Dystonia musculorum deformans: involuntary shaking of 4,000 per day to normal life

Written by Sandeep Nehra

In the eyes of Patrick Joret, Professor Philip Coubes is a “genius” and its operations “miracles”. Patrick Joret, and his son are suffering from dystonia musculorum deformans, but through the efforts of Professor Montpellier, they can “live a normal life.”

The president of the Silver Star, an association against dystonia, sometimes the voice drowned by the tears when he recalls his meeting with Professor Coubes and reminisces about his illness that caused him to 20 years, ” 3-4000 involuntary tremors per day.”

In the eyes of others, with these uncontrolled movements, “I passed for a gogol, he testifies. I was an abnormal, an animal circus,” says this antiquarian installed in Marmande (Lot-et-Garonne) that Today, after surgery, does not feel “any more jolts.

Dystonia musculorum deformans is a genetic disease that causes involuntary muscle contractions and abnormal movements.

This rare genetic disease that affects mostly young people, can make the child bedridden, with risk to his life.

The intervention of Professor Coubes, CHU Montpellier – alone with Paris and Grenoble to perform this operation – involves implanting electrodes in the brain fed by batteries placed under the skin of the abdomen.

These electrodes will block the electrical impulses to the source of uncontrolled disease and movement disorders, says the professor, and that it restores “normal operation”.

“I was four when I was hit by dystonia musculorum deformans” says Joret, which will be operated in 2004 at age 53.

Her illness was initially attributed to encephalitis contracted meningitis four years, “he told AFP. Dystonia is diagnosed much later, so that over time the disease has worsened. “I had sudden movements, intense,” he says. His son, Clement, has the same symptoms. “I even believe that his dystonia was more evolved than mine,” he said.

The child will be the first operated in 2001 at the age of five and a half years. Two months after the operation, “was the miracle. Clement had no sudden movements. So believe me,” said the dad, that encouraged me to have surgery. ” But the list is long and Mr. Joret will wait another 3 years.

With the operation, “we’re living a normal life,” but with the obligation to change the batteries every 4 to 9 years.

Today, Clement has 16 years, follows a normal education and is passionate about cycling, “said her father. What “pride when I go to the races on Sunday and he fights with others”!

En route to Italy, Mr. Joret will be in Montpellier this weekend at a charity led in particular by Didier Van Cauwelaert. The writer presents “The child who came from a book”, a “Romang” (novel doubled on a manga), whose rights will go to the Research Unit of Prof. Coubes on movement disorders.

Saturday, the manuscript will be auctioned, and including eight paintings of himself, who illustrated the book, and 40 plates of manga Patrick Serres.

With the money, “I’ll try to carry two patients per week,” said Professor Coubes, whilst those for only a thirty patients per year and the waiting list is long. Second objective: to continue the search by hiring an engineer.

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