‘Clinical Genetics I99 I : 39 409-41 8

Personality in 47,XXY males during adolescence

C. NETLEY Department of Behavioural Science, University of Toronto, Ontario, Canada

The responses to the High School Personality Questionnaire and the Millon Adolescent Person- ality Inventory of a series of neonatally identified 47,XXY adolescent males and age-matched controls were compared. The extra X males’ results indicated that they were more assertive, impulsive and prone to conflict than controls. Individual differences in these characteristics among the extra X cases had little relationship to quality of parenting but were related to indices of pubertal development.

Received 2 October, accepted for publication 28 November 1990

Key wora!r: personality; puberty; testostcrone; 47,XXY.

There is now a sizeable body of evidence derived from longitudinal studies of neo- natally identified 47,XXY males which indi- cates they tend to differ from chromosomal- ly normal males in behavioural development during childhood (Netley 1986, Walzer et al. 1978). The best established observations in this regard are that extra X males are frequently subject to delays in language de- velopment and reading difficulties prior to adolescence (Bender & Berch 1990, Stewart et al. 1982). There are additional data that point to anomalies in social-emotional func- tioning among 47,XXY males during this stage of maturation, much of it also sug- gesting that they are passive, inactive and socially withdrawing even from infancy (Bancroft et al. 1982, Netley 1988, Walzer et al. 1978).

This report is concerned with several dif- ferent but related goals. The first is to de- scribe the social-emo tional characteristics of a group of neonatally identified extra X males at several ages during adolescence. It is an area worthy of examination since the adult and clinical literature suggests that

anti-social and/or psychopathological be- haviours rather than the passivity and inac- tivity evident earlier may begin to emerge in extra X males during this time (Berch & McCauley 1990, Roy 1984). The second is- sue examined in this report is concerned with the degree to which family dysfunction is associated with maladjustment in extra X males during adolescence, as suggested by the data of Bender et al. (1990). The final issue studied is the degree to which par- ameters associated with the initiation of pu- berty and its course are related to social- emotional functioning in extra X males. This is a plausible area of enquiry because such relations have been reported among normal males (Paikoff & Brooks-Gunn 1990) and, further, anomalies in sexual maturation among 47,XXY males are not uncommon, particularly after mid-adoles- cence (Topper et al. 1982).

Subjects and Methods

Subjects Twenty-nine extra X males, identified by

410 N E T L E Y

means of newborn screening (Bell & Corey 1974) and followed during the course of a longitudinal examination of growth and development (Stewart et al. 1986), were studied. Twenty-six had nonmosaic 47,XXY karyotypes and three 47,XXY/46,XY karyotypes. Male controls, presumed to be chromosomally normal, were recruited from Toronto high schools and matched with cases on the basis of age. These individuals completed the self-report measures of per- sonality used with probands but their pub- ertal development was not assessed. Several sets of control data were collected in order to match probands who were examined at a number of different ages.

Method The general methodology employed in the study of the extra X series consisted of an- nual examinations conducted as close as possible to each case's birthday. While all aspects of functioning were not assessed at each visit, an effort was made to collect blood samples annually for radioimmuno- assays of serum testosterone levels and for physical examinations to provide Tanner- based ratings of pubic hair development (Tanner 1962).

Social-emotional functioning of cases and controls was assessed using two self-report questionnaires: the High School Personality Questionnaire (HSPQ, Cattell & Cattell 1969) and the Millon Adolescent Person- ality Inventory (MAPI, Millon et al. 1982). The HSPQ was administered to the 29 extra X cases at a mean age of 16.6 years (S.D. = 0.91). Twenty-six male controls completed the HSPQ at a mean age of 16.5 years (S.D.= 1.27) (t10.33, NS). The MAPI was completed by 26 extra X males at a mean age of 17.6 years (S.D.xl.13) and by 19 male controls at a mean age of 17.1 years (S.D. = 0.97) (t = 1.59, NS). Eighteen extra X males completed the MAPI a second time at a mean age of 18.6 years (S.D.=O.86).

