plement in testing 57 tall, male juvenile in a medical ward. Eleven of these patients delinquents. The first XYY case from the (91%) had "major criminality with violation prison is summarized as a schizoid, mentally of the penal code" and 36 percent were retarded giant who had been convicted of violent. The frequency of sexual criminality child molesting. The second was a 24 towards children was 27 percent and of year-old with an aggressive personality and a arson, 27 percent. The average I.Q. was history from age five of being difficult, 96.0. Richards (34) examined a 47 year-old hitting and making sexual advances toward man in a mental hospital who was diagnosed siblings, fighting with peers and teachers, as an aggressive psychopath. He had shown and eventually committing petty larceny at homosexual tendencies and had made ag- age 15, and armed robbery at age 23. The gressive attacks unrelated to his sexual case in the mental hospital was a 13 year-old orientation. Thus, many of the cases found in with a diagnosis of undifferentiated schizothe mental hospital are described as impul- phrenia characterized by auditory hallucinasive, destructive and antisocial and some tions, and flat affect. It is again interesting that this patient had tried to bum down a were also mentally retarded. There are several reports on XYY men house prior to his confinement. In ;970, among the criminal and juvenile delinquent Falek (11) was screening a prison populapopulations. Telfer (37, 38) describes five tion, either over six feet tall or with acne, or XYY men who were found among a criminal both, and found one man with the XYY population - two were adult criminals, two syndrome. It is interesting that his crime was were criminally insane and one was a arson and that he had also undoubtedly been juvenile delinquent who was being held for schizophrenic with blunted affect, "loose
450
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
associative linkages" and "paranoid" ideation since the age of 14. Cowie (8), in 1968, described an eight year-old boy with a history of antisocial behaviour, truancy and conflict with the police and school. He was tall for his age and when investigated was found to have an XYY chromosome complement. Hunter (17), in the same year, found three XYY boys from a delinquent population screened for height over the 90th percentile. The last group of XYY men who have been described in the literature have been discovered by screening normal populations. Zeuthen (44) reported in 1975 that he had found five XYY men among 3,840 men being examined for military service in Denmark. Only those who were very tall (181 em. or above) or who had small testes (12 ml) were chosen for chromosome analysis. The intellectual level was within the normal range in all but one; the mean full scale I.Q. was, however, lower than expected. These men showed poor attention and were "concrete and unreflective." Their educational level was significantly lower than expected; four of the five had had difficulties at school and three had been in special classes for children with behaviour disorders or slight retardation, or both. Three of them were hyperactive, restless, hot tempered and impulsive during childhood. All five grew up in "good homes" and were considered different from their siblings. Two of the five had criminal records, in one case, for petty larceny and theft, and in the other, for theft and wanton destruction. On psychological testing, all five men displayed "immaturity in cognitive style and in emotional adaptation. Emotional lability was present in all cases, resulting in lack of control of aggressive impulses, and all the men also showed unfulfilled need for contact and passivity. " The only other survey of a normal population was undertaken by Valentine, McClelland and Sergovich (39) who found four XYY infants by testing 1,066 unselected newborns. They followed these children for 2 1/2 to 3 1/2 years and reported that three of them appeared to have normal intellect and personality, but one was of
Vol. 22, No.8
borderline intelligence (I.Q. = 82) and by age two was exhibiting "aggressive, defiant, and destructive behaviour." The authors discussed the difficulties in doing follow-up studies and the dilemma as to what parents or guardians should be told, if anything. There are also a few isolated case histories of XYY chromosome complement associated with epilepsy (14), multiple congenital abnormalities (20), abnormal electrocardiograms (31) and genital abnormality (40). Thus, of the 153 cases of XYY chromosome complement that were found in the literature, 135 were discovered within institutions: 47 in maximum security hospitals for the criminally insane; 31 in prisons; 24 in psychiatric hospitals; 18 in the delinquent population; and 15 in institutions for the mentally retarded. An additional nine cases were found by surveys of normal populations (39, 44). The remaining nine are isolated case histories. There are only four XYY men described in the literature who were essentially normal in development (3, 35, 36, 42), and in addition there are three XYY children under the age of 3 1/2 years who had remained normal when last evaluated (39). The remainder of the case histories show a predominance of tall men who are either retarded or, if not, often have had difficulties in school that could be called" learning disabilities" (44). The XYY men reported are often impulsive, aggressive and antisocial and have criminal records beginning at an earlier age than other delinquents or criminals (30). Even six of the nine cases of XYY chromosome complement found in general population surveys (39, 44) showed a proclivity to impulsive destructive and antisocial behaviour. Many of the XYY inmates in prisons or maximum security hospitals were also noted to be retarded (5, 9, 18). The nature of the crime and violence tended to be impulsive and destructive and not well thought out. The charges leading to incarceration varied from murder (22) to vagrancy (5). There were a number of case reports that pointed out the incidence of sexual crimes (20 cases), especially against minors, (16, 18, 21-23) and of arson, (six
