About Benjamin's Syndrome
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Causes Of Benjamin's Syndrome
Overview
The real cause or causes of Benjamin's Synrome are not known for certain. Some might say people with Benjamin's Syndrome are born with souls of the opposite gender to their bodies, which may be true but isn't verifiable. Others consider it to be simply an issue of upbringing, which seems unlikely given that it has occured throughout history in all societies and social classes. But the most plausible explanation is that people with Benjamin's Syndrome have brain structures that do not match the sex of the rest of their bodies, due to some genetic or hormonal irregularity.
A Basic Guide To Human Sexual Differentiation
When a human egg is fertalised by a sperm, a new life begins from a single cell containing a mixture of DNA from both parents. This DNA (usually) contains either the chromosome pair XX or the pair XY. The cell replicates itself, and after a while the cells seperate into three distinct groups: one that will later form the skin, nervous tissue, and brain; one that will form bone, blood, and muscle; and one that will form most internal organs. A placenta forms to connect the embryo to its mother. This transfers substances such as nutrients from the mother's blood stream to the embryo, and also produces hormones to regulate the embryo's development.
The embryo begins developing gonads which will later differentiate into either testes or ovaries by means which are not well understood, but involve hormones and the XX or XY chromosomes. The embryo also develops the beginnings of both male and female internal reproductive structures, one of which is absorbed back into the body depending on certain hormones. The presence of testosterone causes the male structures to develop further, and the presence of AMH causes the female structures to be reabsorbed. Both are produced by the testes. In the absence of both hormones, the male structures will be reabsorbed and the female structures will develop. The external genitalia is initially female, but the presence of testosterone causes further development into a male configuration. The seam where the proto-labia fused together can sometimes be seen on the scrotum and the underside of the penis of adult males.
The sexual differentiation of the brain is not as clear cut, but is similarly affected by hormones. The brain develops quite independently from the reproductive system, with the cells that will form the two systems differentiating very early in the embryo's development. Sexual differentiation for the two occurs at different times, may involve different hormones and different genes, and involves tissues that may respond differently to the same hormones. The brain is also exceptionally complex, and could be affected by much smaller hormonal variations than other parts of the body.
What Can Go Wrong
The embryo's DNA is essentially the blueprint from which it knows how to develop. This blueprint contains much more information than the embryo will actually use. Genetic abnormalities can cause errors in the parts of the blueprint that the embryo is using, or tell it to use the wrong parts of the blueprint. So some parts of the body might not know they're supposed to make certain changes when they're exposed to testosterone, for example. Also, the presence of various hormones at various stages during the embryo's development helps regulate which parts of the blueprint are followed. If the wrong hormones are introduced at critical times (eg by the mother taking pills), or something prevents the right hormones from being present, the embryo will not develop as expected.
Abnormalities in sexual differentiation as a group are known as Intersexuality. Androgen Insensitivity Syndrome (AIS) is a good example. Embryos with Total AIS do not recognise testosterone, so their external genitalia is female. However, they are affected normally by AMH, so if they have XY chromosomes they will not develop female internal organs (eg the uterus).
Benjamin's Syndrome is a form of intersexuality in which the brain of a person with XY chromosomes fails to masculinise for some reason, or the brain of a person with XX chromosomes does masculinise (there may be many ways in which this can happen). It is often (but by no means always) accompanied by other signs of mild atypical sexual differentiation, eg a particularly large clitoris or small penis, unusually pronounced proto-labial seam, late or minimal puberty, etc. It can also coexist with other types of intersexuality, though if another kind of intersexuality is present then an additional diagnosis of Benjamin's Syndrome would generally be considered superfluous.
Technically speaking, this makes the brain structure/gender identity of a person with Benjamin's Syndrome the thing which is "wrong" (unless their brain matches their chromosomes and everything else is wrong, but that isn't common). However, the brain is by far the most important part of the body; the rest is just a shell to keep the brain alive. While the gender identity of a person with Benjamin's Syndrome may not be what was intended by their DNA, it is a perfectly normal, healthy state, and for all practical purposes they are the same as if their brain was as intended and their body the "mistake". The XX/XY chromosomes do affect the sexual differentiation of the embryo, but are by no means the only factor, and are not a useful means of determining a person's actual sex. In some species, chromosomes are completely irrelevant, and sex is determined by, for example, the temperature at which the eggs are incubated. Members of other species naturally change sex during their lifetimes.
Women with Benjamin's Syndrome are more common than men (there is no consensus on the exact ratio), which is a natural consequence of human development being female by default (it's generally easier for something to fail to happen when it should than for something to happen when it shouldn't).
Artificially Induced Benjamin's Syndrome
One situation in which the cause of Benjamin's Syndrome is quite clear is the sexual mutilation of children or infants. This most often occurs with physically intersexed infants, where the individual is operated on to make them conform more closely to a sex chosen by a doctor with no possibility of consent or regard for their brain structure/gender indentity. It can also occur if a male infants's penis is acidentally damaged or removed, and it is easier to surgically transform him into a female and raise him as such than to reconstruct the penis. It is common for people in these situations to be very unhappy with what was done to them, and revert to living according to their actual gender later in life.
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