Tourette Syndrome and Down Syndrome

Tourette syndrome (TS) is a hereditary, chronic neuromuscular condition consisting of motor and vocal tics. Tics are sudden, involuntary, brief, repetitive movements and vocalizations.*

Motor tics can include jerking head movements, sudden movement of the arms and legs, facial twitches, and others. Vocal tics can include guttural sounds, yelling out, repeating a word or phrase, and others. 

Diagnosing TS

The criteria used to diagnose TS include: 

• Tics start before age 18.

• Tics are present for at least one year.

• Tics are not caused by a stimulant medication or a medical condition.

• In classic TS, at least two different motor tics as well as one vocal tic are present (the motor and vocal tics do not necessarily occur at the same time).

Attention-deficit disorders (ADD) and/or obsessive-compulsive disorders (OCD) often occur with TS but are not required for diagnosis. In our experience, adults with Down syndrome and TS have often been diagnosed with ADD in childhood and OCD later in childhood or early adulthood. Often tics are not appreciated as part of the condition until later in childhood or adulthood. Sometimes the tics may have been overlooked or thought to be side effects of medications (tics can increase with the stimulants frequently used for attention-deficit hyperactivity disorder). 

We have seen a number of adults or adolescents with Down syndrome who have a Tourette-like disorder. We often label this as Tourette-like (or atypical Tourette syndrome) because the full complement of criteria is usually not present. Most commonly there is an absence of vocal tics. 

Treating TS

In our experience, the treatment for people with Down syndrome who have TS is similar to that for people without Down syndrome who have TS. In our experience, people with a dual diagnosis of Down syndrome and TS respond best to a multifaceted treatment approach involving both behavioral interventions and medications. 

Behavioral strategies for dealing with tics include: 

• Addressing stress that may increase tics.

• Engaging in active pursuits such as sports or recreation activities may reduce tics.

• We have also found it helpful to keep the body part that is most often part of the tic busy. 

Boredom and under stimulation may increase the occurrence of tics. People who are productive and busy are generally happier and less stressed. However, it is important to note that tics are involuntary. The above efforts will have varied benefit depending on the individual. 

Medication considerations for people with TS include: 

• Stimulants used for ADD may cause an increase in tics.

• Medications for OCD may improve the obsessive and compulsive aspects of TS but not the tics. Approved medications for OCD (for adults include: fluoxetine, fluvoxamine, paroxetine, sertraline, and clomipramine. We have seen successful use of all of these medications but find sertraline to work well with limited side effects.

• Medications for tics include:

  • Clonidine or guanfacine. They can help reduce tics. These medications have an FDA indication for tics in children (but not adults but your health care provider may choose to use them "off label" - used for an indication that is not FDA-approved). We have seen some benefit with these medications.

  • Clonazepam. The use of this medication is also "off label." We have limited use of this medication for reducing tics. Clonazepam can cause sedation and so we are less likely to use it for this purpose. 

• Atypical antipsychotic medications. We often have had the best success in treating TS in people with Down syndrome with these medications. These medications are especially helpful in reducing the intensity of tics and the more debilitating compulsive behaviors. We have used pimozide, risperidone, olanzapine, quetiapine, or aripiprazole. Only pimozide has FDA approval for treating TS in adults and children. Aripiprazole is approved for treating TS in children. There are some newer atypical antipsychotics available, but we have not yet used them for TS. Side effects can be a limiting factor for the atypical antipsychotics. Weight gain and sedation are particular problems, and elevated blood sugar and type 2 diabetes mellitus can also occur. We recommend monitoring blood sugar on a periodic basis while taking these medications. Tardive dyskinesia, a movement disorder, is also a potential serious side effect. 

*Tics are sometimes difficult to differentiate from stereotypies. A stereotypy (also known as self-stimulatory behavior) is defined as a motor behavior that is repetitive, often seemingly driven, and nonfunctional. It includes repetitive motor behaviors and the repetitive movement of objects. Stereotypies can be rhythmic such as making wavelike motions with the arms or hands. One of the ways in which tics and stereotypies differ is that tics are associated with a premonitory urge (before the tic occurs, the person senses that the tic is coming on) while stereotypies are not.

Additional information is available in chapter 24 of Mental Wellness in Adults with Down Syndrome, which is available as a free PDF in our Resource Library.