2. HISTORICAL BACKGROUND
Tic was recognized as early as the fifteenth century, although, like other involuntary
movement disorders, it was deemed to be Due to possession by supernatural powers.
Stevens (1971) has drawn attention to the case of a seventh century of Louis XV who
crammed things into his mouth to try to control involuntary barking.
Dr Samuel Johnson (1709–1784) , the famous lexicographer, poet, and writer,
suffered from the disorder.
It has also been argued that Wolfgang Amadeus Mozart (1756–1791) suffered from
Gilles de la Tourette’s syndrome (Simkin 1992 ) and examples of hyperactivity,
bizarre motor acts, coprolalia, and coprographia are cited as support of this.
3. The first medical report of this disorder was in 1825 by Itard, who described the
Marquise de Dampiere Marquise was a noble woman whose symptoms included
coprolalia (the utterance of swear words). She regularly shocked her high society
friends by yelling out obscenities during conversations.
Tics, however, were not clearly delineated from choreas until Gilles de la Tourette
published his landmark paper in 1885.
In 1893 Gilles de la Tourette was shot in the neck by a paranoid woman who
believed she was under his hypnotic power.
Although some reports suggest he never recovered (Lawden 1986 ), his injuries were
minor and he continued working until dying of probable neurosyphilis in 1909.
4. DEFINITIONS
tic is a stereotyped purposeless motor act which occurs irregularly and can be temporarily
suppressed by an effort of will.
The last provision is necessary to help separate tics from myoclonus, chorea, and some other
forms of epilepsy.
Although tics are typically brief, some may be more sustained and have a more dystonic or
tonic appearance.
5. DIVISION OF TICS
Primary or idiopathic : tic is the major neurological feature and it is not caused by
other recognized diseases.
Secondary or symptomatic : tic is not the main neurological feature or, if it is, then it is
due to another recognized cause .
6. SECONDARY CAUSES OF TICS
Neurodevelopmental Disorders:(Mental retardation,Autistic spectrum disorders (including Asperger
syndrome),Rett syndrome,Genetic and chromosomal abnormalities, Developmental stuttering).
Acute Brain Lesions:(Posttraumatic,Vascular,Infectious[Varicella-zoster virus,Herpes simplex
encephalitis,Mycoplasma pneumonia,Lyme disease] ).
Postinfectious:( Sydenham chorea,Pediatric autoimmune neuropsychiatric disorder associated with
streptococcal,infections (PANDAS) )
Neurodegenerative Diseases:(Huntington disease ,Neuroacanthocytosis syndromes, Neurodegeneration
with brain iron accumulation).
Other Systemic Diseases: (Behçet syndrome, Antiphospholipid syndrome).
Peripheral Trauma.
Medications and Toxins .
Functional Ticlike Jerks.
8. CLINICAL SPECTRUM OF IDIOPATHIC TIC
simple tics
Involving a muscle
or group of muscles
complex
Gilles de la
Tourette’s syndrom
9. SIMPLE TIC
Simple tic, habit spasm, or transient tic disorder of childhood is a well-recognized and common
condition.
It is not associated with known anatomical pathology, neurotransmitter abnormality,
pathophysiology, or cause.
simple tics are more common in boys and the male: female ratio is probably about 3:1.
The vast majority of simple tics resolve, although the proportion that clear and the rate of
resolution remain uncertain.
Infrequently simple tics persist into adult life.
Sometimes single tics become multiple and do not clear, merging into the chronic multiple tic
syndrome. If vocalizations are present Gilles de la Tourette syndrome has developed .
10. Simple tics most frequently involve the face, particularly around the eyes.
Winking, unilateral blinking, raising the eyebrows, frowning, moving the scalp, flaring
the nostrils, grimacing, and wriggling the ears are common .
Sniffing, snorting, throat clearing, coughing, humming, and making clicking noises with
the tongue also occur.
Intelligible speech is uncommon.
Shoulder shrugging, head nodding, and head banging may occur.
Tics involving the arms, hands, lower limbs, or trunk are less frequent but can result in
touching, slapping, gesturing, stamping, kicking, and the like.