No age-matched control data were available for comparisons with these observations. All MAPI protocols were computer scored using the Guidance Interpretive Report of National Computer Systems (1 982).

The quality of parental management in the proband series was rated independently on a 5-point scale [ 1 =very poor, 5 =excel- lent) by the writer and by a paediatrician (Dr. D. A. Stewart), both of whom had extensive knowledge of the social and domestic cir- cumstances of each case. These ratings were done without reference to the questionnaire results. The correlation between ratings was highly significant (r = 0.77, p < 0.01) and agreement between scale assignments was achieved in 75% of judgements. When rat- ings differed, they were averaged.

Results

Physical Parameters The age at which puberty began was assess- ed by noting the age at which the P2 stage of pubic hair development was first observ- ed. A further index of pubertal change was arrived at by assessing the age at which the P4 stage of pubic hair growth was attained. (It is recognized that these were somewhat imprecise measurements since examinations were conducted at 12-month intervals. However, any errors of measurements would have been unsystematic across cases and unlikely, therefore, to distort corre- lational analyses with behavioural measures within the extra X series.) The rate of puber- tal change was assessed by computing the linear regression of age on stage of pubic hair development for each case. This yielded a slope and intercept value for each extra X male. This slope was interpreted as a measure of the slowness of pubertal change since cases with high slopes would be older than low slope individuals when they reached any stage of pubic hair growth after P1, which was arbitrarily set at age 10 for

P E R S O N A L I T Y A N D 4 7 , X X Y 41 1

Table 1

Mean values ( f S.D.) of P2 age, P4 age, slope and intercept of age on stage linear regression and testosterone level (nmollL) from 12 to 18 years of extra X cases (shown as T(12), T(13)

8tC.)

Mean (s.12) N

12.83 (1.24) 14.66 (0.86)

1 S9 (0.45) 9.15 (0.97) 2.20 (2.74) 4.70 (4.87) 9.93 (9.76)

12.76 (5.50)

29 29 29 29 24 26 28 28

These observations are consistent with pre- vious reports on extra X males (Topper et al. 1982). The intercorrelations between these variables are shown in Table 2. Quite reasonably, the intercorrelations between testosterone levels are generally significant if measurements are reasonably proximal in time. As expected, there are negative corre- lations between P2 age and testosterone levels early in puberty and between P4 and testosterone somewhat later. Unsurprising- ly, the slope and intercept correlations are respectively associated with low testoster-

15.26 (5.21) 29 one levels during later adolescence and with the early adolescence markers P2, P4 and 16.32 (5.56) 29

14.44 (4.42) 20 . . . . early testosterone levels.

all cases. The intercept value was interpreted to be an index of age at pubertal initiation. Serum levels of testosterone as determined by radioimmunoassay analyses were con- sidered to provide additional indications of pubertal change. The descriptive statistics for each of these variables are presented in Table 1. Although none falls completely outside normal limits (Gupta et al. 1975, Tanner 1962), the mean testosterone values were below average from about age 15 and more significantly, the mean value at 18 was below the mean values at ages 16 and 17.

Psychological Parameters The mean HSPQ personality scores of pro- bands and controls expressed in terms of stanines with a normative mean of 5 are presented in Table 3. The respective low and high score descriptors for each scale as well as its alphabetic indentifier are shown under the title scale in this table. The difference between the groups in scale B, intelligence, is as expected since a number of investiga- tions have reported lowered intelligence in extra X males (see Netley (1 986) for a sum- mary). The relationship of cognitive func-

Table 2

lntercorrelations between measures of pubertal hair change and testosterone (T) levels from 12 to 18 years in extra X sample

Slope (SL) Intercept (In) P2 P4 T(12) T(13) T(14) T(15) T(16) T(17) T(l8)

In -0.SgUa P2 0.05 P4 0.20 T(12) 0.07 T(13) 0.18 T(14) 0.M T(15) -0.14 T(18) - 0 . 3 p T(17) -0.50- T(18) 0.09

