Sexological Institute, Charles University,Prague, Czechoslovakia

THE MENTAL LEVEL IN 47 CASESOF [00BB]TRUEKLINEFELTER'S SYNDROME[00AB]

By

Jan Raboch and I. \l=S^\\l=i'\pov\l=a'\Statistical analysis by MgMat. V. Mal\l=y'\

ABSTRACT

In a group of 47 cases of [00BB]trueKlinefelter's syndrome[00AB]mainly examinedfor sterility, attention has been paid to their educational standard, schoolresults, occupation, and Raven's test results of their mental level.None of the 47 subjects with female nuclear sex attained university\x=req-\standard education. The school results were poor in the case of 20 of them,and excellent only in three. Subnormal results of Raven's test (below 80)were found in 12 out of the 47 subjects examined. Only in the case of twopatients was the result of the test above average (more than 120). Theaverage value of Raven's test for the whole group was 91.9 (s = 16.4).These findings are explained by the simultaneous disorder of the sex

glands as well as of the nervous system of these subjects due to a develop-mental disturbance which has taken place before birth.

Some authors state that frequently there are mental deficiencies in men witha female sex chromatin pattern (Züblin 1953; Pasqualini et al. 1957; Ferguson-Smith 1958; Prader et al. 1958 a, b). Ferguson-Smith (1958) arrived at thisconclusion after having investigated 325 mentally defective males, in fourof whom he found female nuclear sex; three of them were feeble-minded andone an idiot. Prader et al. (1958 a, b) found eight subjects to be chromatin-positive among 336 boys of a special State school for children with mild mentaldeficiency (where the IQ generally ranged between 75 and 85). On the otherhand, not a single case of this disturbance was found in a control group of390 secondary school pupils of normal intellect.

We have ourselves examined so far 64 patients with female sex chromatin.

All of them were asked to appear in the fall of 1959 for psychological in¬vestigation, including Ravens test of mental level. 47 of the patients came

and 30 of them had been originally examined for sterility, 12 in the course ofan endocrinological investigation, and 5 had complained of functional sex

disorders.The present paper deals with the educational standard, school results,

occupation, and Raven's test results of these 47 cases of »true« Klinefelter'ssyndrome.

METHODS

The sex chromatin pattern was determined by the oral-mucosal smear technique. Theslides were evaluated according to Kosenow et al. (1957). In each case at least 200fully spread and well stained nuclei were evaluated. The finding that more than 5 °/oof cells contained sex chromatin justified the diagnosis of female nuclear sex. Thepatients were divided into the following subgroups according to the quantity ofejaculate, which was repeatedly examined.

Subgroup A-

with less than 1.5 ml of spermatic fluid which we regard as an

expression of the production of testicular androgens down to the eunuchoid level (19patients).

Subgroup -

with more than 1.5 ml of the ejaculate (28 patients).The psychological examination included a detailed subjective social history con¬

ducted by means of an interview and Raven's test of the mental level.

RESULTS

The age structure of the 47 patients with »true« Klinefelter syndrome accordingto date of birth is shown in Table 1.

Table 1.Age structure of 47 patients with female nuclear sex according to date of birth.

Date of birth No. of cases

1900-09 31910-19 31920-24 101925-29 121930-34 141935-39 31940-44 2

The results of the investigation of the patients are summed up in Table 2.

s-S» id ueuË "° -Ö

H H< -

S. s s

IO

H¿

^3i> y *-

-a o_; £

Ihcö CCÏa, -33 O

r.

a ;»Cm ^3 -2

-

—

S £3 ¿5ce <-(-l

^4J ^

^ ,2 ^¿ ri y ¡_ '(_"U < ¡

ri -û ^ ri» « ^ Seu ri^ u Í Êri r- "

oh « to

CU ^-h o ta

CJ «

Bti

Oh

_ Oo su

V

ce: ß

o _

tVO S

Il I

CM CM co "

m in * Il

* * * Il

Il I II

I I «5 I

co I I

CM r^ r~

Il I -il

oo r- uo II

-je

—

Sz

ci2-

M_ -O

cc¡ c

C eur" en

S «"^ eu

·£ "Si•1 s

PP O tu

3

c

o

The collected results in Table 2 show that none of the 47 subjects withfemale nuclear sex attained university-standard education. The largest groupcomprises 21 patients who passed a technical school or who failed to completetheir secondary education. The school results were poor in the case of 20 ofthe patients, and they were excellent in only three. Most of them were activeas skilled workers (26 cases). Subnormal results of Ravens test (below 80)were found in 12 out of 47 subjects examined. On the other hand, only in thecase of two patients was the result of the test above average (more than 120).The average value of Ravens test for the whole group was 91.9 (s = 16.4).

We also endeavoured to ascertain whether there was any correlation betweenthe Raven test results and the testicular hormonal activity of our patients. Forthis reason we compiled Table 3, in which the subjects examined are dividedinto subgroup A (with less than 1.5 ml of the ejaculate) and subgroup (withmore than 1.5 ml of spermatic fluid).

Table 3.The results of Ravens test in subgroup A (19 patients with testicular androgen of a

eunuchoid level) and subgroup (28 patients with adequate hormonal testicularactivity). The figures given in brackets indicate the frequencies expected assuming

that the values of Raven's test are the same in both subgroups.

Subgroup No. of cases

Ravens test

<80 80-100 101 4-

19 7 (4.85) (8.89) 4 (5.26)

2 S 5 (7.15) 14 (13.11) 9 (7.74)

Total 47 12 22 13

Statistical evaluation of the data appearing in Table 3 by means of the ^2-testrevealed that the difference between observed and expected frequencies is not

statistically significant ( 2 = 2.256). The difference between the average valueof Ravens test in subgroup A (M = 87, s = 17.4) and in subgroup (M = 93,s = 15.1) is also not significant. The level of testicular androgens does nottherefore seem to be of any major significance with regard to the result ofRavens test in our 47 patients with »true« Klinefelter syndrome.

DISCUSSION

We have examined a group of 47 subjects with female sex chromatin pattern,most of whom (30 cases) complained of sterility. We have found that the schoolresults of 20 of them were poor. Subnormal values of Raven s test (below 80)existed in practically one in four (12 out of 47).

These results support the view that not only the activity of sexual glands butalso the function of the nervous system, also reflected in mild mental de¬ficiencies, are often deranged by a developmental disturbance in patients witha »true« Klinefelter syndrome.

It is stated, that 50 °/o to 65 °/o of oligophrenias has its origin before birth.A relatively simple method of determining the nuclear sex has now exposed a

strictly defined group of subjects whom we may often expect to be sufferingfrom low intellectual development.

REFERENCES

Ferguson-Smith . .: Lancet 1 (1958) 928.Kosenow W'.. Glörfeld M. Se Hellmann U. E.: Klin. Wschr. 35 (1957) 826.Pasqualini R. Q., Vidal G. Se Bur G. E..- Lancet 273 (1957) 164.Prader ., Schneider J., Frances J. M. 8c Züblin W.: Lancet 1 (1958 a) 968.Prader ., Schneider J.. Züblin W., Frances J. M. Se Rüedi K.: Schweiz, med. Wschr

88 (1958 b) 917.Züblin W.: Acta endocr. (Kbh.) 14 (1953) 137.