11. Treatment is usually not required for simple tic although botulinum toxin injections can
be effective .
If neurological examination is normal and there are no untoward factors, explanation
and reassurance should suffice.
It seems sensible to advise parents to overlook the symptoms and avoid emphasizing
them by attempts at correction, although there is no scientific evidence to support this.
12. TOURETTE SYNDROME (TS)
A primary tic disorder. Classified as transient or chronic, tic disorders represent a
heterogenous group of entities, many of unknown cause, in which tics are the core
clinical feature, causing distress or significant impairment in social or occupational
functioning.
Standard diagnostic criteria for TS require multiple motor and phonic(vocal) tics of
onset below 21 years of age with a duration of at least 1 year, during which a tic-
free period cannot last longer than 3 consecutive months.
Tic disorders lasting longer than 1 year chronic.
a primary tic disorder of less than 1 year in duration transient.
The estimated prevalence of Tourette syndrome is between 3/1000 and 8/1000
subjects between 6 and 18 years of age.
13. Patients with TS may exhibit a wide range of other tic like, complex stereotyped
behaviors that include
1- Echophenomena (echopraxia, echolalia), ie, nonvoluntary repetition of movements
or vocalizations made by another person.
2-Paliphenomena: (palilalia, palipraxia), ie, nonvoluntary repetition of the patient’s
own vocalizations (usually syllables, words, or phrases) and movements.
3- Coprophenomena (coprolalia, copropraxia) nonvoluntary utterance or performance
of obscene or socially inappropriate words, remarks, or gestures.
14. Tics typically begin in patients between 5 and 8 years of age and may progress in a
rostrocaudal fashion over time.
Tic severity peaks during puberty but decreases throughout the second decade of
life.
Tics become gradually more suppressible with age.
15. DIAGNOSTIC CRITERIA (DSM-IV) FOR GILLES DE LA
TOURETTE’S SYNDROME
1. Age of onset during childhood or adolescence; before age 18.
2. Multiple involuntary motor tics, or involuntary sensory tics that may induce voluntary tonic
or other movements.
3. One or more involuntary vocal tics. Motor and vocal tics have been present at some time
during the illness, although not necessarily concurrently.
4. Tics irresistible but can be suppressed for seconds to hours.
5. Variations in the number, frequency, type, location, or severity of tics occur over time
(‘waxing or waning’). 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 three consecutive months.
6. The disturbance is not due to the direct physiological effects of a substance (e.g. stimulants)
or a general medical condition (e.g. Huntington’s disease or post-viral encephalitis).
16. BODY PARTS AFFECTED BY TICS
(APPROXIMATE FIGURES)
Face 90–95 %
Head and neck 90 %
Arms 50 %
Trunk 40 %
Legs 40 %
17. COMORBIDITIES
Obsessive-compulsive symptoms occur in 30% to 50% of Tourette syndrome patients.
Tic-related obsessive-compulsive symptoms differ from classic obsessive compulsive
disorder in natural history, phenomenology, and response to treatment.
Attention deficit hyperactivity disorder is present in 40% to 50% of children with
Tourette syndrome and is associated with greater academic and social impairment
and a higher frequency of disruptive behaviors.
Specific subtypes of impulse control disorders occur in Tourette syndrome, including
intermittent explosive disorder, rage attacks, and self-injurious behaviors.
These disorders occur more commonly in patients who have Tourette syndrome with
comorbid attention deficit hyperactivity disorder and obsessive-compulsive disorder.
18. PHENOMENOLOGY OF THE THREE MAIN TYPES OF ABNORMAL REPETITIVE BEHAVIOR EXHIBITED BY PATIENTS WITH
TOURETTE SYNDROME: TICS, COMPULSIONS, AND IMPULSIVE BEHAVIORS.
Premonitory sensation
,the patient experiences an
uncomfortable tension in the neck
muscles
Tic
the patient perform an
abrupt head flick
movements, he perceives
the movement as difficult to
control under volition
Outcome
the uncomfortable
premonitory sensation is
temorarly relieved.