0.73= 0.43uu - 0.34' - 0 . 7 F - 0.45- - 0.21 0.04 0.21

-0.10

0.44u" -0.28' -0.31' -0.66w - 0 . w o.s= -0.46w -0.37" 0.48' 0.65= -0.32' -0.52xu 0.18 0.41" 0.47- -0.23 -0.- 0.37' 0.25 0.31' 0.6luX -0.02 -O.3Su 0.44= 0.08 0.14 0 . 3 p O.7fw

0.02 -0.14 0.28 0.27 0 . 4 P 0.34' 0 . 4 p 0.w"

XXX P<O.Ol, XX P<0.05, X PCO.10

412 NETLEY

Table 3

Mean values ( fS.0.) on HSPQ of probands and controls

t P Scale 47,xxY Controls

A (aloof vs outgoing) 4.8 (1.61) 6.2 (1.9) 2.75 < 0.01 B (low vs high intelligence) 4.0 (1.9) 8.3 (1.9) 4.62 <0.01 C (easily upset vs calm) 6.9 (2.2) 7.5 (1.6) 1.12 NS D (inactlve vs excltable) 5.0 (1.7) 4.7 (1.5) 0.72 NS E (docile vs assertive) 6.7 (2.1) 5.5 (2.2) 1.98 co.06 F (serious M enthusiastic) 4.9 (2.1) 5.0 (1.6) 0.21 NS G (disregards rules vs conscientious) 4.3 (2.0) 5.9 (1.6) 3.04 <0.01 H (shy M socially bold) 5.2 (2.1) 5.4 (2.0) 0.25 NS I (tough-minded vs sensitlve) 5.8 (2.4) 8.3 (2.1) 0.86 NS J (zestful w reflective) 5.9 (1.9) 6.0 (2.3) 0.16 NS 0 (secure M Insecure) 5.3 (2.3) 4.5 (2.2) 0.62 NS Q2 (group dependent vs independent) 6.1 (2.1) 5.2 (2.1) 1.54 NS

Q4 (relaxed vs tense) 5.4 (2.6) 5.0 (1.7) 0.71 NS Q3 (uncontrolled vs controlled) 5.3 (2.2) 6.4 (2.0) 1.77 NS

tioning to sexual maturation is examined separately (Stewart et al., in press) and will not be discussed further in this paper. The remainder of the data reflect the differences between the self-reported social-emotional characteristics of cases and controls. They indicate that during mid-adolescence the extra X males were more aloof, disregarding of rules and assertive than chromosomally normal youth.

The MAPI results for cases and controls are presented in Tables 4, 5 and 6 under this instrument’s three headings; Personality Style, Expressed Concerns and Behavioural Correlates. The values shown are base rate scores with a normative median of 50. These

Table 4

Mean values ( f S.D.) of probands and controls on MAPI personality style scales

Scale

Introversive Inhibited Cooperative Sociable Confident Forceful Respectful Sensitive

47.XXY Controls

44.3 (31.6) 41.6 (26.7) 57.1 (26.6) 48.0 (29.7) 39.7 (23.7) 57.9 (23.3) 45.7 (22.4) 55.9 (30.7) 49.5 (25.0) 58.2 (24.7) 67.7 (22.3) 39.6 (24.8) 48.5 (22.8) 63.0 (17.3) 61.3 (32.3) 42.5 (24.7)

t

0.31 1.26 2.57 1.22 1.17 3.91 2.44 2.21

-

-

P ~~

NS NS

< 0.02 NS NS

<0.01 < 0.02 < 0.04

results, obtained about 1 year after the HSPQ data, are similar in their impli- cations. In terms of the MAPI’s Personality Style scales the extra X males were less co- operative and respectful and more forceful and sensitive than the controls (Table 4). Related qualities are evident in the MAPI Expressed Concerns scales (Table 5 ) with the extra X males expressing greater con- cerns in relation to tolerating social norms, relating to family members and coping with academic requirements. The final category of functioning assessed by the MAPI, Be- havioural Correlates, also yielded similar re- sults. Group differences here (Table 6) indi- cate that the extra X males were more likely than controls to have problems in impulse control, confohng to social norms and in making satisfactory academic progress.

