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Tourette Syndrome
Gilles De La Tourette Syndrome

Tourette syndrome is a common tic disorder that is a subset of hyperkinetic movement disorders. Hyperkinetic disorders are characterized by abnormal involuntary movements. These can range from jerks, chorea, and dystonia to tics.

Images

WIKIDATA, Public Domain
WIKIDATA, Public Domain
WIKIDATA, Public Domain
WIKIDATA, Public Domain

Presentation

The tics may be either simple or complex. Simple tics may be:

  • Blinking
  • Shoulder shrugging
  • Head nodding or jerking

Complex ones include:

  • Scratching 
  • Body gyrations
  • Kicking
  • Jumping
  • Seductive and obscene gestures 

The tics are suppressible, but they are associated with an irresistible urge and sensations that are relieved when the tic occurs. Involuntary vocalizations, may range from sounds to words. The words may be obscene (coprolalia) which occurs in 40% of patients with word tics. Some may repeat a single word many times. Many also experience copropraxia (obscene gestures), echopraxia (mimicking of gestures) with unusual fixations and fanaticises. The neurologic exam is usually normal except for the tic [9].

Entire Body System

  • Crying

    "I tried to hide it as much as I could from the cameras," the 29-year-old said and started to cry. "People noticed and I think that made it worse — and let me tell you, people are mean. People are very, very mean. They are so quick to judge." [intouchweekly.com]

    Is this like your desperate cry for attention? Reporter: This girl, just a few years older than our actors, reacts with anger. Is this contagious? Were you born ignorant? Reporter: And she has no problem calling out our bullies. [abcnews.go.com]

    I often started crying right there on the field. I was 10 when the symptoms began to appear. First came the touching: I walked through the house tapping certain objects in a particular order. Touch the railing. Touch the door frame. [web.archive.org]

    The second patient reported complete remission of coprolalia soon after surgery, and the third reported a reduction in tics and obsessive crying by 1 year after surgery. [ncbi.nlm.nih.gov]

  • Family History of Depression

    […] and depression have a positive family history of depression. [ncbi.nlm.nih.gov]

Gastrointestinal

  • Food Intolerance

    intolerance to its presence. [dx.doi.org]

Musculoskeletal

  • Muscle Twitch

    The side effects of typical neuroleptics include: drowsiness shaking trembling muscle twitches spasms Side effects of both typical and atypical neuroleptics include: weight gain blurred vision constipation a dry mouth Adults with Tourette's syndrome who [hse.ie]

    English Muscle weakness, muscle twitches or a pounding heart beat (palpitations). [mymemory.translated.net]

    Side effects include anxiety, restlessness, confusion, muscle twitching, muscle spasms, seizures, unusual behavior, thoughts of hurting self or others, unusual bleeding, trouble sleeping, and seeing or hearing things that are not there. ( 14 ) Oxazepam [draxe.com]

Psychiatrical

  • Impulsivity

    SIB, obsessions, compulsions, tic severity, attention deficit hyperactivity disorder related impulsivity, risk taking behaviours, and rages were systematically assessed in all subjects. [ncbi.nlm.nih.gov]

    […] other conditions depression impulse control disorders in adults (1,2) Reference: (1) Cavanna AE, Seri S.Tourette's syndrome. [gpnotebook.co.uk]

  • Psychiatric Manifestation

    Trial Registration clinicaltrials.gov Identifier: NCT00139308 Tourette syndrome (TS) is characterized by motor and vocal tics associated with various psychiatric manifestations, which can cause major familial and social disability. 1 In patients with [dx.doi.org]

    Neurologic and psychiatric manifestations of celiac disease and gluten sensitivity. Psychiatr. Q. 2012, 83, 91–102. [ Google Scholar ] [ CrossRef ] [ PubMed ] Bushara, K.O. Neurologic presentation of celiac disease. [mdpi.com]

Face, Head & Neck

  • Facial Grimacing

    Some of the more common tics include: eye blinking and other vision irregularities, facial grimacing, shoulder shrugging, and head or shoulder jerking. [healthcare.utah.edu]

    Tarimas: /tʊˈrɛts/ tourette sindromas Paaiškinimas anglų kalba neurological disorder characterized by facial grimaces and tics and movements of the upper body and grunts and shouts and coprolalia A neuropsychological disorder related to alterations in [angluzodynas.lt]

    Common tics include: Eye twitches Facial grimaces Throat clearing Sniffing Grunting Head or shoulder jerking Shoulder shrugging Words or phrases Deep brain stimulation surgery for Tourette's syndrome at UCLA Behavioral therapy and medicines are the standard [neurosurgery.ucla.edu]