December, 1977
Xyy CHROMOSOME COMPLEMENT
cases - 11, 19, 21, 23). Of those cases described in detail there are at least 11 with a diagnosis of schizophrenia or "chronic functional psychosis" (1, 2, 9, 11, 12,29), with an additional four cases of very severe schizoid personality (7, 12,21). In addition, some of the 47 patients found in maximum security hospitals were probably schizophrenic, although the psychiatric description or diagnosis was not included in many of the case reports. The current literature does not indicate that there is any physiological or anatomical abnormality of high incidence other than the prolonged P.R. interval on the ECG (31), and tallness. The following case histories describe patients recently discovered who show certain features similar to those surveyed in the literature. Case 1 This unmarried, 22-year-old plumber's helper had been staying in a psychiatric hospital for five months because of fears that he was going to lose control and hit or kill someone for no reason. He felt nervous and very guilty because he could not control these feelings and wept as he talked of these impulses. For the past two years the patient complained of "increasing difficulty in thinking straight, difficulty in getting along and in talking with other people." He stated that he was anxious when around people and that he had frequent anger outbursts during which he is afraid of losing control. For several years, he tended to isolate himself because of his anxiety and so he would not hurt anyone. On the night prior to admission, he had had an argument with someone in the house where he lived and was afraid he would hit or kill this person, although the patient recognized the triviality of the argument. Past Legal and Psychiatric History: At age 14, he was convicted of raping a 9 year-old girl. At age 15, he was convicted of breaking and entering. He was convicted of assault with a deadly weapon, at age 16, when he displayed a knife during a fight. He had numerous convictions for drinking while under age. At age 17, he was admitted to a psychiatric hospital where he was difficult to manage because of fire-setting on the ward. He did not cooperate in follow-up treatment and for the next few years lived a solitary life, while being on welfare and unemployed. Personal History: Birth and development were
451
apparently normal. Beginning at age five he showed a constant behaviour problem, setting fires and fighting with teachers. He did poorly in school, failing examinations several times in the lower grades. He had always been a loner and felt other people were looking down on him. This was aggravated by his frequent failures at school and by his legal problems, which he thought were widely known in the community. He had few friends and a decreasing interest in sex with it being less now compared to when he was a teenager. The patient said the rape incident was motivated by curiosity rather than "passion". He does not enjoy his occasional sexual feelings now. He has always had difficulty in controlling tension and would frequently punch walls or get drunk to relieve the tension. His work record was poor, with most jobs coming quickly to an end because of his anxiety and impulsive aggressive behaviour. Family History: The patient describes his father as an alcoholic and a very rough man, who would beat him with a belt for no reason. He describes his mother as a good person, but he could not confide in her because she told everything to his father. Neither parent was as tall as the patient. He is the third of five siblings - the others are all doing well with no known emotional or psychiatric problems. No one else in the family has legal problems or has been admitted to a psychiatric facility. Mental State: On examination the patient was very muscular, 6 feet, one inch tall, and weighed 180 pounds. He was unshaven but otherwise neatly dressed, appeared nervous and agitated, and frequently changed his position. On the ward, he would have poor impulse control and kick or punch the wall angrily with little or no provocation. He complained of frequent angry feelings which he found difficult to control. He felt depressed and said that life was not worth living but denied any thought of taking his own life. The patient states that his mind wanders and that he has difficulty in concentrating and organizing his thoughts. He showed blocking and tangentiality of thought and had great difficulty in expressing himself. For example, "Last week I ... its been I don't know what building ... I get paranoid is happening. Its ... I'm confused ... my mind is empty . . . they make it that way." The patient said "a force field is hacking up my thoughts." He also felt at times that people were interfering with his thoughts "shoving them aside" or even "crawling inside." At other times he experienced his thoughts as "floating away" , which left his mind empty.
