Winter 2007 Newsletter

by Joohi Jiminez-Shahed, M.D.

Tourette syndrome (TS) is defined by the presence of multiple motor and vocal tics with onset prior to age 18 and with no other medical explanation for the tics. Most of the time, there are other features that co-exist with the tics, such as attention deficit disorder with or without hyperactivity (AD/HD), obsessive-compulsive behaviors (OCB, not always full-blown obsessive-compulsive disorder), mood disturbances such as depression, anxiety, and phobias, and other behavioral problems. The focus of this article will be to discuss factors that may influence whether symptoms of TS will remit or persist into adulthood.

TS is definitely a syndrome, with a spectrum of expression that can include any combination of the major features. For example, some may have tics with AD/HD, tics with OCB, or tics with everything. As a consequence, TS affects people in different ways. For some people, tics may be the major source of disability, while for others, it may be the AD/HD, or the OCB, or again, all of the above. It is quite common for AD/HD to become apparent by age 4, and OCB by age 7, and tics somewhere in between, usually motor before verbal. The good news for most people is that most TS symptoms resolve or significantly improve by late adolescence. Patients, parents, and doctors need to think carefully about what problems are occurring and whether or not they warrant treatment.

The worst-ever period of tics often occurs between the ages of 10 and 12, followed later by a significant reduction by the time they reach adulthood. Even though up to 20% of children may experience such severe tics that school functioning is impossible or in severe jeopardy, estimates of tic remission range from 26-90%. Other studies have shown that 20-90% of children with TS will still have tics in adulthood, but it is not entirely clear why some do and some don't. Some research has suggested that measures of hand dexterity, the volume of a brain area called the caudate, or the severity of childhood tics may predict persistence into adulthood.

The good news is that it is very rare to see an adult with tics that are as intense as when they were growing up. More often than not, adults will have milder or less intense versions of their old tics. In some rare cases, we see that tics come back after having disappeared, perhaps when people reach their 30s or 40s. We don't have good explanations for this either, but fortunately, these individuals can be treated with the same medications that are typically used to treat tics in childhood. When adults with debilitating tics are not treated successfully with medications, they may be considered as candidates for deep brain stimulation surgery, though this is best undertaken at specialized centers such as ours. To date, we have treated 2 adolescents and one adult with measurable improvements following surgery.

Some research shows that children with TS are likely to have more symptoms of OCD into adulthood even after the tics have improved. In one study, this correlated with the presence of a higher IQ. AD/HD may or may not improve. Some investigators have found that children with TS are prone to develop lifelong psychiatric problems such as depression, anxiety, and bipolar disorder. These may become more apparent at the transition through adolescence or even later. A few studies have looked at overall functioning in adults with childhood-onset TS. Approximately 1/3 of adults with childhood-onset TS will continue to experience occupational, social, or educational dysfunction, according to one study.

Because of their TS, children may have trouble with socialization, schoolwork, and self-esteem. The more co-morbidities there are, the more problems a child may have, and these difficulties may permeate into adulthood. However, the adult with TS may have new challenges, such as getting into and completing college, getting a job and/or staying employed, and developing meaningful relationships. Having said that, most are quite successful at doing so and live fulfilling and productive lives.

There are a certain number of people who will come to the doctor's office for the first time as adults for evaluation of tics. TS is usually diagnosed in childhood, unless symptoms are mild and it goes unrecognized. Therefore, on careful questioning, we often do discover that patients presenting for the first time with tics as an adult may have had milder, non-bothersome tics when they were growing up that have now resurfaced in a more noticeable manner. Such patients can be diagnosed with TS. Otherwise, there are a number of other neurologic conditions that can cause tics that should be considered. Many of these conditions have features that are highly unusual for TS, and therefore are readily recognized.