    Tic Categories Two categories of TS tics and some common examples are: Simple: Motor - Eye blinking, head jerking, shoulder shrugging and facial grimacing; Vocal - Throat clearing, yelping and other noises, sniffing and tongue clicking. [web.archive.org]

    For most, Tourette’s involves: tics, along a range of simple (e.g., rapid eye-blinking, facial grimacing, shoulder-shrugging) to complex (involving several muscle groups, such as hopping, bending, or twisting); and vocalizations, also along a range of [parentcenterhub.org]

Neurologic

  • Hyperactivity

    The pathophysiology of TS mainly involves dysfunction of basal ganglia-related circuits and hyperactive dopaminergic innervations. [ncbi.nlm.nih.gov]

    Methylphenidate and dextroamphetamine treatments of hyperactivity: are there true non-responders? Psychiatr Res 36: 141–155, 1991. CrossRef Google Scholar 138. Erenberg G, Cruse RP, Rothner AD. [springerlink.com]

  • Vocal Tic

    Symptoms usually begin with simple motor or vocal tics which then evolve into more complex motor and vocal tics over time. [ncbi.nlm.nih.gov]

    Vocal tics can also be utterances of inappropriate, undesired statements or obscenities (coprolalia). Like somatic motor tics, vocal tics can also be divided into simple and complex tics. [web.archive.org]

    Tourette Syndrome is characterized by motor or vocal tics that last more than one year. Tics are abrupt, repetitive and involuntary muscular contractions or sounds. Besides motor or vocal, tics may be simple or complex. [news.softpedia.com]

  • Tic Disorder

    BACKGROUND: Few studies have investigated mortality risk in individuals with tic disorders. [ncbi.nlm.nih.gov]

  • Coprolalia

    Coprolalia is one of the most distressing symptoms in Gilles de la Tourette syndrome. [ncbi.nlm.nih.gov]

    Despite widespread publicity, coprolalia/copropraxia is uncommon with tic disorders. Neither echolalia (echo speech) or coprolalia/copropraxia is necessary for the diagnosis of Tourette syndrome. [web.archive.org]

    Obsessive-compulsive behavior, attention-deficit disorder, and other psychiatric disorders may be associated; coprolalia and echolalia rarely occur; autosomal dominant inheritance. [medical-dictionary.thefreedictionary.com]

  • Grunting

    Examples of physical tics include: blinking eye rolling grimacing shoulder shrugging jerking of the head or limbs jumping twirling touching objects and other people Examples of vocal tics include: grunting throat clearing whistling coughing tongue clicking [nhs.uk]

    A master at concealing twitches, tics, grunts and barks, Brian Conaghan couldn't keep his wife in the dark about his Tourette's forever... I agonised over telling her. For over a year I agonised. [telegraph.co.uk]

    Tics that involve movements (motor tics) include things like blinking, frowning, and head jerking Tics that involve sounds (vocal tics) include things like throat clearing or grunting Tics may come many times a day or only every few months Tics that involve [merckmanuals.com]

    […] tu̇-ˈrets- \ variants: or Tourette syndrome \ tu̇-ˈret- \ : a familial neurological disorder of variable expression that is characterized by recurrent involuntary tics involving body movements (such as eye blinks or grimaces) and vocalizations (such as grunts [merriam-webster.com]

Workup

Once the history and exam is taken there is no further workup required to make the diagnosis.

Diagnostic criteria for Tourette syndrome (DSM-IV) are as follows:

  • Both multiple motor and 1 or more vocal tics have been present at some time during the illness, though not necessarily concurrently. A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization.
  • The tics occur many times a day (usually in bouts) 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 18 years.
  • The disturbance is not due to the direct physiologic effects of a substance (eg. stimulants) or a general medical condition (eg. Huntington disease or postviral encephalitis) [9] [10].

Treatment

  • Dopamine agonists/antagonists reduce the frequency and intensity of the tics in up to 80% of the patients. 
  • Habit reversal training includes tic awareness training to try and help the patient prevent the tic before it occurs. The other is compete response training to train the patient to perform a movement that will not allow the tic [11].
  • Botulism toxin may be used to paralyse small muscle groups to try and reduce the tic. 
  • Others drugs such as topiramate may be useful.

Prognosis

The condition can persist throughout life, in children it can go into remission and 50% will have reduced tics by the age of 18. Some tics are not distressing or life limiting, but other can cause a lot of distress and anxiety. The vocal sounds and words can be profane or loud causing the sufferer to be very conscious in public. Some tics are violent and cause injury to the patient.

Etiology

The discovery of genes that may be responsible for the syndrome was a big step in trying to elucidate the cause of the Tourette syndrome. The mutation in the SLITRK1 gene on chromosome 13q is expressed in the regions in the brain that are implicated in the disease; these include the hippocampus, cortex, thalamic, subthalamic and globus pallidus nuclei). The gene appears also to be important in dendritic growth.