452
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
The patient states that he heard voices talking to him, calling his name and also commenting on his actions in the third person. He also described hearing high pitched noises from time to time. The patient's memory was intact but his attention and concentration appeared to be somewhat impaired. His ability on tests of similarities, comparisons and proverbs was essentially normal. Investigations: A chromosome karotype showed 47 chromosomes, and the extra "Y' chromosome was confirmed by fluorescence. Tests showed an I.Q. of 106 with little inter-test variability. Despite a normal I.Q., the school reports showed poor progress throughout school. Serial EEGs consistently showed a diffuse dysrhythmia of intermittent 4-7 c.p.s. theta activity of low and medium voltage. Epilepsy, however, and focal abnormalities were ruled out. Course in Hospital: In hospital, his condition responded quickly to Trifluoperazine and Pericyazine. However, the patient remained highly dependent on the security of the social atmosphere of the hospital. He continued to show a very low frustration tolerance, remained explosively angry and would periodically hit walls, windows and furniture. He was preoccupied with fires and on one occasion was found setting a ping-pong ball on fire and watching it bum. He continued to have ruminations, not always unpleasant to him, of raping little girls. With prolonged individual and group psychotherapy, an intensive rehabilitation program and the use of Pericyazine and Thioridazine, the patient's condition improved. Following discharge, he has continued to see the author. He completed a rehabilitation course in the community and has been steadily employed for ten months. The diagnoses are acute paranoid schizophrenia and personality disorder (severe) of the antisocial and passive-aggressive type.
Case 2 This 18 year-old male was referred to a psychiatric hospital by his probation officer. The patient said that for the past 3-4 years he had suffered from extreme nervousness and unhappiness. He felt crowded in by people, felt that they stared at him and talked about him, and that strangers called him "queer" when he walked down the street. The patient tended to isolate himself because of these fears and had no friends. He also complained of lifelong aggressive tendencies over which he had little control. His behaviour became much worse at the age of 12 when his mother left the family. He gave a history of frequent fights in school - he eventually was
Vol. 22, No.8
expelled. In adolescence, fights had occurred in billiard halls, or bars and he quit or lost at least three jobs because of feeling picked on, and because of anger outbursts when he would put his fist through a wall.
Personal History: The patient's birth and early development were normal. From the age of five he has always been a very angry, tough, little boy. In class he was hyperactive and easily distracted. From age 7 or 8, he has hated animals and has often been cruel to cats. He described several incidents where he very much enjoyed setting fire to garbage cans in the neighbourhood, feeling excited while watching the police and firemen arrive. Throughout his school years, he has had difficulties and attended at least five schools before he was 15. When he reached grade 8, at age 15, he was transferred to a vocational school where he continued to get into numerous fights. He had avoided trouble with the police until he was 15 years of age, when he played truant from school and at 16, when he cashed his brother's pay cheque and was put on probation for one year. He had also been charged for shoplifting items which he did not need. He has never had friends, and none of his four jobs has lasted more than a few weeks. When he returned home, he ran off frequently and unexpectedly, and often slept in garages and subway stations. Family History: The patient describes his father as a caretaker, 5 feet 8 inches in height, a quiet man who, however, has serious violent outbursts. The mother is also described by him as being explosive, and he reports, without being able to give details, that she has had "nervous breakdowns". The patient's father told us that his wife deserted the family when the boy was 12 years of age and following that, the patient's behaviour deteriorated with increased truancy and impulsive aggressive behaviour. There is one younger brother who does not have any emotional problems, but who the patient feels deliberately provokes him by always calling him "dummy" . Mental Status: On examination, the patient was 6 feet 4 inches tall and weighed 170 pounds. He appeared much older than his stated age of 18. He was anxious and restless and had difficulty in sitting still. There was a certain grandiosity and bravado in his plans and many discrepancies in his story. There was a lack of logic in his thoughts and his speech was circumferential, almost tangential. However, there was no definite looseness of associations or autistic thinking. The diagnosis was personality disorder with aggressive, antisocial and paranoid features.