Because the results from a second admin- istration of the MAPI to 18 extra X males at a mean age of 18.6 years (S.D.=0.85) were available, it was possible to examine the consistency and reliability of MAPI data over a I-year period. Tables 7,8 and 9 pres- ent the intercorrelations between MAPI scales and t-tests of differences between scale means at the two points in time. No differences in mean scores were found and

P E R S O N A L I T Y A N D 4 7 , X X Y 413

Table 5

Mean values (*S.D.) of probands and controls on MAPI expressed concern scales

Scale 47,XXY Controls t P

Self-concept 59.2 (27.5) 47.4 (25.5) 1.48 Personal esteem 62.7 (26.0) 57.0 (22.9) 0.18 Body comfort 49.3 (21.0) 47.3 (19.2) 0.33 Sexual acceptance 52.9 (19.6) 46.0 (20.0) 1.14 Peer security 59.1 (20.9) 57.2 (25.6) 0.27 Social tolerance 61.3 (24.4) 33.2 (25.1) 3.75 Family rapport 66.1 (28.1) 42.7 (28.0) 2.77 Academic confidence 58.7 (23.7) 42.5 (23.5) 2.29

NS NS NS NS NS c 0.01 c 0.01 < 0.03

Table 6

Mean values ( f S.D.) of probands and controls on MAPI behavioural correlate scales

Scale 47,XXY Controls t P

Impulse control 60.1 (25.2) 35.5 (18.1) 3.82 CO.01 Societal conformity 59.8 (19.4) 39.5 (18.0) 3.62 CO.01 Scholastic achievement 55.9 (24.6) 39.3 (23.3) 2.30 CO.03 Attendence consistency 50.6 (24.3) 44.0 (18.7) 1.04 NS

all intercorrelations, except for the Social Tolerance Scale, were significant, indicating that the MAPI findings were generally stable and replicable.

Relationships Between Pubertal and Parent- ing Measures and Personatity The issues explored in the following analy- ses were directed towards determining the

Table 7 Mean values (*SO.) of 47,XXY males on sec- ond administration of MAPl’s personality style scales, correlations with first administration of MAPI and t comparisons of first and second

MAPI results

Scale

Introversive Inhibited Cooperative Soclable Confident Forceful Respectful Sensitive

~

Mean ( f S.D.)

43.4 (27.1) 55.5 (33.0) 40.9 (29.2) 52.4 (27.0) 57.8 (28.9) 64.9 (28.2) 56.3 (25.9) 56.4 (34.0)

r t P

0.72 (CO.01) 0.10 NS 0.61 (CO.01) 0.17 NS 0.41 (C0.06) 0.12 NS 0.61 (<0.01) 0.87 NS 0.69 (CO.01) 0.99 NS 0.57 (<0.01) 0.37 NS 0.70 (CO.01) 1.03 NS 0.75 (CO.01) 0.48 NS

relative contributions of the quality of par- enting and pubertal change indices to indi- vidual differences in those social-emotional variables which discriminated extra X cases from controls. (Although no control data were available to compare with the extra X series second MAPI results, these obser- vations are included in the analyses. This seems justified, given their generally signifi- cant relationship to the first set of MAPI results. Moreover, their inclusion provided an opportunity to examine the replicability of associations between the first MAP1 data and the predictor variables).

The correlations between rated quality of parenting, measures of pubertal change (P2, P4 ages of onset, slope and intercept of pub- ertal hair change and testosterone (T) levels from 12 to 18 years) and those HSPQ scales on which extra X males differed from con- trols are presented in Table 10. Tables I t , 12 and 13 present the correlations for each of the three components of the MAPI test which distinguished cases and controls. It

414 N E T L E Y

Table 8 Mean values ( f S.D.) of 47.XXY males on second administration of MAPl's expressed concern scales, correlations with first administration and t comparisons of first and second MAPI results

Scale Mean ( f S.D.) r t P

Self-concept Personal esteem Body comfort Sexual acceptance Peer security Social tolerance Family rapport Academic confldenc

