Tourette’s syndrome, also known as Gilles de la Tourette’s syndrome, is a neurobehavioral disorder which was first recognized as a medical condition almost two centuries ago. Originally, Tourette’s syndrome was perceived to be a rare condition and only a few papers were published about the syndrome in the 19th century after its discovery.
This has since changed and in the present day, a substantial number of papers have been documented. In the early days of the study of Tourette’s syndrome, scientists described it as a disorder that demanded immediate clinical conceptualization of the mysteries leap between the body and mind.
The patients who were studied mostly stated that their tics originated from within, from an urge which they felt was too difficult to resist. As research continued to shed more light about the condition, a debate arose on whether Tourette’s syndrome should be treated as a motor-form of obsessive –compulsive disorder or whether obsessive-compulsory disorder is actually a mental version of the Tourette’s condition.
A careful observation of the Tourette’s syndrome reveals that the epistemological issues confirm that both perspectives are both accurate. In order to help those suffering from the condition, it is important to understand the issues surrounding Tourettes’s syndrome including its cause, diagnosis and treatment.
History of the condition
The first lucid description of the condition was made with the documentation of Marques de Dampierre’s (1799-1884) condition by a physician known as Jean-Marc Gaspard Itard. Dampierre was an upper class, intelligent and well mannered woman who was known for involuntarily speaking obscene words in the middle of a conversation that otherwise seemed normal (Kushner, 2000).
Itard reported Dampierre’s unusual case in a medical journal describing how she often felt embarrassed by her vocal tics even though she had no control over them. Several years later, in 1885, Georges de la Tourette, a French neuropsychiarist documented the case of eight other patients who exhibited vocal and motor tics that were associated with the syndrome.
It was during this period that Tourrette’s mentor, who was a medical doctor, named the illness after him. Tourette’s descriptions of the symptoms exhibited by the eight individuals emphasized on three symptoms shared by all the victims namely multiple tics, echolalaia, and coprolaliua.
Echolalaia and coprolalia are the involuntary echoing back of what others say, and the involuntary and inappropriate swearing, respectively. Since the time of Tourette’s documentation, numerous other studies have been carried out and documented concerning the condition.
Symtoms of Tourette’s syndrome
The condition is characterized by a reoccurring pattern of tics, both vocal and motor. Tics are uncontrollable movements and sounds that occur when the brain communicates to one or more muscles to contract resulting in unwanted movements and sounds.
The sounds and movements usually occur in a sudden manner and repeat without warning. The motor tics may either occur in brief, rapid or darting sessions at the face, shoulders or in other cases, may involve more complex movements. Similarly, vocal tics may either take the form of simple sounds or complex sounds
The tics may either be simple or complex depending on the number of an individual’s body parts that are involved in the tics. Single movements involving a single body part are referred to as simple tics. In relation to voice tics, sounds such as sniffing and grunting which are the result of air being forced through the throat, nose or vocal cords are referred to as simple vocal tics.
Complex tics entail utilization of multiple muscles to cause multiple movements. An example of complex motor tics is the involuntary making continuous jumping movements. Examples of complex motor tics include echopraxia and copropraxia which are the copying of another individual’s deeds and making of gestures that may appear offensive, respectively.
Complex vocal tics include echolalia, palilalia and coprolalia (Hamiton, 2005). The coprolalia symptom is the most popular of these symptoms and is also known as cursing or publicly saying inappropriate words. An example of an individual who experienced coprolalia was Madame Diamperre mentioned above.
Causes of Tourette’s syndrome
The process of determining the exact cause or causes of Tourette’s syndrome is still a subject of extensive research. What has clearly emerged from the numerous studies is that the syndrome is an inherited neurological disorder that cannot be outgrown.
Many researchers believe that abnormal metabolism of the neurotransmitter dopamine is what triggers the occurrence of the condition. Other chemicals found in the brain such as serotonin have also been associated with causing the condition. In some rare occasions, a virus or head trauma, or oxygen deficiency during birth have been known to cause the disorder.
