New Treatment Holds Promise For Patients With Tourette Syndrome

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Tourette Syndrome

Research out of the Neurological Institute at University Hospitals Case Medical Center finds that Deep Brain Stimulation (DBS) helps patients who suffer from Tourette Syndrome (TS). This first-of-its-kind study of five adults with TS determined that DBS can reduce tic frequency and severity in some people who have exhausted other medical treatments.

Tourette syndrome is a neurobehavioral disorder characterized by sudden, repetitive muscle movements (motor tics) and vocalizations (vocal tics). It often begins in childhood. By young adulthood the tics have usually diminished in frequency and severity. However, in some adults, like those that participated in this clinical trial, the tics become more disabling even with best medical therapy.

The UHCMC team's prospective double-blind study followed these five adults with TS over the course of 12 months. The 3-month data are published in the November issue of the Journal of Neurosurgery. The team concluded that the majority of patients experienced a significant reduction in symptoms and an improved quality of life. The study followed their landmark case in 2004 when the UHCMC neurological team used DBS to treat a 31-year-old man with Tourette Syndrome. Once his deep brain stimulators were turned on he had an immediate and nearly complete resolution of his symptoms.

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"This is this first and only careful scientific surgical trial for Tourette Syndrome, and provides the highest level of data showing that this surgery is proven to be significantly beneficial for medically refractory Tourette," says Robert Maciunas, MD, Vice Chairman and Professor of Neurological Surgery at UHCMC and lead author of the paper. "This study promises to change the standard of care for patients who fail to respond to traditional medications. It promises to usher in a new era of medical research and treatment for Tourette Syndrome with DBS," he says.

DBS is approved by the Food and Drug Administration for treatment of Parkinson's disease, essential tremor and dystonia. DBS involves the surgical implantation of electrodes in the brain and pulse generators in the upper chest just beneath the collarbones. The implanted pulse generator (IPG) is connected to the electrode in the brain by a thin cable that is placed under the skin. The IPG is then programmed to deliver a high-frequency electrical stimulation to the targeted area of the brain.

In this double-blind study the electrodes were placed in the thalamus, a portion of the brain that is involved in controlling movement. Prior to surgery the neurologic team at UHCMC mapped out regions of the patients' brains using MRI (magnetic resonance imaging) scans and 3-D computer images. Their goal was to locate the safest and most direct route to reach the thalamus. Following surgery the researchers conducted double-blind video-based rating assessments of tic frequency and severity. They found that three of five patients had a marked reduction in their motor and vocal tics and all but one patient had a significant improvement in quality of life.

"Deep Brain Stimulation has been used to successfully treat certain movement disorders for years," says David Riley, MD, Director of the UH Movement Disorders Center and Professor of Neurology at Case Western Reserve University. "We carefully studied its use in these five patients suffering from refractory Tourette Syndrome and found that it significantly reduced the tics in most of the patients and improved their quality of life."

"The use of DBS for Tourette syndrome is still an emerging area of study but in this first of its kind clinical trial, our team found that DBS has good potential to be a viable treatment for people with this potentially debilitating syndrome," says Christina Whitney, DNSc, RN, clinical nurse specialist in the movement disorders center. "The treatment was found to be safe and we plan to conduct a larger clinical trial to further determine its efficacy in helping patients who have failed traditional medical therapy for Tourette syndrome".