Adults with TS who still have problems with OCD and/or AD/HD are treated with the same types of medications as children with TS, with little long-term risk. Tics are treated essentially the same way as they are in children as well, but with a few exceptions. Depending on the severity of tics, different medications can be used. Botulinum toxin offers relatively little risk, because it is injected directly into muscles that are producing the abnormal movements. This works best for a facial (e.g., eye blinking or rolling) and neck tics (e.g., "whiplash" tics"), or other single, relatively simple tics involving one body part (e.g., in the vocal cords, hand or shoulder). Topiramate, a seizure medication, carries no risk of tardive dyskinesia (TD), a condition mentioned by Dr. Jankovic in the last newsletter. It also has a different side effect profile than other agents, and may not be as effective when there are multiple, complex, or severe tics.

However, when dopamine blocking medications are needed, they can and should be used, following a careful discussion of the risks and benefits of such treatments. Even though these medications are highly effective, they can be associated with TD, and this risk is higher in adults than in children. Because of this, I try to use topiramate first, both in children and adults with tics. Using topiramate first may also reduce the need for or the dose required of stronger medications. Fortunately, tetrabenazine (a different type of dopamine blocking agent) does not cause TD, and is now on its way to being available for prescription in the United States. Until then, it continues to be available through our clinic.

Symptoms of TS can persist into adulthood, though they are generally not as distressing as they were in childhood. Many factors that are not well understood may determine this. Despite this, it is important to remember that most children with TS will still live normal lives as adults, and should be encouraged to pursue their hopes and dreams. Treatment options for adults with TS are quite similar to those for children, but specific thought should be given to the potential side effects before starting treatment.

Joohi Jiminez-Shahed, M.D. is an Assistant Professor of Neurology at the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine in Houston. Click here for further information.

by Emily Stark, North Texas Support Group Leader

PART ONE of TWO

Since my son was diagnosed with TS in 2003, we have worked hard to get a support group going in the North Texas area and keep it going. In doing so, I have had the opportunity to meet many people within Texas Education Agency (TEA), more specifically within Region 10, which covers Dallas County and six surrounding counties. In 2004, working with Margie Gunther, the Special Education Director for the Carrollton-Farmers Branch ISD, I was introduced to Ivan Vance, Senior Consultant, Parent Involvement and Mental Health, Special Populations, Division of Instruction, Region 10, Texas Education Agency.

Through Ivan and Region 10, we have been able to bring speakers to our area to help educate our parents, nurses, teachers, counselors and administrators about TS and all of its co-morbid. Ivan has brought in Sheryl Kadmon, the Executive Director for the Tourette Syndrome Association of Texas, whom we all know and love; Brad Cohen, school teacher in Atlanta, GA and author of Front of the Class - How Tourette Syndrome Made Me the Teacher I Never Had; and also Sue Conners, the Tourette Syndrome Association's Education Specialist. Ivan has helped those of us in the North Texas Support Group by bringing in these presenters to further educate and provide us all with the correct information necessary in order to serve our children's needs and help them succeed.

When Ivan contacted me about the Parent Organizations Academy (the first and hopefully an annual event) and asked if I would like to participate and represent our Region, how could I refuse? I wasn't even sure when he asked me what I was getting myself into, but Ivan has helped us so much, it wouldn't have mattered, I would have returned the favor. What I didn't realize was, once again, he was helping me. And by providing me with information, I can then help others. I initially thought this was going to be an open forum with speakers coming in to discuss the dos and don'ts of special ed and we would be allowed to get in our two cents! It was that and so much more!

The topics presented and discussed were the Texas Assessment Program for Students With Disabilities; Transition Planning; Response to Intervention; Texas Behavior Support Initiative: Schoolwide Positive Behavior, and the Texas Complaint Resolution Process. In attendance were a representative from each of the twenty (20) Regions in Texas, and a representative from a group in each Region that supports parents who have children with disabilities and are in need of special services. On the last day of the conference, they provided time for each individual support service group to stand up and give a brief explanation of their organization and what the organization does. I have listed those websites and/or contact information at the end of this article.