52.8 (30.7) 58.3 (27.1) 44.8 (26.4) 48.2 (22.3) 55.6 (23.2) 58.8 (20.8) 58.5 (28.7)

e 57.6 (28.0)

0.71 (40.01) 0.82 ( CO.01) 0.57 (CO.01) 0.86 (CO.01) 0.64 ( C 0.01) 0.18 (NS)

0.62 (CO.01) 0.64 ( c 0.01)

0.71 0.76 0.80 0.72 0.51 0.37 1.10 0.14

NS NS NS NS NS NS NS NS

can be seen that the rated quality of parent- ing was significantly related to Scale A (aloof vs outgoing) on the HSPQ and to the Cooperative, Forceful and Social Tolerance dimensions of the MAPI at one or other test administration. However, the corre- lations are modest and less consistent than are those which involve some of the meas- ures reflecting physical maturation. Al- though the number of significant corre- lations between pubertal measures and so- cial-emotional functioning is sizeable it could be argued that they have emerged from a large data set and, in some cases, are due to chance. In order to minimize this possibility, it was decided in the case of the HSPQ results to adapt the stringent p value of 0.01 for significance. The MAPI data were treated differently and in their case particular significance was ascribed to as- sociations if a p criterion of 0.05 was ex- ceeded with results from either the first or

second test administration and a replication observed at the p = 0.10 level or less in the other correlation. Applying these standards, it can be seen in the case of the HSPQ data that Scale E (docile vs. assertive) was positively related to testosterone levels at 16. In addition, Scale G (disregards rules vs. conscientious) was positively related to both P4 age and the slope of pubertal hair devel- opment and negatively related to testoster- one levels at 14 years. The associations are generally consistent among themselves and imply that tendencies towards assertiveness and a disregarding of rules were most evi- dent in those cases with relatively quick pu- bertal development and high levels of serum testosterone.

The MAPI data carry implications similar to those evident in the HSPQ results. Using the stringent criteria for significance de- scribed above, the following scales were re- lated to the slope measure of pubertal hair

Table 9

Mean values (&S.D.) of 47,XXY males on second administration of MAPl's behavioural correlates scales, correlations with first administration and t comparisons of first and second MAPI results

~~

Scale Mean (f S.D.) r t P

impulse control 52.8 127.6) 0.73 (CO.01) 0.89 NS Social conformity 55.1 (24.3) 0.58 (C0.01) 0.88 NS Scholastic achievement 47.4 (28.0) 0.69 (40.01) 1.04 NS Attendance consistency 46.3 (25.0) 0.79 (CO.01) 0.57 NS

P E R S O N A L I T Y A N D 4 7 , X X Y 415

Table 10

Correlations of parenting quality, pubertal hair indices and testosterone levels with HSPQ scales on which extra X males and controls differed

HSPQ Scale

A (Aloof vs Outgoing) E (Docile vs Assertive) vs Conscientious) G (Disregards rules

Parenting quality 0.45- 0.05 0.08 P2 - 0.25' 0.24 0.10 P4 - 0.03 - 0.2v 0.43= Slope O.3lu - 0.25' O M u x Intercept - 0.32' - 0.02 -0.10

-0.19 0.15 - 0.23 ~ ( 1 3 ) 0.25' 0.25' -0.13 ~ ( 1 4 ) 0.22 0.20 - 0 . w T(15) 0.11 0.20 - 0.23 T(161 - 0.04 0.54= - 0 . w

T ( W 0.24 -0.18 - 0.23 T(17)

xxx p<O.Ol, xx p<o.o5. x pxo.10

-0.31' 0.27' - 0.3V

change; Forceful (negative), Respectful (positive), Family Rapport concerns (nega- tive), Impulse Control dificulties (negative). In addition, testosterone levels at various ages were related to many of the same scales or related ones (Respectful and testosterone at 14 (negative), Impulse Control difficulties and testosterone at 14 and 17 years (posi-

tive), Societal Conformity difficulties and testosterone at 14,years (positive). Only in the case of the MAPI's Social Tolerance expressed concern scale did rated quality of parenting meet the criteria employed to mark particular significance. It would seem, therefore, (as was the case with the HSPQ results) that self-reported tendencies

Table 11

Correlations of parenting quality, pubertal hair indices and testosterone levels with MAPI person- ality style scales on which extra X males and controls differed on Test 1. Test 2 results also shown.

