Tourette’s Syndrome – Deep Brain Stimulation

One in 2,000 of us, suffer from Tourette’s Syndrome, to some degree. A controversial new surgery promises hope to those with this debilitating disorder.

Keith Cofer has Tourette’s Syndrome. Like many sufferers he is plagued by uncontrollable movements and vocal tics. There is no cure or specific drug treatment for Tourette’s Syndrome, but a radical form of brain surgery called deep brain stimulation, DBS, may now offer hope to people like Keith.

Electrodes are implanted into a patient’s brain during surgery, while they remain fully conscious. Wires carry an electric pulse to the deepest parts of the brain, essentially rewiring tourette’s.

75 year old Dr Donald Richardson, Professor of Neurosurgery at Tulane Hospital, New Orleans, is currently one of only a handful of pioneering neurosurgeons prepared to carry out this radical procedure.

Professor of Neurosurgery
Dr Donald Richardson

He defends his actions “The best thing we can offer someone with Tourette’s Syndrome is deep brain stimulation. It works better than anything else we’ve tried and it seems to be relatively specific, and it’s safe.”

This operation has not yet been approved by the medical establishment. Controversy surrounds what many regard as Richardson’s experiment on tourette’s sufferers. They claim that without official guidelines the life-threatening dangers associated with the procedure cannot be correctly evaluated.

The argument against Deep Brain Stimulation

Dr Neal Swerdlow puts the argument against “This can’t be done with lone cowboys out on the range. It has to be done by bringing individuals together, to help them collect the information, and then they, as a group, can assess it objectively in a manner that isn’t biased by their own wish for the procedure to work”.

Dr Richardson counters “This operation has been done thousands of times. This is exactly the same procedure we use for Parkinson’s Disease and the risk is very low. The only difference for tourette’s is we locate the electrodes in a different part of the brain”.

Tourette's Syndrome
Sean Hulme

Dr Richardson’s first patient was 32 year old Sean Hulme. He is one of a tiny minority of tourette’s sufferers to undergo this revolutionary surgery. Sean lives in rural Alabama and is a keen hunter. Prior to the surgery Sean’s muscle tics caused him to punch himself in the head and genitalia. Sometimes he would punch his genitalia so badly that he could barely walk. Sean’s life was completely transformed by his DBS surgery.

News of the Deep Brain Stimulation procedure and the miraculous transformation of Sean Hulme has travelled like wildfire through the tourette’s community. Desperate for a solution to a lifetime of torment, Keith Cofer made a home video hoping he could persuade Dr Richardson to repeat his experimental surgery.

Keith was told by doctors that, as he got older, his tourette’s would burn itself out. That was supposed to happen in puberty, but it didn’t. It got worse.

However, there is one place in Keith’s brain where tourette’s does not rule. His wife, Deborah, explains “The only time, maybe, that Keith does not have tourette’s would be, possibly, during sex. During sex there is no tourette’s, there are no tics or vocalisations”.

Keith is in crisis. His vocal tics are now threatening his career. He’s worked as a paediatric nurse for 12 years, but his increasing use of the word ‘nigger’ has pushed some of his black co-workers to make official complaints about his apparent racism.

Keith managed to get an appointment with Dr Richardson at Tulane Hospital, New Orleans and he offers Keith the chance to be his third patient to undergo the radical brain surgery.

Dr Donald Richardson is seen as a maverick and has always been at the forefront of experimental brain surgery. In the 50s he championed the lobotomy, a controversial procedure involving the destruction of parts of the brain as a cure for a variety of psychiatric disorders, including tourette’s.

Operation day is the biggest day of his life and Keith knows he might not survive it. He has written his will and said his goodbyes in preparation.

Keith must remain awake during surgery, The only painkiller he is given is a local anaesthetic to numb his skull. There’s no way to prevent Keith ticking during the operation so Dr Richardson screws a metal frame to his twitching head. This device locks his skull onto the operating table preventing any movement.

Cranial Scaffold
Head Frame

Keith’s conscious reactions, as the electrodes are inserted, are crucial, as they will allow Richardson to know when he has hit his mark. The electrodes are inserted into the thalamus, the small section of brain responsible for tics, but it also controls instinctive emotions like fear and happiness.

This is the most dangerous part of the procedure. As the metal tip pushes through grey matter, it could rupture a blood vessel. Keith risks major brain damage or even death.

Deep Brain Stimulation
Skull Hole

With the electrodes inserted to within a few millimetres of the calculated position, Dr Richardson turns the electrodes on. The effect is instantaneous, within a few seconds Keith stops ticking. The electrodes are moved to the final, calculated position but Keith reports that his earlier elation has changed to sadness. The electrodes are too deep. Dr Richardson pulls them back explaining that finding the exact location for a given patient is often a matter of trial and error which is why the patient must remain conscious to provide the feedback.

After six hours of surgery, Dr Richardson is confident that he has Keith’s tourette’s under control.

Deep Brain Stimulation
Keith Cofer

However, the operation is not yet over. Keith must be given a general anaesthetic so that the battery-powered brain pacemakers may be inserted into his chest. These will provide the electric pulse to the electrodes in his brain.

Just a week after the surgery, Keith feels well enough to join Deborah on a camping vacation. Surgery has not damaged his brain, but it has caused it to swell. Keith must wait for a month for this to subside before the brain pacemakers can be activated.

Dr Richardson seems to be offering genuine hope for tourette’s sufferers, but controversy still surrounds him. Within weeks of Keith’s surgery, the 75 year old shocked the medical establishment and tourette’s experts once more when he decided to carry out DBS on the youngest patient ever – 13 year old Brett from California.

Keith is now home in Memphis. Before surgery, Keith’s racist remarks were destroying his life. “I’m still having some tendency towards vocalisation, but I’m able to restrain it better” he remarks. This has given Keith a tremendous psychological boost and his improved control has allowed him to get his job back.

Additional Reading

[amazon template=template&title=-&artist=-&asin=019852398X] Tourette Syndrome: The Facts – Mary M. Robertson, Simon Baron-Cohen

[amazon template=template&title=-&artist=-&asin=1853466565] Tourette Syndrome: A Practical Guide – Amber Carroll, Mary M. Robertson

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