When your child receives a diagnosis of any type of disability, it is devastating as a parent. You bounce back and forth between guilt and loss and all the child wants to know is "Am I going to die from this?" Then you go to the school with your diagnosis and usually what you find is the school wants to cooperate, but they don't want to do more than is required, and they won't tell you what you can and cannot do for your child. This is where your Special Education Department comes in. Every school district has one and each Region has a fully staffed department to assist you in determining what you are entitled to as far as special services for your child's individual needs

While attending the conference, after the first couple of hours I realized that the speakers were talking in code. Fortunately for me, I was familiar with most of it and I had Ivan next to me to define what I didn't know. However, I unintentionally ruffled a few feathers when I raised my hand to discuss the whole "code" issue. I cautiously explained that when a parent comes to the school for help, they have recently received a new diagnosis for their child, they are naturally upset, don't know what to do, and don't know what to expect from their school personnel. Then school personnel start talking about IEPs, BIPs, ARDs, RTIs, and the list goes on. The parents feel confused, maybe even like they lack the intelligence to help their own child; it's very frustrating. It is always important to ask questions if you don't understand any part of the process. School personnel hold many of these meetings and sometimes forget that we, as parents, don't always know what they are saying. NEVER feel embarrassed if you do not understand; you cannot help your child otherwise. Needless to say, the ruffled feathers were soon smoothed out!

IEP - Individual Education Plan

BIP - Behavior Intervention Plan

ARD - Admission, Review and Dismissal

RTI - Response to Intervention

Let's start with the Assessment Process. The general idea behind the process of getting your child "in the system" goes something like this: you make a request IN WRITING regarding your child's issues; you send this request not only to your campus personnel, and also to the special education department for your district, requesting your child be evaluated for Other Health Impaired (OHI) due to Tourette's syndrome and any other co-morbidities (e.g., OCD or AD/HD), if you are aware of any at the time of your TS diagnosis. If your child should go through some diagnostic testing at the school, tell your child about this testing process. Help them to understand it is not a test for a grade so they won't be nervous, and also let them know they are not in any trouble because of the testing.

Teachers can also refer a student for testing if they feel there is a problem in the classroom. BUT, keep in mind that school personnel cannot diagnosis your child. They may be able to suggest, but they are not licensed medical professionals and you should only accept a diagnosis from a doctor. After the diagnostic testing is completed, a group at your school will review the tests and make a determination as to whether your child should or should not require special services. This group at your child's school will usually consist of one or more your child's teachers, the counselor, the behavior specialist, the diagnostician, the content mastery lead, an assistant principal and/or the school nurse. It does not have to be all of the parties listed above; it can be a committee of just two or more. However, if you do not agree with the schools assessment, you can appeal.

If you and the school agree on the assessment of your child, you start the ARD process whereby you outline your child's IEP and BIP. This entire process, from your letter writing and receipt of such by school personnel, to the end result should all take place within ninety (90) days. However, most attendees at the conference agreed that they try to get the end results and meetings scheduled within thirty (30) days. All of this begins your journey into accommodations for your child to meet your child's specific needs, to ensure that your child is successful.

It was also discussed that if your school does not suggest certain services be brought in to help your child, then you need to suggest to the school what you, as the child's parent, think your child will need in order be successful. Who knows your child better than you? For example, if your child has trouble with handwriting and they do not offer a suggestion like note taking assistance, or offer the use of a computer for typing assignments, you should suggest this to them. All school districts have funds set aside for just this purpose. If each classroom does not provide a computer for your child, then you can suggest some type of word writer product that the student can carry with them from class to class, download to one main computer, and get print outs of all they have typed each day. They refer to these as ATs, Assistive Technology. Again, this is just an example, as there are many, many things to assist your child.

This article is based upon information gathered by Ms. Stark at the Parents Organization Academy Conference on November 15-16, 2007. The conference was sponsored by the Parent Coordination Network, Region 9 ESC, and the Texas Education Agency. If you have specific questions regarding your child or school, please contact Sheryl Kadmon, TSA of Texas Executive Director, at 281-238-8096.

Look for Part Two of this article in the next issue of "Bonne Nouvelle". Part Two covers the Transition/Graduation and Response to Intervention portions of the conference.