December, 1977 Discussion
X'YY CHROMOSOME
The similarities between these two cases and some of the cases described in the literature are quite striking. Both young men were over 6 feet tall (7, 30), impulsive, aggressive and antisocial, to a degree that they resemble the criminal populations studied by Falek (1 I), Nielsen (23, 26) Telfer (38) and Wiener (43). This abnormal behaviour began in both patients at a very early age and they therefore resemble the delinquent populations studied by Borgaonkar (4), Cowie (8) and Marinello (21). Both patients were convicted of offences prior to age 18, similar to the patients of Price (30). In addition both were quite suspicious and even paranoid toward the environment, and the patient in Case 1 suffered from an acute paranoid schizophrenia illness that is similar to the cases described by Abdullah (1) and Marinello (21). Whether there is any connection between this type of acute psychotic illness and the chronic psychosis also described in the literature (2, 9, 12) is unknown. The (Case 1) patient had been convicted of rape at the age of 14 and therefore shows a similarity to the 20 cases of sexual crimes among XYY men described in the literature (16, 18, 21, 37). Although he was of normal intelligence, he did not progress well in school (44), and the patient in Case 2 had no better than borderline intelligence similar to the retardates (6, 7, 19, 33) and retarded criminals (5, 7, 12,23,41). Other similarities include the preoccupation with fire (11, 21, 23) and the normal anatomy and physiology except for tallness. It must be recognized that most of the cases described in the literature were found by screening the biased or abnormal populations of prisons, psychiatric hospitals and institutions for the retarded. It is not known yet whether any of this pathology is more common in XYY men than in the normal population. However, from the nine XYY cases found by the general population surveys of Valentine (39) and Zeuthen (44), at least six have had a grossly abnormal psychosocial development with difficulties in school, restlessness and impulsivity, criminal convictions, lack of control of aggres-
453
COMPLEMENT
siveness and emotional lability. The three "normal" XYY infants followed by Valentine were not, in fact, followed past the age of 3 1/2 years and their future development may be in doubt. Because there are a few normal XYY cases reported and because very few of the XYY men come to medical and legal attention, it is unlikely that this chromosome complement is a sufficient and necessary cause for the described psychopathology. It is also apparent that genetic influences in the criminal and mentally ill population may be underestimated. Investigation of tall, impulsive, aggressive men with criminal records beginning at an early age and involving arson and sexual offences, as well as persons with a paranoid and schizoid life style, may well reveal an XYY chromosome complement. Because so many of the cases seem to follow a pattern, it is possible that this chromosome pattern predisposes to an abnormal personality development which becomes manifest only if certain abnormal environmental influences are present. In this context, the impulsive, aggressive parents of these two patients could be important. However, if we are to further elucidate the phenotype of an XYY genotype, we will need to screen and test a very large segment of the normal population for sex chromatin abnormalities or else test newborns and do a follow-up study to assess their personality and behaviour. The medical and ethical problems of such a study has been discussed by Valentine (39). Summary
Two cases with XYY chromosome complement are presented and compared with 153 cases described in the literature. Some patients with abnormalities of personality development and manifest psychopathology may have sex chromosome abnormalities and should be studied further for the interaction of genetic and environmental factors in personality development. In particular, tall, schizoid impulsive men with a history of criminality, arson or sexual offences should be screened for this particular genetic configuration. Because of the biased manner by which most of the cases
454
CANADIAN PSYCHIATRIC ASSOCIATION JOURNAL
are found, It IS not yet known whether an X'r'Y genotype results in a characteristic phenotype. What is needed is a genetic study of a normal population and a prospective study of newborns with various genotypes which would, unfortunately, create complex research and ethical problems. Acknowledgements
The author is grateful to Drs. F.H. Lowy and E. Persad for bringing the second case to his attention, and to Mrs. Lillian Scarpellino for typing this manuscript.
