Definition of Tourette Syndrome - Case Study Example

Tourette syndrome Definition: Tourette syndrome (TS) is a rare habit disorder with onset in childhood or adolescence between the ages of 2 and 15 and characterized by multiple tics1, compulsive barking and grunting or shouting obscene words. These tics characteristically wax and wane. It is named after the French doctor Georges Gilles de la Tourette, who first described the condition in 1885. Some consider it an inherited neurological disorder (Dalton 2000). Incidence and prevalence: The prevalence rate is 0.5/1000 individuals. It appears prior to 7 years in 50% of the cases. In most cases it is a genetic condition thats inherited, or passed on from parent to child. It affects boys 3 to 4 times more than girls. It is more common in whites than in other races (Dalton 2000). Etiology: The etiology is uncertain. It is thought that genetic, neurobiologic, psychologic and environmental factors probably have a role (Dalton 2000). Genetic studies have shown that the majority of cases of TS are inherited. A person with TS has about a 50% chance of passing the gene to one of his or her children. The exact mode of inheritance is not yet known and no gene has been identified. Although early family studies suggested an autosomal dominant mode of inheritance, more recent studies suggest that the pattern of inheritance is much more complex (NINDS). Some researchers have suggested that there is lack of normal symmetry within the striatum as seen in neuroimaging (Dalton 2000). The tics are believed to result from dysfunction in cortical and subcortical regions; the thalamus, basal ganglia and frontal cortex. Drugs that increase dopaminergic action precipitate or worsen the symptoms in this condition. Hence abnormal metabolism of the neurotransmitters dopamine and serotonin might be involved with the disorder. There is evidence to suggest that a subgroup of children who develop Tourette syndrome suffer with pediatric autoimmune neuropsychiatric disorder in which antibodies to group A streptococcal infections cross react with basal ganglia tissue and subsequently precipitate symptoms (Dalton 2000). It is not contagious. Clinical features: In 50% of the cases, the symptoms manifest before 7 years of age. The first symptoms usually are involuntary movements (tics) of the face, arms, limbs or trunk. The symptoms are usually mild. Although in some, they can be bizarre. Primary stuttering usually begins as an atypical development during speech development and learning. The children initially repeat consonants and then as they learn words and phrases, they repeat them. Children may present with motor tics (sudden, apparently uncontrollable movements like exaggerated blinking of the eyes, sniffing and facial movements) or vocal tics (such as coughing and throat clearing). These tics are frequent, repetitive and rapid. The most common first symptom is a facial tic (eye blink, nose twitch, grimace), and is replaced or added to by other tics of the neck, trunk, and limbs. These symptoms can become severe and last longer when the child is under stress. Some children, who can understand their habit, become anxious and they may develop behavioral changes. They may try to suppress their tics for a short time. Gradually tension builds, and it eventually has to be released as a tic. While trying to control the tic, the child may not be able to focus on anything else. Hence the child or adolescent may not be able to pay attention in class or study well leading to poor academic performance. About 5% of children stutter. Tics may be either simple or complex. Simple motor tics involve just a few muscles. Some examples are eye blinking and grimacing. In contrast, complex motor tics usually involve more muscle groups. For example, a person might touch a body part or another person repeatedly. In rare cases, people with TS might have a tic that makes them harm themselves, such as head banging, body rocking, teeth grinding, hitting or biting one’s own body parts, hair pulling, etc. Simple vocal tics can be throat clearing, sniffing, or humming, whereas complex vocal tics can involve repeating other peoples words (a condition called echolalia) or involuntary swearing (called coprolalia). Most cases resolve spontaneously. It remits more readily in girls. About 20% of children continue to suffer even in adulthood. Many children with TS may have other associated problems like attention deficit hyperactivity disorder, obsessive compulsive disorder, learning disabilities like dyslexia, difficulties with impulse control and sleeping problems. They have normal life expectancy and intelligence (TSAI). Diagnosis: Before TS can be diagnosed, the child must have tics for at least a year and there must not be a tic-free period longer than 3 months. TS may be diagnosed when a child exhibits both multiple motor and one or more vocal tics