Obsessive thought
the patient experiences an intrusive
thought characterized by fear
(eg,something terrible may happen to
his parents);this is accompanied by
raised anxiety levels with autonomic
arousal.
Compulsion
the patient performs
aseries of voluntary
routine actions in a
preordained and rigid
eequence.
Outcome
,the anxity related to the
patients obsessive thught is
temporarily relieved.
Risk- taking or sensation-
seeking
,the patient perceives the potential
risk associated with a specific
behavior (eg ,banging his head
against the wall);this is accompanied
by the build up of a mental urge to
act out his behavior.
Impulsive behavior
,the patient performs risk-
related behavior, which
potentially leads to self-
harm.
Outcome
the patient paradoxically
experiences an immediate
gratification from the
performance of the risk-
related behavior.
19. TREATMENT
PHARMACOLOGIC TREATMENT
The efficacy of antipsychotic medications in the treatment of tics has been recognized
since the earliest observational cohort studies, which reported a favorable response
to D2 dopamine receptor blockers in up to 70% of cases.
Among first-generation antipsychotics, haloperidol and pimozide appear to be
similarly effective for tics (all first generation antipsychotics may cause increased
anxiety and dysphoria (also after withdrawal), hyperprolactinemia (leading to
gynecomastia, galactorrhea, irregular menses, and sexual dysfunction), hypotension,
electrocardiographic changes, and weight gain.)
Second-generation antipsychotics are now probably more commonly used than first-
generation antipsychotics in TS. A recent meta-analysis found no significant difference
in the efficacy of risperidone (daily dose range 1 mg to 6 mg), haloperidol, pimozide,
and ziprasidone, and significant superiority to placebo.
20. Comorbid obsessive compulsive symptoms and problems with impulse control use
risperidone.
Alpha-2 agonists (clonidine and guanfacine) are considered first-line medications for
the treatment of tics in the United States and Canada. Benefit from these drugs is of
greater magnitude in patients with Tourette syndrome with motor tics plus attention
deficit hyperactivity disorder than in patients with motor tics alone.
Other pharmacologic agents for the treatment of tics include tetrabenazine,
topiramate, baclofen, benzodiazepines, cannabinoid agonists, dopamine agonists, and
botulinum toxin, but their effectiveness and safety are supported only by small clinical
trials.
21. COGNITIVE-BEHAVIORAL
TREATMENT
Habit Reversal Training (HRT), a related and expanded version of the HRT package called
Comprehensive Behavioral Intervention for Tics (CBIT), and Exposure Response Prevention for
tics.
HRT consists of teaching patients to manage tics as they occur in real timeand is based on
three main components:
(1) awareness training, which develops the patient’s ability to detect tics when these occur
and when he or she experiences a premonitory urge;
(2) competing response training,which allows the patient to select and implement a
behavior designed to prevent or hinder the occurrence of Tics.
(3) social support, whereby a designed support person promotes the use of HRT strategies
outside of therapy.
Current drawbacks of cognitive-behavioral therapy in Tourette syndrome are the limited
availability of adequately trained therapists, its long duration, the increased cost, and the lack
of clear selection criteria regarding the candidates for therapy most likely to benefit.
22. DEEP BRAIN STIMULATION SURGERY
Deep brain stimulation should be restricted exclusively to adult patients with drug-
refractory Tourette syndrome who remain significantly disabled by the severity of
their tics or comorbid psychopathology.
23. CONCLUSIONS
TS is the most disabling chronic tic disorder, with an estimated prevalence close to 1%
in the age range between 6 and 18 years.
TS is likely to be a complex polygenic disorder, with contribution of environmental
factors and genetic susceptibility factors shared with other neurodevelopmental
disorders, such as autistic spectrum disorders.
Recent advances in the understanding of the natural history and phenomenology of
chronic tic disorders have highlighted the relevance of sensory manifestations such as
premonitory urges and somatic hypersensitivity, the complex comorbidity profile
including obsessive-compulsive symptoms, attention deficit hyperactivity disorder, and
impulsive behaviors, as well as the importance of contextual factors such as
psychosocial stress in modulating the course of the illness.