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An Unpleasant Decade: Tourette Syndrome Tourette Syndrome (TS) is a mysterious syndrome with no known etiology, although causes are suspected with this disorder. One suspected cause of TS is believed to be a childhood onset hereditary familiar neurological disorder possibly due to a genetic mutation. This results in a neuropsychiatric brain developmental disorder as well as unregulated neurotransmitters in the brain of a TS patient- dopamine in particular, which is elevated beyond normal measures. The syndrome has been identified since the early 1800s by a French doctor. Boys are three to four times likely to have TS than girls. Secondary causes for TS exist, and these causes may be physiological events, such as a head trauma, encephalitis, or a stroke. Or there may be psychogenic causes of TS that may include autism, schizophrenia, and retardation. Also, excessive and prolonged drug use from cocaine has been known to cause tics resembling TS as well. The identifying traits in suspected TS patient are what are known as tics. Tics are a form of what is known as dyskinesia- which is the inability for one to control their movements. Such movements are involuntary and habitual muscular movements and contractions combined with vocal sounds made suddenly, rapidly, and frequently. For TS to be diagnosed, both vocal and motor displays of dyskinesia must exist consistently for over a year before the age of eighteen. Yet these displays of tics by TS patients are not consistent in about 20 percent of patients. In addition, the diagnosis is made by observations of the patient’s health care provider, as there is no diagnostic criteria available. TS is unfortunately often misdiagnosed. Recently, a book was created that is called, “Against Medial Advice”, which was written by James Patterson and Hal Friedman, who is the father of Cory, the boy with TS that is discussed in the book. Cory’s father vividly describes the hell he experienced for quite some time trying to get the appropriate diagnosis and treatment for his son. There is so little known about TS by health care providers, along with standard treatment options that do not exist. Both of these factors make things difficult for both the patient and their family often. An ever changing cocktail of powerful drugs make tics worse for the TS patients frequently, as no pharmacological treatment regimen works the same for every TS patient. With diagnosing the TS patient, If only motor or vocal exists separately in a chronic manner, this is known simply as a tic disorder, and not TS. In either one of the disorders, the symptoms with the tics that are expressed by such patients range from simple to complex. If the TS patient tries to suppress their involuntary tics, they will express themselves later in the day at a greater magnitude. Onset of TS is typically at the age of six or seven, with the symptoms of the tics peaking around ten years of age. There are approximately 200,000 people in the United States with TS presently. Simple tics expressed with TS patients may include rapid eye blinking, shoulder jerking, sniffing, grunting, and barking. In more severe cases, TS patients may experience face grimacing, arms flapping, and excessive words that take three forms. The first form, which many associate with TS, is the cursing words, known as coprolalia. The cursing is experienced in only less than 20 percent of TS patients. Overall, the tics expressed by the TS patient become worse if the person is also experiencing anxiety, stress, or fatigue The other forms of the use of excessive words manifest into either palilia, which is repetitive repeating of the same word, or echolalia, which is the repeating of the words of others. Overall, the most common tics expressed by those with TS are throat-clearing and eye-squinting. Such involuntary actions by the TS patient are socially inappropriate, and the TS patient will often inhibit their interaction with others as a result and often develop a school phobia as well. Other medical conditions often exist with the TS patient as well. While most TS patients have behavioral problems, the most common medical conditions with the TS patient are ADHD, learning disabilities, and OCD. Usually, only one of these medical conditions is experienced by the TS patient. About one third of TS patients experience each one of these medical conditions. Fortunately, most TS patients are mild with their symptoms, and not severe. Pharmacological treatment usually is needed for only a year or two when the tic symptoms peak around the age of ten. Those TS patients who also have ADHD have taken stimulants to treat this medical condition, and stimulants may make the tics worse for them. An anti-hypertensive drug called Clonidine is standard drug treatment for the TS patient. Other therapies that have helped TS patients have been botox injections at various locations on their head and neck. Also, nicotine patches have proven to be beneficial as well for unknown reasons. A procedure known as deep brain stimulation has proven to be beneficial for the TS patient. In this procedure, small electrodes are placed on the thalamus inside the brain, and electrical stimulation is given to this area of the brain for long periods of time and is consistent. A battery is placed underneath the skin on the chest of the TS patient. This treatment has proven to be beneficial for Parkinson’s patients as well. In addition to these treatment methods, TS patients learn through others on how to manage their disruptive behavior. This may include cognitive behavioral therapy and relaxation techniques. Fortunately, the entire lifespan of this annoying syndrome lasts about ten years, and the tics fade as the TS patient experiences their teenage years. Remission is common after these years. Researchers continue to identify specifically a cause for the syndrome, and the most effective treatments for TS patients.