-~

Cooperative Forceful Respectful Sensitlve Test number 1 2 1 2 1 2 1 2

0.16 0.09

- 0.07 0.20 - 0.04

-0.11 - 0.25 - 0.35'

0.m - 0.3F -0.16 - 0.08

0.41' -0.10

0.18 0.13

-0.12 0.15 0.1 1

-0.18 0.11 0.11

-0.01 0.12

- 0 . w 0.25 0.13

- 0.47= 0.soUX

-0.11 -0.11

0.17 - 0.02 0.18 0.23 - 0.03

- 0.39 -0.01 -0.22 - 0.36' 0.19

-0.18 - 0.05

0.29 0.20 0.10 0.15 - 0.01

0.17 -0.05 0.44= 0.33'

-0.12 -0.17 -0.10 - 0.36-

0.06 -0.12 - 0.24 -0.09

0.19 - 0.02 0.26 0.47- - 0.26

-0.21 - 0.09 - 0.56= -0.16 - 0.43" -0.51=

0.W

-0.27 0.06 - 0.27

-0.27 0.13 0.24 0.05 0.12

-0.19 0.07 0.26 - 0.01

-0.11 -0.25 -0.45'' - 0.30 -0.09

0.43' 0.31 0.W' 0.26 0.32' 0.38X 0.25

XXX p<O.O1, XX pc0.05, X pC0.10.

416 N E T L E Y

Table 12 Correlations of parenting quality, pubertal hair indices and testosterone levels with MAPI ex- pressed concern scales on which extra X males and controls differed in Test 1. Test 2 results also

shown.

Social tolerance Family rapport Academic Test number 1 2 1 2 1 2

- 0.43" 0.18 0.08

-0.17 0.24 - 0.04 0.03 0.01 0.00 - 0.08 - 0.01 - 0.22

- 0.w - 0.27 - 0.23 - 0.35' - 0.02 0.10 0.08 0.13

-0.15 0.1 1 0.43" 0.35'

- 0.26 0.07 - 0.3V

-0.29' 0.15 0 .W 0.05 0.20 - 0.05 0.08 0.26 0.08

- 0.29 - 0.04 -0.41' - 0.57x"

0.26 0.21 0.09 0.42= 0.26 0 . 5 p 0.soUX 0.31

- 0.30 0.08

-0.31' - 0.27' 0.14 0.15 - 0.03 0.19 0.14 0.03 0.20

-0.08

-0.02 0.00 - 0.47"

- 0.34' 0.13 0.38' 0.15 0.5p 0.37' 0.54'" 0.56"' 0.31

XXx p<O.Ol, XX p <0.05, X p<O.lO.

towards assertiveness, difficulties in relation to family relationships, impulse control and conforming with social norms within the extra X series were most pronounced in in- dividuals with relatively rapid pubertal de- velopment and higher levels of testosterone. The relationship of parenting quality to these characteristics was quite limited.

Discussion

The present results are distinctly different from those obtained in earlier examinations of this series of extra X males. Prior to puberty they presented little evidence of problematic behaviour, tending instead to be quiet, docile, easily managed boys (Net-

Table 13

Correlations of parenting quality, pubertal hair indices and testosterone levels with MAPI behav- ioural correlates scales on which extra X males and controls differed on Test 1. Test 2 results are

also shown.

