Wednesday, November 28, 2007

The Imp of the Perverse: Self-Mutilation and the Lesch Nyhan Syndrome

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When I was a medical student and working at the local pediatric hospital i met a young boy with a terrible condition. I was hovering at the back of the large team on the morning ward round- and wasn't paying much attention until one of my colleagues whispered to me conspiratorially : "Last week he chewed a bit of his tongue off and spat it at the nurse.".

Needless to say this comment suddenly piqued my interest in the "case". It turned out that the child had a condition known as Lesch Nyhan syndrome. This is known as an 'inborn error of metabolism' - a term that does no justice to the horror of being afflicted.

I had always thought that the self mutilation was due to a combination of sensory deficit and mental retardation- but it is much more horrible even than that. There is a great article from earlier this year from the New Yorker about the condition- and it turns out that some sufferers can have normal intelligence and can describe the horrible compulsion they have to gnaw their own body parts- they can completely chew off their lips, tongue, fingers. They have been known to enucleate their own eyes.

These seem to be true 'compulsions'- ego-dystonic, irresistible mutilatory urges. Who could think of a more horrible state?- like living in a nightmare or horror film.

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