Genetics
Most scientists believe that either parent having the gene or genes that cause Tourette’s syndrome can pass them to their child. In incidences where one of the parents is a carrier of the gene, each of the children has a fifty percent chance of inheriting the gene and later on developing the symptoms associated with the syndrome or in some cases, may exhibit no symptoms at all.
Among the children who inherit the Tourette’s syndrome gene, the probability of the male children developing the syndrome’s symptoms is three times that of the female children (Leckman & Cohen, 2001). Studies have also shown that approximately a third of the female children who inherit the gene show no signs associated with the disorder while the same is true for only one out of a hundred male children.
Of all individuals who develop the condition, only a tenth of them ever develop symptoms serious enough to warrant medical interventions.
Neorological Correlates
In recent research on Tourette’s syndrome, scientists have examined the neurological correlates of the syndrome mostly by using brain imaging procedures which enable physicians identify any abnormal or neuroanatomical differences among individuals with Tourette’s syndrome.
Generally, Tourette’s syndrome is thought to be related to abnormalities in the activities of neurotransmitter which then trigger the disinhibitation of circuits that link frontal lobe regions with subcortical structures (Clickerman & Ellison, 2009).
More specifically, Tourette’s syndrome has been said to occur when reduced motor inhabitation occur caused by the impaired modulation of neurological activity within the thalamus and the basal ganglia.
Consistent results have associated the tics experienced by those suffering from Tourette’s syndrome with the basal ganglia (Clickerman & Ellison, 2009). This has been possible through the use of brain imaging techniques which have revealed that children suffering from Tourette’s syndrome have reduced volumes of basal ganglia and also, their basal ganglia do not have the typical asymmetry that normal ones have.
Research has shown that basal ganglia may activate the pre-motor area, motor cortex, and the cingulated motor area resulting in tics because the basal ganglia normally interacts with other areas of the brain, including the motor thalamus and motor cortex, to both determine the functioning of the motor and to produce movements.
Basal Ganglia could also be involved in causing the symptoms of attention deficit hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD).This can be used to explain the high rates of comorbidity associated with ADHD, OCD and Tourette’s syndrome.
Other studies have indicated that children and adults with Toureette syndrome experience significant reductions in caudate volume compared to control populations. Adults with Tourette’s syndrome also exhibit reduced volumes of globus pallidus and putamen.
Yet another study revealed that caudate volumes in children with Tourette’s syndrome could be used to predict the severity of OCD and Tourette’s syndrome when the individual reaches early adulthood. All these results appear consistent with earlier findings that implicated the limbic system to Tourette’s syndrome and proposed that Tourette’s syndrome results from disinhibition of functions associated with the limbic system.
Dopamine
It is a common belief that abnormalities in the dopamine neurotransmission have an important role in the pathophysiology of Tourette’s syndrome.
This hypothesis arises partly from the clinical observation that reveal that dopamine receptors decrease tics and from the clinical observation that the potentiation of dopamine transmission with stimulant medication results in either an increase or in a reduction in the severity of tics.
However, measurements of presynaptic content, dopamine receptors, or function in pathological or imaging studies have given contradicting results about these findings (Weyandt, 2006).
Despite the contradicting data and limitations in studies, the dopamine hypothesis of Tourette’s syndrome is still regarded as important. There may be considerable abnormalities of functions mediated by dopamine even without primary abnormalities of post-synaptic receptors or dopamine neurons.
Diagnosis of Tourette’s syndrome
Even though tics are the most obvious of symptoms exhibited by an individual suffering from Tourette’s syndrome, not all individuals with tics have Tourette’s syndrome. There are many other medical conditions affecting the nervous system that can cause tics which are unrelated to Tourette’s syndrome.
For instance, head injuries caused by a blow or blows to the head can leave an individual with tics. There are also a range of ticks that may occur during one’s childhood due to the development of the nervous system. These tics are normal and disappear on their own after some time as the individual develops.
Some children under the age of five have been observed to develop tics for a few months every years especially when their nervous system is under stress.
These tics are usually mild and hardly noticeable and even tics that are visible rarely interfere with the day-to-day activities of those experiencing then and should therefore not be a source of concern. It is those tics that last for a period exceeding a year that may be related to Tourette’s syndrome.