Impulse Control Societal conformity Scholastic achievement Test number 1 2 1 2 1 2

-0.23 -0.35 -0.02 -0.20 -0.33' -0.32 -0.34" -0.41"

0.13 0.05 0.24 0.24 0.17 0.21 0 . 3 p 0.59xx" - 0.03 0.25 0.19 0.34' 0.sx 0.45" 0.22 0.31

- 0.33 0.04

-0.21 - 0.32 0.17 0.14 0.11 0.34' - 0.04 0.16 0.31' 0.20

- 0.31 - 0.29 - 0.20 0.10 - 0.47" - 0.31 - 0.38' - 0.24 0.02 0.11 0.21 0.26 0.22 0.05 0.53"' 0.24 0.32' -0.09 0.36' 0.03 0.35' 0.28 0.11 @.a

0.05 -0.12 - 0.41 xx - 0.34'

0.03 0.31 0.20 0.57u" 0.23 0.33X 0.47u 0.41'

xxx p<o.1, XX p<o.05, x p<O.lO.

P E R S O N A L I T Y A N D 4 7 , X X Y 417

ley 1988, Stewart et al. 1982). The data re- ported here, obtained during adolescence, indicate something different, with the extra X males showing significant tendencies towards assertiveness, defiance, poor im- puke control and conflict in relation to so- cial norms and family relationships.

What can be inferred about the determi- nants of individual differences in these dis- tinctive socialemotional characteristics of extra X males during adolescence? One poss- ible contributory factor, the quality of par- ental management, appears to have relatively little importance. In contrast, the variables reflecting pubertal development, pubic hair changes and testosterone levels, have size- able and fairly consistent relationships with those traits which distinguish the extra X series from controls. If it is assumed that tes- tosterone levels are primarily responsible for variations in the pubertal hair indices of physical maturation, these findings are not unexpected since positive correlations be- tween testosterone levels and aggressiveness as well as low frustration tolerance in chro- mosomally normal male adolescents have been reported (Olwens et al. 1980). Thus, in both extra X and chromosomally normal males relatively high levels of testosterone are associated with antisocial or aggressive characteristics. However, beneath this simi- larity lies a dserence. This can be inferred from two points: 1) testosterone levels in extra X adolescent males are lower than those of age-matched normal males (Topper et al. 1982); 2) the antisocial or aggressive qualities of extra X adolescent males are, on the basis of the data presented here, stronger than those of the chromosomally normal males of the same age. It follows then that the behavioural implications of testosterone levels are different in the two groups. This is clearest in the case of levels which for the chromosomally normal are average or slightly below average, since these would be associated with prosocial behaviour among

normals and antisocial behaviour among extra X males. This difference in the apparent sigdkance of testosterone when expressed in absolute terms is unexplained. However, it suggests that a complete account of the causes of the behavioural differences be- tween adolescent extra X males and age- matched chromosomally normal males must make reference to factors beyond those examined in the present study. It is conceiv- able, for example, that the pubertal anom- alies which characterize extra X males during mid to late adolescence somehow affect brain maturation and, as a result, behavioural de- velopment. This view was advanced by Fein- berg (1 990) in his proposal that low androgen levels associated with delayed puberty reduce cortical synaptic pruning, leading to disturb- ance in brain organization and, eventually, to major psychopathologies in the chromo- somally normal. While this hypothesis can- not explain the behavioural characteristics of extra X adolescent reported here (since those with least self-reported disturbance were most atypical in pubertal development), it is possible that some variation on it may ulti- mately prove to be useful.

References Bancroft, J., D. Axworthy & S. Ratcliffe (1982).

The personality and psycho-sexual develop- ment of boys with 47,XXY chromosome con- stitution. J. Child Psychol. Psychiatr. 23, I6P-180.

Bell, A. G. & P. N. Corey (1974). A sex chromatin and Y-body survey of Toronto newborns. Can. J . Genet. Cytol. 16, 239-250.

Bender, B. G. & D. B. Berch (1990). Overview: psychological phenotypes and sex chromosome abnormalities. In Sex Chromosome Abnormali- ties and Human Behavior: Psychological Stud- ies, D. B. Berch & B. G. Bender (eds.) Boulder, Col., Westview Press.

Bender, B. G., M. Linden & A. Robinson (1990). SCA: In search of developmental patterns. In Sex Chromosome Abnormalities and Human Behavior: Psychological Studies, D. B. Berch and B. G. Bender (eds.) Boulder, Col., Westview Press.