References
1. Abdullah, S.: Extra Y chromosome and its psychiatric implications. Arch Gen Psychiatry, 21: 497,1969. 2. Akesson, H. O. et al: Gross chromosomal errors in tall men admitted to mental hospitals. Acta Psychiatr Scand, 45: 37, 1969. 3. Berg, J. M., Lowy, F. H.: XYY syndrome: a comment. ModMed Can, 30: 692,1975. 4. Borgaonkar, D. S., Unger, W. M., Moore, S. R., Crofton, T. A.: 47, XYY syndrome, height and institutionalization of juvenile delinquents. Br J Psychiatry, 120: 549, 1972. 5. Casey, M. D., et al: YY chromosomes and antisocial behaviour. Lancet, 2: 859, Oct. 1966. 6. Clarke, D. F., Johnson, A. W.: XYY individuals in a special school. Br J Psychiatry, 125: 390, 1974. 7. Court Brown, W. M., Price, W. H., Jacobs, P. A.: Further information on the identity of 47, XYYmales.BrMedJ, 2: 325,1968. 8. Cowie, J., Kahn, J.: XYY constitution in a prepubertal child. Br Med J, 1: 748, 1968. 9. Daly, R. F.: Mental illness and patterns of behaviour in 10 XYY males. J Nerv Ment Dis, 149: 318, 1969. 10. Daly, R.F.: Neurological abnormalities in XYY males. Nature, 221: 472,1969. 11. Falek, A.: An attempt to identify prisoners with an XYY chromosome complement by psychiatric and psychological means. J Nerv Ment Dis, 150: 165, 1970. 12. Forssman, H.: The mental implications of sex chromosome aberration. Br J Psychiatry, 117, 353, 1970. 13. Forssman, H., Hambert, G.: Chromosomes and antisocial behaviour. Lancet, 282, July 1966.
Vol. 22, No.8
14. Forssman, H.: Epilepsy in an XYY man. Lancet, 1389, June 1967. 15. Hook, E. B.: Behavioural implications of the human XYY genotype. Science, 179: 139, 1973. 16. Hope, K.: Psychological characteristics associated with XYY sex-chromosome complement in a state mental hospital. Br J Psychiatry, 113: 495-8, 1967. 17. Hunter, H.: Chromatin-positive and XYY boys in approved schools. Lancet, 816, April 1968. 18. Jacobs, P. A., Bronton, M., Melville, M. M.: Aggressive behaviour, mental subnormality and the XYY male. Nature, 208: 1351,1965. 19. Kelly, S., Almy, R.: Another XYY phenotype. Nature, 215: 405,1967. 20. Kosenow, W., Pfeiffer, R. A.: YY Syndrome with multiple malformations. Lancet, 1375, June 1966. 21. Marinello, M. J., Berkson, R. P.,: A study of the XYY syndrome in tall men and juvenile delinquents. JAMA, 208, 321, 1969. 22. Melnyk, 1.: Sex chromosome XYY phenomenon occurrence examined in mental patients. Nature, 224: 369,1969. 23. Nielsen, J.: Criminality among patients with Klinefelter's syndrome and the XYY syndrome.BrJ Psychiatry, 117: 365,1970. 24. Nielsen, J.: Klinefelter's syndrome and the XYY syndrome. Acta Psychiatr Scand, Supplement, 209, 1969. 25. Nielsen, J.: Prevalence and a 2 1/2 years incidence of chromosome abnormalities among all males in a forensic psychiatric clinic.BrJ Psychiatry, 119: 503-12,1971. 26. Nielsen, J., Tsuboi, T., Sturup, G., Romano, D.: XYY chromosomal constitution in criminal psychopaths. Lancet, 576, Sept. 1968. 27. Parker, C. E., Melnyk, J., Fish, C. H.: The XYYsyndrome.AmJMed, 47: 801, 1969. 28. Polani, P. E.: Abnormal sex chromosomes and mental disorder Nature, 223: 680, 1969. 29. Price, W. H., Strong, J. A., Whatmore, P. B., McClemont, W. F.: Criminal patients with XYY sex-chromosome complement. Lancet, 565, March 1966. 30. Price, W. H. Whatmore, P. B.: Behaviour disorders and patterns of crime among XYY males identified at a maximum security hospital. Br Med J, 1: 533, 1967. 31. Price, W. H.: The electrocardiogram in males with extra Y chromosomes. Lancet, 1106, May 1968. 32. Rainer, J. D., Abdullah, S., Jarvik, L. F.:
December, 1977
33. 34. 35. 36. 37. 38.
39.
40. 41.
42. 43.