although these do not need to be concurrent over the period of a year, with no more than three consecutive tic-free months (DSM IV TR). The onset must have occurred before the age of 18, and cannot be attributed to any direct physiological effects of a substance or a general medical condition. Some mild cases may be undetected. There is no specific diagnostic test for TS. The diagnosis is purely based on history and clinical examination. Magnetic resonance imaging tests, computerized tomography scans, electroencephalograms, or blood tests may be done to rule out other conditions that might have symptoms similar to TS (seizures, hypothyroidism). In teenagers and adults presenting with a sudden onset of tics and other behavioral symptoms, urine screening for cocaine and other stimulants must be done. In those with a family history of liver disease, serum copper and ceruloplasmin levels must be done to rule out Wilson’s disease. Treatment: There is no cure for TS. Most children get out of this problem spontaneously. Also, as the child grows, the intensity, duration and frequency of tics decrease. Explanation, reassurance and education are often sufficient treatment. If the symptoms interfere with daily activity and schooling, some doctors advice medications. Dopamine antagonists like haloperidol or pimozide may be useful. These drugs reduce tics by 65%. They can have long-term and short-term adverse effects. Anti-hypertensives like clonidine are also used to treat tics. They have variable efficacy, but a lower side effect profile than the neuroleptics. Though TS is not a psychological disorder, consulting a psychologist or a psychiatrist may help in counseling. The children and adolescents cope with stress better, and learn relaxation techniques. Supportive psychotherapy and cognitive behavioral therapy may help to ameliorate depression and social isolation, and to improve family support Each child with TS will cope differently with its physical, emotional, and social challenges. Since usually there is no restriction in activities, these children should be able to enjoy and participate in the same activities as their peers. Getting children engrossed in activities like sports, exercise, or hobbies, can make their tics milder and less frequent. Creative activities such as writing, painting, or making music help focus their mind on other things. Adolescent and adults with TS can feel more in control of their lives by researching TS, asking their doctors plenty of questions, and taking an active role in their treatment. Children may be encouraged to help others so that knowing that he or she helped others might help build the childs confidence, and lessen any self-consciousness that the child might have about feeling different. Children and adolescents with TS who have learning difficulties are candidates for psychoeducational testing. Co-morbid conditions like ADHD must be identified and treated appropriately, because these can lead to disruptive behaviors, impaired functioning, or cognitive impairment. Differential Diagnosis: 1. Transient tic disorder: This consists of multiple motor tics, phonic tics or both, with duration of between four weeks and twelve months. 2. Chronic tic disorder: In this condition, either single or multiple, motor or phonic tics (but not both) are present for more than a year. 3. Tourettism: This refers to the presence of Tourette-like symptoms secondary to other disorders which may be genetic (Down’s syndrome, Fragile X-syndrome), post-infectious (Syndeham’s chorea, encephalitis), developmental (Autism, static encephalopathy) or due to drugs (Levodopa, phenytoin), toxins (Mercury, Wax venom) or insults (hypoglycemia, head trauma). Prognosis: There is no cure for TS. The disorder is generally lifelong and chronic. The condition in many individuals improves in the late teens and early 20s. Some may actually become symptom-free or no longer need medication for tic suppression. The disorder is not degenerative and individuals with TS have a normal life expectancy and normal intelligence. They are productive and people with TS are present in all walks of life. However, the associated neurobehavioral problems such as depression, anxiety, mood swings, and antisocial behaviors can persist and cause impairment in adult life. References Dalton, R. (2000). Nelsons Text Book of Pediatrics: Habit disorders. 16th edition. Pages: 75-76. Singapore: Harcourt Asia PTE limited. Syndrome Fact Sheet. National Institute of Neurological Disorders and Stroke (NINDS). Retrieved 28th July 2007 from http://www.ninds.nih.gov/disorders/tourette/detail_tourette.htm What is Tourette Syndrome? Tourette Syndrome Association Incorporation (TSAI). Retrieved 28th July 2007 from http://www.tsa-usa.org/Medical/whatists.html. What is Tourette Syndrome? Tourette Syndrome. Retrieved 28th July 2007 from http://members.tripod.com/~tourette13/ Read More