The waning and waxing of the symptoms of Tourette’s syndrome can sometimes delay a medical referral. It is therefore important that parents and teachers are aware of the criteria used to diagnose Tourette’s syndrome.
Even though both motor and vocal tics must be present for the diagnosis of Tourette’s syndrome, other disorder tics may also be present and should also be addressed by a physician. Before the diagnosis of Tourette’s syndrome, the following conditions have to be satisfied (Boyles & Contadino, 1998):
- Both multiple vocal and motor tics must be present though they not necessarily occur together.
- The individual has to have experienced tics severally in a single day for either daily for a period exceeding a year. In case of tic-free breaks, these must not have exceeded three months continuously period.
- The affected individual must have also been affected by these tics in the various aspects of his or her life.
- These disturbances must not be due to alcohol use or the use of any other drugs or other conditions affecting the central nervous system, an example being Parkinson’s disease.
- The onset of tics must have occurred before the individual attained the age of eighteen.
As aforementioned, individuals with Tourette’s syndrome may also experience symptoms of ADHD. However, the phenomenology of ADHD symptoms in individuals with Tourette’s syndrome is similar to ADHD symptoms in individuals without Tourette’s syndrome.
Some experts in this field have suggested that individuals suffering from both ADHD and Tourette’s syndrome experience more impulsivity and over activity and fewer problems with inattention compared with subjects suffering from ADHD alone.
Even with these suggestions, there is yet to be a well documented study of rigorous tests comparing patients suffering from both Tourette’s syndrome and ADHD to those suffering from ADHD alone to determine any differences in symptoms exhibited.
Treatment of Tourette’s syndrome
When it comes to the treatment of this condition, only symptomatic treatments are currently available (Frucht & Fahn, 2005). It is therefore important to determine which symptom mostly interferes with the individual’s lifestyle and seek treatment for that symptom.
In some patients, psychiatric comorbodity is the main disabling feature and treatment of this symptom alone may be sufficient. Tics themselves do not require treatment but there are medicines that can be used to reduce their occurrence and eventually treat them.
Given that Tourette’s syndrome is a result of biological malfunctions of the brain, psychological and behavioral therapies alone are less successful in treating the syndrome than when these are combined with medication.
Medications that target the activities of the brain’s chemical messenger dopamine are usually the most effective in managing the syndrome’s symptoms. In the recent times, technological advancements have made it possible for neurological techniques to be used to treat severe symptoms of the syndrome.
Even with the high level of success achieved in treating symptoms of the syndrome using medications, it is important that these treatments be accompanied by psychological or behavioral therapies in order to achieve the highest level of symptoms containment.
When it comes to containing tics, it is important for first identify what triggers them and then seek ways to avoid these triggers or develop mechanisms to reduce the effects of these triggers.
For instance, stress management techniques such as relaxation and exercise therapies can be applied to reduce the incidences of tics and the anxieties that they provoke. Other behavioral therapies such as habit reversal training have also proved successful in reducing severity of the symptoms of Tourette syndrome.
References
Boyles, N.S. & Contadino, D. (1998). The learning differences sourcebook. New York, NY: McGraw-Hill Proffesional.
Clickerman, M.S. & Ellison, P.A.T. (2009). Child Neuropsychology: Assessment and Interventions for Neurodevelopmental Disorders, 2nd Edition. Lansing, MI: Springer.
Frucht, S.J. & Fahn, S. (2005). Movement disorder emergencies: diagnosis and treatment. Totowa, NJ: Humana Press.
Hamilton, D. (2005). Teaching students with Tourette syndrome: an educator’s guide to managing and understanding students with Tourette syndrome and associated disorders. Delmar, NY: Diane Hamilton.
Kushner, H. (2000). A cursing brain?: The histories of Tourette syndrome. New York, NY: Harvard University Press.
Leckman, J.F. & Cohen, D.J. (2001). Tourette’s Syndrome – Tics, Obsessions, Compulsions: Developmental Psychopathology and Clinical Care. New York: John Willey and Sons.
Weyandt, L.L. (2006). The psychological bases of cognitive and behavioral disorders. Mahwah, NJ: Routledge.