455
Xyy CHROMOSOME COMPLEMENT
XYY karyotype in a pair of monozygotic twins: a 17 year life history study. Br J Psychiatry, 120: 543, 1972. Ricci, N., Malacame, P.: An XYY human male. Lancet, 721, March 1964. Richards, B. W., Stewart, A.: The YY syndrome. Lancet, 984, April 1966. Sandberg, A. A., Koepf, G. F., Ishihara, T., Hauschka, T. S.: An XYY human male. Lancet, 488, Aug. 1961. Stenchever, M. A. MacIntyre, M. N.: A. normal XXY man. Lancet, 680, 1969. Telfer, M. A., Baker, D., Longtin, L.: YY syndrome in an American Negro. Lancet, 95, Jan. 1968. Telfer, M. A., Baker, D., Clark, G. R., Richardson, C. E.: Incidence of gross chromosomal errors among tall criminal American males. Science, 159: 1249, 1967. Valentine, G.H., McClelland, M. A., Sergovich, F. R.: The growth and development of four XYY infants. Pediatrics, 48: 583, 1971. Vignetti, P., Capotorti, L., Ferrante, E.: XYY chromosomal constitution with genital abnormality. Lancet, 588, Sept. 1964. Welch, J. P., Borgaonkar, D. S., Herr, H. M.: Psychopathy, mental deficiency, aggressiveness and the XYY syndrome. Nature, 214: 500, 1967. Wiener, S., Sutherland, G.: A normal XYY man. Lancet, 1352, 1968. Wiener, S., Sutherland, G. et al: XYY males in a Melbourne prison. Lancet, 150, Jan. 1968.
44. Zeuthen, E., Nielsen, J., Hansen, M., Christensen, A.: A psychiatric-psychological study of XYY males found in a general male population. Acta Psychiatr Scand, 51: 3-18, 1975.
Resume On presente deux cas de complement chromosomique XYY et on les compare avec 153 cas decrits dans le litterature. Certains patients montrant des anomalies du developpement de la personnalite avec psychopathologie evidente peuvent avoir des anomalies des chromosomes sexuels, ce qui demande une etude plus en profondeur de I' interaction des facteurs genetiques et de I' environnement sur le developpement de la personnalite. Plus particulierernent, on devrait faire le depistage de cette configuration genetique chez des hommes grands, irnpulsifs et schizoides, ayant une histoire d'offenses criminelles, sexuelles ou incendiaires, Cependant, vu la facon selective dont la plupart des cas ont ete detectes, on ne sait pas encore si un genotype XYY correspond a un phenotype caracteristique. Il serait done necessaire de proceder a une etude de genotype chez la population normale et aune etude prospective chez les nouveaux-nes lesquels presentent des genotypes varies. Toutefois, ceci exigerait des recherches complexes et poserait des problemes d'ethique.
Association des Psychiatres du Canada Canadian Psychiatric Association DEMANDE D'ADMISSION Monsieur Ie Secretaire, Association des psychiatres du Canada, Bureau 103, 225, rue Lisgar, Ottawa, Canada K2P OC6 Je demande mon admission comme membre actif, membre associe, membre correspondant, membre affilie, membre en formation ou membre inactif de l'Association des psychiatres du Canada. (Biffer les mots qui ne s'appliquent pas). Je suis autorise exercer dans la province de . _
a
Signature
Date
Nom et prenoms: (lettres moulees) . .
.
Adresse de bureau
.
Domicile:
.
...._.
.
. .
_ .
Date du diplome en medecine et endroit:
.
Certificat du College Royal des medecins et chirurgiens du Canada:
Qui
Certificat d'un bureau provincial: Nom du bureau provincial:
_ _
.
Date de naissance:
.
. Non
0 0
. ....
_
0 0
.
_ Date
---.-.-.--------------------------------
.
.__.
_ _ _
Bref expose de la formation et de l'experience en psychiatrie: Genre de travail actuel en psychiatrie: Si vous etes inscrit l'universite
aun cours de specialisation, mention de
.__._.
_
a
Je m'engage me conformer aux Statuts et Reglements ..__... de l'Association des psychiatres du Canada
_
Signature du candidat
Recommandation: (A etre signee par deux MEMBRES ACfIFS de I'Association)* (1) _ Nom en lettres moulees
Signature
(2)
Nom en lettres moulees Signature ·Aucune recommandation exigee pour un membre correspondant,
,
REGLEMENTS
I. 2. 3. 4. 5. 6.
Les membres actifs versent une cotisation annuelle de $125.00. Les membres associes versent une cotisation annuelle de $30.00. Les membres correspondants versent une cotisation annuelle de $30.00. Les membres en formation versent une cotisation annuelle de $20.00. Les membres affilies versent une cotisation annuelle de $25.00 Un mari et sa femme qui sont tous deux membres actifs versent une cotisation annuelle reunie de $240.00. 7. La cotisation comprend I' abonnement a la Revue de I' Association des psychiatres du Canada et a la Lettre-nouvelles de I'A.P.C. 456
