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Gilles de la Tourette


 

Gilles de la Tourette

by Kenneth Lyen

Today I was introduced to a family whose 10-year-old son has little jerky movements consisting of sudden and involuntary shrugging of the shoulders, splaying of the arms, twiching of the face, fluttering of the eyelids, and if seated, kicking of his legs. On top of that, perhaps even more startling, is that he can unexpectedly blurt out an obscenity. For a well-educated genteel upper middle class family who would never use four-letter swear words even under extreme duress, this tendency to vulgarities is most bizarre and bewildering to them.

What the little boy has is a condition called the Tourette Syndrome. It was first described in 1884 by a French physician, Gilles de la Tourette (1857-1904). He wrote about nine patients who were affected with the symptoms that were later to bear his name. One of these was the Marquise de Dampierre, an aristocratic lady who developed compulsive tics from the age of seven years, and they persisted until her death at the age of 80 years. She became a recluse and according to another doctor, she "ticked and blasphemed" all her life.

This condition affects 2% of the population, and among famous patients afflicted with this disease was Amadeus Wolfgang Mozart. Perhaps Mozart had outbursts of profanities, or coprolalia, which may have inadvertently upset poor Salieri, who is alleged to have murdered Mozart.

By the way, "coprolalia" is derived from the Greek words "kopros" (dung) and "lalein" (to babble).

The condition is inherited as an autosomal dominant, which means that it can be passed on by either parent. But to date, the gene has not been identified, nor the biochemical aberration which gives rise to the condition. Medical treatment is available but drugs only suppress the symptoms, and when the medication is stopped, the manifestations may recur.

When I asked the parents where the boy may have picked up his scatology, they immediately blamed the school. Apparently it is the norm for schoolboys to use four-letter swear words that would shock any grandmother. Definitely they would not have picked it up from Singapore television which is squeaky clean. How then, I asked, is it possible to make a diagnosis of Tourette Syndrome, if the only manifestation was the coprolalia?

The disease remains as baffling and mystifying as it did 120 years ago when Dr Tourette published his findings.