LHON in Women and Children

Does LHON Affect Women?

 

Yes.

For years doctors know very little about LHON. Medical textbooks and even many research papers described LHON as something like:

“Sudden los of central vision affecting young men”.

This made doctors ignore LHON as a cause even when women patients had the “classic” symptoms.

There are now many well documented cases of LHON in women.(23665487)(22269948)(18173420)(21734595)(8016139)(25194554)

LHON is much less common ini women, and there are about 9 men affected by LHON for every woman affected by LHON.

LHON Male-Female Ratio 9 to 1

Doctors do not yet know why mean are more likely to be affected than women.

Some researchers have found that the female hormone Estrogen seems to protect cells from the effects of LHON in laboratory tests.(20943885)

There are also cases where low Estrogen levels might have triggered LHON in a woman.(23748049)

This can’t be the whole answer, because there are many cases where a woman has developed LHON without having low Estrogen levels.

The reason could be that there is an unkown genetic factor protecting most women from LHON.  If this factor is on the X chromosome, then men are less likely to have it, as they have one X and one Y chromosome.(20300564)(1415219)

Another possiblity is that something in the metabolism of female nervce cells protects them from the programmed cell death response triggered by LHON. (22201999)

 

Does LHON Affect Children?

Yes.

Cases of LHON in children are sometimes called Early-onset cases.

LHON can affect someone at any age.(23864591)(23665487)(24398099)(19024058)(18173420)

An Italian study of 180 affected people in 56 different families found that about 1 in 9 cases were affected children.  Four out of five cases showed some recovery of vision. (17122117)

If a child develops LHON, he/she does seem to have a better chance of recovering some eyesight.

A 10 year  old boy recoverd some sight after 18 months. (22684678)

An 8 year old boy had recurrent vision loss due to LHON but by the age of 16 his vision had recovered to 8/10 in one eye and 9/10 in the other. His brother was also affected at age 12. (18563041)

A 12 year old Hispanic boy developed LHON (15884420)

 

Bibliography

Skip to toolbar