418 N E T L E Y

Berch, D. B. & E. McCauley (1990). Psychosocial functioning of individuals with sex chromo- some abnormalities. In Psychoneuroendocrinol- ogy: Brain, Behavior, and Hormonal Interac- tions. C . S . Holmes (ed.) New York, Springer, pp. 164-183.

Cattell, R. B. & M. D. L. Catell (1969). The' High School Personality Questionnaire. Illinois, NFER.

Feinberg, I. (1 990). Neurodevelopmental models of mental illness. Clin. Genet. 37, 491- 492.

Gupta, D., A. Attanasio & A. Raaf (1975). Plas- ma estrogen and androgen concentrations in children during adolescence. J. Clin. Endocrin- 01. Metab. 40, 636-643.

Millon, T., C. V. G m n & R. B. Meagher (1982). Millon Adolescent Personality Inventory. Min- neapolis, Minn., National Computer Systems.

National Computer Systems ( I 982). Interpretive Scoring Systems. Minneapolis, Minn.

Netley, C. (1988). Relationships between hemi- spheric lateralition, sex hormones, quality of parenting and adjustment in 47,XXY males prior to puberty. J. Child Psychol. Psychiatr.

Netley, C. (1986). Summary overview of behav- ioural development in individuals with neo- natally identified X and Y aneuploidy. Birth Defects: Original Article Series, 22, 3, 2 9 3 306.

Olwens, D., A. Mattsson, D. Schalling & H. Low (1980). Testosterone, aggression, physical and personality dimensions in normal adolescent males. Psychosomat. Med. 42,253-269.

Paikoff, R. L. & J. Brooks-Gunn (1990). Associ- ations between pubertal hormones and behav- ioral and affective expression. In Psychoneu- roendocrinology: Brain, Behavior and Hormonal Interactions, C. S . Holrnes (ed.), New York, Springer-Verlag. pp. 205-226.

Roy, A. (1984). Psychiatric disorders in relation to Klinefelter's syndrome. In Klinefelter 's Syn- drome H. J. Bandman & R. Briet (eds.). New York, Springer-Verlag, pp. 192-201.

29, 281-287.

Stewart, D. A., J. D. Bailey, C. T. Netley & E. Park (in press). Growth, development and be- havioural outcome from midadolescence to adulthood in subjects with chromosome aneu- ploidy. Birth Defects: Original Article Series 26, 4.

Stewart, D. A., J. D. Bailey, C. T. Netley, J. Rov- et & E. Park (1986). Growth and development from early to midadolescence of children with X and Y chromosome aneuploidy: the Toronto study. Birth Defects: Original Article Series, 22, 3, 119-182.

Stewart, D. A., J. D. Bailey, C. T. Netley, J. Rovet, E. Park, M. Cripps & J. A. Curtis (1982). Growth and development of children with X and Y chromosome aneuploidy from infancy to pubertal age: the Toronto study. Birth De- fects: Original Article Series, 18, 4, 99-154.

Tanner, J. M. (1962). Growth at Adolescence wirh a General Consideration of the Egeects of He- redity and Environmental Factors Upon Growth and Maturation from Birth to Maturity. 2nd Edit. Oxford, Blackwell Scientific Publications.

Theilgaard, A. (1984). A psychological study of the personalities of XYY and XXY men. Acta Psychiatr. Scand. Suppl. 315, 69, 1-133.

Topper, E., Z. Dickerman, R. Prager-Lewin, H. Kaufman, Z. Maimon & Z. Laron (1982). Pu- berty in 24 patients with Klinefelter Syndrome. Eur. J. Pediatr. 139, 8-12.

Walzer, S.. P. H. Wolff, D. Bowen, A. R. Silbert, A. S. Bashir, P. S. Gerald & J. B. Richmond (1978). A method of the longitudinal study of behavioral development in infants and children: the early development of XXY children. J. Child Psychol. Psychiatr. 19, 2 13-229.

Address: Dr. C. Netley University of Toronto Dept. of Behavioural Science McMurrich Building Toronto. Ontario MSS lA8 Canada