Other genes have been discovered in families with Tourettes; one of note is the HDC gene located in chromosome 15. There also appears to be non-genetic factors, because there have been differences in monozygotic twins. Proposed postulates include a difference in the immune response to streptococcus infection. There is on-going research to the possible connection with streptococcus infections [2] [3].

Epidemiology

The overall international data shows are prevalence of about 1%, but these figures are thought to be unreliable due to different propensities for people to seek medical care in different communities and cultures. The typical age of onset is five to ten years of age.

There is a high incidence of attention deficit hyperactivity disorder (ADHD) (60 percent), and obsessive-compulsive disorder (OCD), in this population [4] [5] [6].

Pathophysiology

The neurophysiology of this syndrome is not yet known. It is thought to be caused by pathologies in the inferior frontal cortex and the basal ganglia. There appears to be loss of normal asymmetry in these regions on imaging. Biochemical studies from autopsies have shown decreased levels of dopamine but its role in the pathophysiology is not known. There have been signs from of increased dopamine receptors and production. It is thought that problems in the synthesis and uptake of dopamine could be the cause [7] [8].

Prevention

Genetic counselling is the only know prevention, due to its strong genetic component. Parents with suffering from tics have to know the risks of their offspring having the same condition as them and the associated comorbidities, such as obsessive-compulsive disorder.

Summary

Tourette syndrome (TS) is a neurological disorder characterized by tics. Tics are repeated, individually recognizable, intermittent movements or movement fragments that are almost always briefly suppressible and are usually associated with awareness of an urge to perform the movement. The tic can be brief movements of utterances such as sounds or words. Of note these movement disappear with sleep. The tic are suppressible, but the longer they are suppressed the stronger the urge [1].

Patient Information

  • Definition: Tourette syndrome is a disorder that causes unusual movements or sounds, called “tics.” Examples include blinking and shrugging. People with the disorder have little or no control over their tics. The symptoms may be mild but some can be sever and distressful. 
  • Cause: The cause is thought to be genetic. The abnormal genes are passed down from the parents. It is though that these gene changes cause chemical changes within the bairn.
  • Symptoms: Motor tics are tics that involve unusual movements. They can be mild (called “simple motor tics”) or more extreme (called “complex motor tics”). Examples of motor tics include: Blinking, briefly grimacing, and shrugging the shoulders. Vocal tics include throat-clearing, or other noises, or swearing and repeating words or phrases (including echoing what other people say).
  • Diagnosis: The diagnosis is made by history and examination. The doctor will make the diagnosis from this and there is rarely any need to do more tests.
  • Treatment: These include medicines that can reduce the tics, and habit reversal training which help the patient recognise when the tic is about to happen, so they can prevent it. Other medicines such as botulinum toxin, have been used to paralyse muscles that cause the movements.

References

  1. Kurlan R. Clinical practice. Tourette's Syndrome. N Engl J Med 2010; 363:2332.
  2. Devor EJ. Untying the gordian knot: the genetics of Tourette syndrome. J Nerv Ment Dis. Nov 1990;178(11):669-79
  3. Pauls DL. Issues in genetic linkage studies of Tourette syndrome. Phenotypic spectrum and genetic model parameters. Adv Neurol. 1992;58:151-7
  4. Prevalence of diagnosed Tourette syndrome in persons aged 6-17 years - United States, 2007. MMWR Morb Mortal Wkly Rep. Jun 5 2009;58(21):581-5.
  5. Robertson MM. The prevalence and epidemiology of Gilles de la Tourette syndrome. Part 1: the epidemiological and prevalence studies. J Psychosom Res. Nov 2008;65(5):461-72
  6. Hebebrand J, Klug B, Fimmers R, et al. Rates for tic disorders and obsessive compulsive symptomatology in families of children and adolescents with Gilles de la Tourette syndrome. J Psychiatr Res. Sep-Oct 1997;31(5):519-30
  7. Miller AM, Bansal R, Hao X, Sanchez-Pena JP, Sobel LJ, Liu J. Enlargement of thalamic nuclei in Tourette syndrome. Arch Gen Psychiatry. Sep 2010;67(9):955-64
  8. Abelson JF, Kwan KY, O'Roak BJ, et al. Sequence variants in SLITRK1 are associated with Tourette's syndrome. Science 2005; 310:317.
  9. American Psychiatric Association. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th ed, Text Revision. Washington, DC: American Psychiatric Association; 2000.
  10. Leckman JF, Zhang H, Vitale A, et al. Course of tic severity in Tourette syndrome: the first two decades. Pediatrics 1998; 102:14.
  11. Bockner S. Gilles de la Tourette's disease. J Ment Sci. Oct 1959;105:1078-81
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