New Options Becoming Available For Treating Tourette Syndrome by Gina Shaw Steve Blackman was 3 years old when he first started developing baffling tics and twitches, but it would be another 10 years before his condition would be correctly diagnosed: Tourette syndrome. Although the neurological disorder, which is characterized by sudden, rapid, involuntary movements or vocalizations, had been identified by French doctor Gilles de la Tourette in the late 1800s, in the 1960s, when Blackman was a child, Touretteís was still relatively unknown in the United States. But getting a proper diagnosis at last at the age of 13 didnít provide an answer as to what to do about Blackmanís ticsóthe worst of which involved sharp jerking of his head. ìI tried every treatment out there,î Blackman recalled. ìI went to the NIH [National Institutes of Health], I went to the Mayo Clinic, I went to hospitals in New York, and nothing helped.î Blackman worked for a number of years as an emergency medical technician (EMT), but about 10 years ago, the tics worsened and eventually damaged his neck so severely that he developed partial paralysis on his left side, forcing him to go on disability. Although few cases are as severe as Blackmanís, Tourette syndrome is not as uncommon as doctors once thought. According to Dr. Jonathan Mink, an associate professor of neurology and pediatrics and chief of child neurology at the University of Rochester Medical Center, Touretteís occurs in anywhere from one to 10 of every 10,000 peopleóadults and children. Its most common characteristics are uncontrolled muscle movements, such as jerking of the neck or rapid blinking of the eyes. People with Touretteís also sometimes make involuntary noises, like grunting or throat clearing. If your only picture of Touretteís comes from movies like ìDeuce Bigalow: Male Gigolo,î you may think that people with the disorder also blurt out obscenities or inappropriate phrases, but that behaviorócalled coprolaliaóis actually fairly uncommon. The major puzzle with this neurological disorder, said Dr. James Leckman, a child psychiatrist and research director at the Yale University School of Medicineís Child Study Center, lies in who develops it and why. ìMany children have transient tics or periods of tic behavior,î said Leckman, the author of ìTouretteís Syndrome ñ Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care.î Indeed, tics can occur in as many as one of every 100 children, but most of them ìgrow out of it.î Why tics develop in the first placeóand why some continue into adulthood, while others fade awayóis one of the major questions surrounding Touretteís. ìAnother major question is why some peopleís tics become more severe later in life, while others experience tics only under stress,î said Leckman, who noted that some of the worst cases of Touretteís are found in adults. Advances in brain imaging are beginning to offer some clues to these questions. Recent research presented to the Society for Neuroscience indicates that there may be brain volume differences in children who go on to develop more severe forms of Touretteís. ìThis focuses our attention on specific brain areas,î said Leckman. ìOther recent research indicates that there may be a loss of key coordinating cells in the structures of the basal ganglia of the brain, which may well hold some clues as to why people have a more severe course of illness.î These new areas of study offer the prospect of a better understanding of Touretteís origin and may guide the development of future treatments. But for the moment, thereís no ìcureî for the disorder. Medications can helpóin particular, the drug Haldol (haloperidol) has been found to dramatically reduce tics in 60 percent to 90 percent of patients, although it usually doesnít eliminate them entirely. ìAlthough theyíre not ideal, for many individuals the medications give them substantial symptomatic benefits,î said Mink of the University of Rochester Medical Center. A variety of other treatments have been tried as well. As a fact sheet from the Tourette Syndrome Association and the Pennsylvania Tourette Syndrome Association observes, ìIf you can name a treatment, some Tourette patient has probably had it prescribed as a cure for his condition.î One promising approach for many Touretteís patients is called ìhabit reversal trainingî (HRT). ìItís a behavioral intervention, substituting another motor act or vocal act that will take the place of the tic,î said Leckman. Therapists work with patients to identify situations in which they particularly experience tics, help them become aware of the ìtic urge,î and head it off with another behavior that makes it difficult or impossible to perform the tic. For example, instead of rapid eye blinking, a patient might gently close his eyes and keep them closed for 10 seconds. If the tic is a vocalization (these arenít as common as motor tics), the patient might substitute controlled, rhythmic breathing. Studies have found that HRT can reduce tic severity by about 35 percent and decrease the ticsí interference with a personís daily life by about 55 percent. Mink noted that these studies have been relatively small so far, but called them ìvery promising.î But for a patient such as Steve Blackman, behavioral interventions werenít the answer. So in the spring of 2004, he was excited to learn that a new surgical approach, deep brain stimulation (DBS), which has shown promise in treating Parkinsonís disease and tremor, had been used for the first time in a person with Tourette syndrome. Thirty-one-year-old Jeff Matovic had lost jobs and relationships because of the severity of his muscle spasms, which could shatter drinking glasses as he held them. Guided by magnetic resonance imaging (MRI) and 3-D computer imaging, a neurosurgical team at the University Hospitals of Cleveland led by Dr. Robert J. Maciunas implanted two electrodes around key cells in the thalamus portion of Matovicís brain. Those electrodes, connected to a battery implanted beneath the collarbone, deliver continuous high-frequency electrical stimulation that rebalances the control messages running through the brainís movement centers. As soon as he heard about Matovicís surgery, and the impressive resultsónearly complete resolution of his symptomsóBlackman called Leckman. ìI asked him, ëDo you think Iíd be a candidate for this?í He told me, ëSteve, if youíre not, then nobody is,íî Blackman recalled. It was July 2004 and Blackmanís tics had done so much damage over the years that he often found it necessary to use a scooter to get around. ìHeíd already had dramatic loss of function, and it was just a matter of time before he became permanently paralyzed on his left side,î said Leckman. Blackmanís DBS surgery was performed on July 24. ìImmediately after the surgery, my tics stopped completely,î he said. In the intervening time, theyíve returned somewhat, as Blackman and his doctors work to fine tune the stimulatorís programming. ìRight now, my tics have been reduced by about 75 percent, which is great,î he said. ìI only went in expecting a 5 [percent] to 10 percent reduction.î The existing neurological damage, unfortunately, is irreversible, but now Blackman has hope that it will not get any worse. ìObviously, itís not right for everyone, but there will be some people that it will help.î There are some risks involved: DBS is, after all, brain surgery. ìItís also very expensive, and itís still in an experimental phase,î cautioned Mink, noting that much more is known about DBS for Parkinsonís disease and tremor because more is known about precisely where those disorders affect the brain and how. ìA lot of things make Tourette different, and that makes us more cautious about recommending DBS without more substantial study, which is beginning now. Thereís still a lot we donít know, such as precisely the best part of the brain to target, and which patients are most appropriate to offer this to. But there may be real promise here.î In light of advances such as HRT and DBS, Leckman said heís ìmore optimistic than I have been for a decadeî about the prospect of relief for people with Tourette syndrome. ìOn one side of the spectrum, we have behavioral therapy that uses willpower and determination to change things, and on the other end, if nothing else is working and things are really severe, we have a surgical way of intervening with DBS,î he said. Thereís no ìone sizes fits allî solution for Touretteís, said Mink. ìIn order to choose the right treatment approach for a particular patient, you need to sort out all the components of the disorder and its associated symptoms,î he said. ìTherapy really has to be individualized.î For more information on Steve Blackmanís experience with Tourette Syndrome, you can e-mail him at sblk@localnet.com. Gina Shaw is the medical w riter for The Washington Diplomat.