Age-related changes within the Tourette Syndrome

Alex D. PadillaRISE Program

2009

Abstract

The Tourette syndrome is a common neurological disorder that affects many people and it’s characterized by its symptoms which include motor and vocal tics. Even though the exact cause of this syndrome is not know, there are certain genetic studies that support the theory that the TS is inherited and it is a developmental disorder of transmission. The first signs of TS begin to show before adulthood and they tend to worsen as the child comes to his teenage years. In many people this tics lessen when they reach adulthood but there is a small percent that don’t.

Introduction

Tourette syndrome (TS) is, according to the National Tourette Syndrome Association (TSA), a common neurological disorder characterized by motor and vocal tics. Affected individuals typically have repetitive, stereotyped movements or vocalizations, such as blinking, sniffing, touching the ground, or tensing of the abdominal musculature. Other neurobehavioral manifestations include attention-deficit-hyperactivity disorder, obsessive-compulsive disorder, poor impulse control, and other behavioral problems. Symptoms vary significantly from one patient to another. Depending on the person they can have both the tics and the vocal outbursts or just one of them. Once considered a rare syndrome, epidemiological studies have shown that it is a relative common syndrome occurring in up to 1% of the children, especially in boys.

The exact cause for the TS remains unknown, but several genetic studies suggest and support the theory that the Tourette syndrome is an inherited developmental disorder of neurotransmission. . Volumetric MRI suggests that the normal asymmetry of the basal ganglia is lost in affected individuals. (Black 2009)

Symptoms and Diagnosis

There are two main types of tics, movement-based tics or motor tics and vocal tics. TS most common symptom is the tics which are involuntary, rapid, sudden movements or vocal outbursts that occur repeatedly. Tics typically begin at about 6 or 7 years of age, initially presenting in

midline body regions where there are many muscles: the head, neck and facial region. Movement-based tics are called motor tics. Involuntary sounds produced by moving air through the nose, mouth, or throat is called verbal tics, vocal tics, or phonic tics. One of the most famous symptoms is the Coprolalia which is saying socially unacceptable or obscene phrases. Another one is Copropaxia which is performing obscene or forbidden gestures, Echopraxia that is imitating movements that were seen by the patient, Echolalia which is the repetition of words that were heard by the patient and Palilalia which is repeating their own words, phrases or sounds. Coprolalia is estimated to occur in less than 15% of patients with Tourette syndrome in spite of it being the most famous symptom.

Fig. 1: Tourettes syndrome and other tic disorders. Graphic shows the relative likelihood of lifetime sensory tics in a given region, as based on self-report of patients with Tourettes syndrome. Taken from Black, K. 2009

Diagnosis for TS is made strictly by observing the patients behavior. Before a patient is officially diagnosed with TS, he must meet a certain criteria stipulated by the TSA. The criteria for diagnosing a person with TS includes the following: both multiple motor and one or more vocal tics have been present at some time, the tics occur many times a day nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months, the onset is before age being 18 years, and The disturbance is not due to the direct physiological effects of a stimulant or general medical condition. (TSA 2009)

Treatment

First of all, no curative or preventive treatments are known based on the fact that the exact cause of this syndrome remains unknown. Nevertheless, treatment is not always needed according with the TS experts. Even though it is not necessary, people tend to treat their tics and other OCD and ADHD symptoms associated with the TS with neuroleptic drugs.

TS in children and adolescents

The TS has a prepubertal onset. This means that initial symptoms of TS begin generally between 6 or 7 years of age. The severity of tics is age dependent, with peak severity between 8 and 12 years of age. It is around this time that tics usually have the greatest impact on a young person's self-esteem and self-image, peer relationships, and family relationships. Social or educational dysfunction occurs in around half of TS patients. Children below 10 years of age are often not aware of an urge associated with their tics. For them, tics virtually constitute an involuntary movement disorder. However, most children over the age of 10 years would report experiencing an irresistible somatosensory urge that probably reflects a defect in sensor motor gating. These urges (or the need to tic) that intrudes into the child's conscious awareness, resulting in distraction and distress, are relieved after the tic. Children often report a decrease in tics during goal-directed or focused activity and intense concentration, and they report an increase in tic severity during stress or excitement. Clinical and epidemiological studies point to a decrease in tic symptoms over the course of adolescence, and it has been suggested that this improvement with age is the result of frontal compensatory responses, with frontal cortices becoming more efficiently connected to the striatum and to the motor and sensor motor cortices. (Eapen and others 2009)

TS in adults

Young people with TS are usually told that their symptoms will lessen once they reach adulthood, and for most that’s the case. However, a significant group of people with TS go through their 20's and 30's waiting for the promised improvement that never comes. Often, hitting 40 or 50 marks the turning point. According to the TSA, there is a tendency for the improvement of tics in the majority of patients whit TS.

Conclusion

The Tourettes Syndrome is a neurobiological disorder that its onset is usually at an early stage of childhood. Also it is generalized that most patients when they reach their adulthood, they have a significant decrease in their symptoms while a minor percentage of adults remain with the tics. Therefore an early conclusion can be said that depending on the age of the person is the severity of the symptoms but with no cure or treatment for the syndrome, there is no assurance that this symptoms will disappear forever. Furthermore study is needed to find the real cause of this syndrome and therefore find a cure or an effective treatment for the TS.

References

Black, K. 2009. Tourettes Syndrome and Other Tic Disorders.

Bloch, M., Leckman, J. 2009. Clinical Course of Tourette Syndrome. Journal of Psychosomatic Research 67: 497-501.

Church A., Dale R, Lees A, Giovannoni G, Robertson M. 2003. Tourette’s syndrome: a cross sectional study to examine the PANDAS hypothesis. J Neurol Neurosurg Psychiatry 2003; 74:602–607

Eapen, V., Crncec, R. 2009. Tourettes syndrome in children and adolescents: Special considerations. Journal of Psychosomatic Research 67: 525-532

Grados, M.; Mathews, C. 2009. Clinical phenomenology and phenotype variability in Tourette syndrome. Journal of Psychosomatic Research 67: 491-496.

Robertson, M.; Eapen, V.; Cavanna, A. 2009. The international prevalence, epidemiology, and clinical phenomenology of Tourette syndrome: A cross-cultural perspective. Journal of Psychosomatic Research 67: 475-483.