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Medical Forum / General / General / April 2006

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what is or was "gargoid mongolism"?

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Jack Campin - bogus address - 17 Apr 2006 19:28 GMT
This is annoyingly inexplicable.

Back in the early 1970s I spent a summer working in a long-stay mental
hospital in New Zealand; most of the patients were never going to come
out, with dementia, very severe mental retardation or totally disabling
psychosis.  Most of them had conditions that I can easily locate in
medical books now, but there were a few odd genetic conditions I have
found nothing about.  The most haunting was a kid of about 6 with what
was described in his notes as "gargoid mongolism".  He was totally
mute, had no bladder control, and didn't respond to most people.  My
guess is that this might have been because he looked extraordinarily
weird and few people would have been able to look at him without
showing obvious signs of disgust.  I gave a him a big smile when I
first saw him; he smiled back and crawled over to me to sit on my
lap (which he peed on after a few minutes).  Apparently this hardly
ever happened.

His face reminded me more than anything of a traditional Maori
sculpture (he was white, though).  Severe hypertelorism with his
eyes slanted up about 30 degrees.  Very wide mouth.  Some sort
of congenital abdominal malformation that had required surgery
immediately after birth and left him with a very large scar.  I
don't think he could walk normally, I only remember him crawling.
He looked nothing like anyone I've seen with Down's syndrome.  I
was told "what he's got is very rare" (the only one in New Zealand?)
and the nurse who said that was not kidding - I can't find any
reference to anything like his condition anywhere.  It can't have
been Hurler's syndrome since his appearance had been the same since
birth.  Every so often I look at books about genetic disorders or
search Google on the off-chance I might spot a similar case or
matching description but have always come up with a blank.

This kind of thing, minus the tattoo spirals:

http://www.johnroyalgallery.com/cgi-bin/imageFolio.cgi?action=view&link=Contempo
rary_Art/Maori_Artists&image=koruru.JPG&img=&tt
=

What might his syndrome have been?

==============  j-c  ======  @  ======  purr . demon . co . uk  ==============
Jack Campin:  11 Third St, Newtongrange EH22 4PU, Scotland | tel 0131 660 4760
<http://www.purr.demon.co.uk/jack/>   for CD-ROMs and free | fax 0870 0554 975
stuff: Scottish music, food intolerance, & Mac logic fonts | mob 07800 739 557
Phil Anthropist - 18 Apr 2006 06:32 GMT
> This is annoyingly inexplicable.
> post snipped for brevity <

Might be worth checking out gargoyle mongolism (Hurler's syndrome).

Phil
----
Jack Campin - bogus address - 18 Apr 2006 16:16 GMT
> > This is annoyingly inexplicable.
> > post snipped for brevity <
> Might be worth checking out gargoyle mongolism (Hurler's syndrome).

I did (as I mentioned in the post).  I haven't seen many pictures
of it but I don't see how it could be the same thing - the facial
deformities were congenital, but Hurler's kids start out looking
normal, don't they?

==============  j-c  ======  @  ======  purr . demon . co . uk  ==============
Jack Campin:  11 Third St, Newtongrange EH22 4PU, Scotland | tel 0131 660 4760
<http://www.purr.demon.co.uk/jack/>   for CD-ROMs and free | fax 0870 0554 975
stuff: Scottish music, food intolerance, & Mac logic fonts | mob 07800 739 557
Phil Anthropist - 18 Apr 2006 20:38 GMT
"Jack Campin" wrote:
> I did (as I mentioned in the post).  I haven't seen many pictures
> of it but I don't see how it could be the same thing - the facial
> deformities were congenital, but Hurler's kids start out looking
> normal, don't they?

Sorry, I didn't spot your mention of Hurler's. My understanding is that they
look normal at birth. This might interest you:
http://images.google.co.uk/images?q=Hurler%27s+syndrome&hl=en&btnG=Search+Images.
Good luck with your search. You may be looking for a needle in a haystack.

Phil
----
Jack Campin - bogus address - 18 Apr 2006 22:48 GMT
>> I did (as I mentioned in the post).  I haven't seen many pictures
>> of it but I don't see how it could be the same thing - the facial
[quoted text clipped - 4 lines]
> http://images.google.co.uk/images?q=Hurler%27s+syndrome&hl=en&btnG=Search+Images.
> Good luck with your search. You may be looking for a needle in a haystack.

Thanks.  Those kids all look pretty normal compared with the one I met.

==============  j-c  ======  @  ======  purr . demon . co . uk  ==============
Jack Campin:  11 Third St, Newtongrange EH22 4PU, Scotland | tel 0131 660 4760
<http://www.purr.demon.co.uk/jack/>   for CD-ROMs and free | fax 0870 0554 975
stuff: Scottish music, food intolerance, & Mac logic fonts | mob 07800 739 557
 
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