There are more mentally deficient men than women
The distributions of
general cognitive abilities, as indexed,
say, by IQ, are significantly different in men and
women. Though the average intellectual
abilities are expectedly similar (recall that IQ tests are designed to equalize
the two sexes), there are more geniuses
and cankers who are male. In other
words, the male distribution is somewhat
more platykurtic (flat), even though in
both sexes, the expected bell-shaped curb is obtained. Though it is rarely mentioned in the
voluminous literature on cognitive sex differences, there is a very obvious biological
explanation for one aspect of this sex difference. Indeed, a greater incidence of male mental
deficiency is wholly to be expected given the way the sex chromosomes interact
with each other. The female of the
human species, as of the other mammalian species is blessed by a genetic
protective device called "lyonization" after Mary Lyon, a British
geneticist who first recognized the phenomenon. Recall that women are born from the
combination of two X chromosomes, one
contributed by the father, and the other
by the mother. In female mammals, one of the two X chromosomes is inhibited
(inactivated) by the other in anywhere between 15 to 65% of the body’s cells,
in a somewhat random manner. When one of
these two X chromosomes contains a disease-determining gene, enough cells contain an inactivation of the
mutant gene to protect the female from the disease. This inhibition is not complete, but is nevertheless quite effective. Recall
that men are born from the combination
of a paternal Y chromosome and a maternal X chromosome. The tiny Y chromosome, being so small, and also very different from the X chromosome,
is unable to protect the male from bad genes located on the large X
chromosome. As a result, all recessive X-linked genetic disorders
affect males more often and more severely than they do females. Most X-linked
diseases are recessive. In the few cases
whre the X-linked disease is determined by a dominant gene, it is females who express the disease more
severely. One example of such a disorder
is a special form of rickets, which
affects women twice as often as men.
However, though men are less frequently affected, those who suffer it have a more severe form
of the disease. Even though some
X-linked diseases are manifest only in girls (such as Rett’s syndrome), they can be of the recessive type. In those
cases, the disease is lethal for the male fetus. Are X-related "bad" genes frequent
enough to explain the extent of the known sex difference in prevalence of
mental deficiency ? Easily ! There are dozens of X-linked hereditary
diseases leading to mental deficiency in boys and men only. In fact,
as other authors have pointed out,
female mammals are «genetic mosaics»,
a phenomenon reflected very concretely in the calico fur of female cats.
Table 3.
Mental deficiency syndromes due to genes located on the X chromosome
Syndrome
|
Description
|
Martin-Bell syndrome
|
Fragile X syndrome, may present as autism, macro-orchidy, large
ears
|
Golabi syndrome
|
Microcephaly, excessive
growth, eye, heart and gastrointestinal deformities, diaphragmatic hernia,
brain tumors
|
Menkes syndrome
|
Copper deficiency,
anemia, kinky hair, progressive
cerebral atrophy, reduced life expectancy
|
Juberg syndrome
|
Retarded growth,
deafness, microgenitalism, ocular deformities, flat nasal bridge
|
Rénier syndrome
|
Epilepsy, spasticity et
deafness
|
Lujan
syndrome
|
Marfanoid habitus,
psychosis, cardiopathy
|
Renpenning syndrome
|
Microcéphaly and retarded
growth, up-slanting palpebral fissures and
small testes
|
Coffin-Lowry syndrome
|
Severe psychomotor
retardation, facial and digital dysmorphisms, and progressive skeletal
deformations
|
Lowe’s syndrome
|
Major abnormalities of
eyes, nervous system, and kidneys. Clinical manifestations include congenital
cataract and renal tubular dysfunction
|
Schimke syndrome
|
Progressive atrophy of
the basal ganglia, growth retardation, external ophthalmoplegia, postnatal
microcephaly, deafness,
spondyloepiphyseal dysplasia, nephrotic syndrome and defective cellular
immunity
|
X linked hydrocephalus
|
Spastic paraplegia,
adducted thumbs, and agenesis of the corpus callosum, hypoplasia of the
pyramidal tract and septum pellucidum, and a thin cerebral mantle with
hypoplastic white matter
|
Norrie disease
|
Ocular atrophy, deafness, dysmorphic features, cataplexy,
abnormal REM sleep, absent platelet MAO-B activity, increased serum serotonin
|
Seemanova syndrome
|
Microcephaly with
epilepsy, spastic tétraplegia, absence of abdominal reflex, lymphoreticular
malignancies
|
Lesch-Nyhan syndrome
|
Over-production of uric
acid, spasticity, choreoathetosis and a compulsive form of self-mutilation
|
Opitz FG syndrome
|
Macrocephaly, imperforate
anus, hypotonia, partial agenesis of the corpus callosum, joint hyperlaxity,
subtle facial abnormalities, sagittal craniosynostosis and split hand
malformation
|
Other x-linked mental deficiency
syndromes include the Davis, Atkin, Garéis, Vasquez, ATR-X, MASA and SPG1
syndromes.
As we shall see in several of the next chapters
however, there is also a predominance of
mental deficiency in the male sex associated with several causes which have
nothing to do with X-linked genetic mutations.
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