TOURETTE SYNDROME & OTHER TIC DISORDERS

JINU JANET VARGHESE

PARASSERIL MARGRACE DAVID

GROUP: 4

YEAR: 5

TBILISI STATE MEDICAL UNIVERSITY

INTRODUCTION

Tourette syndrome is a genetic, neuropsychiatric disorder. It is named

for Georges Gilles de la Tourette, who first described this disorder in

1885. Begins in childhood and is characterized by multiple physical

(motor) tics and at least one vocal tic. A tic is a rapid, repeated, non-

rhythmic movement or sound production that occurs suddenly, serves

no purpose, and is experienced as meaningless.

MOTOR TICS

It is classified into simple and complex motor tics.

Simple motor tics affects only a small number of muscle groups and

involve only short lasting, circumscribed movements. Ocular tics are

particularly common.

Complex motor tics are defined as those that involve multiple muscle

groups and/or seem to fulfill a purpose. Copropraxia, echopraxia, and

palipraxia are special types of complex motor tics.

Simple Motor Tics Complex Motor Tics

• Winking, blinking, eye-rolling, wide eye-opening (without moving the eyebrows)

• Eyebrow-raising • Wrinkling or turning up the nose• Puffing up the cheeks• Mouth opening, pulling the corners of the

mouth• Lip movements• Sticking out the tongue• Jaw movements• Frowning• Grimacing• Tooth-chattering• Head-shaking, throwing, turning, twitching, or

twitching, or nodding• Shoulder-shrugging• Arm and hand movements• Abdominal movements• Trunk movements

• Seemingly intentional movements, facial expressions, gesticulations with the head, hands, arms, trunk, legs, feet

• Picking at clothes• Hopping, jumping• Clapping, finger-tapping• Spinning• Bending and bowing the trunk• Wide arm movements• Foot-stamping• Dystonic tics (rare) with slow turning

movements• Writing tics• Echopraxia: non-purposeful imitation of other

other p ersons’ observed movements• Copropraxia: making obscene gestures such

such as showing the middle finger, indecent indecent movements of the trunk and pelvis, pelvis, crotch-holding

• Palipraxia (rare): repetition of one’s own

VOCAL TICS Especially in children, tics are often misdiagnosed as an airway disease

such as asthma or an allergy. Throat-clearing and sniffling are the most

common kinds of vocal tic; exclamations and shouts are much rarer.

Coprolalia (19-32%), echolalia, and palilalia are complex vocal tics.

Coprolalia is more common in more severe cases of Tourette syndrome

with multiple comorbidities.

Simple Vocal Tics Complex Vocal tics

• Throat-clearing

• Sniffling, snorting

• Coughing, rasping

• Snuffling

• Blowing out the lips/tongue

• Inhaling or exhaling noisily

• Squeaking, squealing, grunting

• Whistling, humming

• Shouting

• Saying syllables (hm, eh, ah, ha)

• Making animal noises or other

sounds– spitting

• Echolalia: repetition of heard sentences, words,

words, syllables,

or sounds, not for the purpose of

communication;

can also lead to the generation of new tics

• Coprolalia: saying obscene words

• Palilalia: involuntary repetition of one’s own

spoken words

• Blocking of speech, stuttering

• Saying fragments of speech

• Saying other socially inappropriate words

(NOSI = “non-obscene, socially inappropriate

inappropriate behavior,” e.G., “Fat, fat, fat,”

fat,” “help, help,” “yes, yes, yes”)

ICD-10 DIAGNOSTIC CRITERIA FOR TIC DISORDERS Tourette syndrome -The ICD-10 diagnostic criteria further require the

onset of illness in childhood or adolescence, duration of at least one

year (though there may be symptom-free intervals lasting several

months), and fluctuation of the the tics over time. No particular degree

of severity is required.

Chronic motor tic disorder – absence of vocal tics. The motor tics are

usually mild, and the comorbidities are rarer and generally less severe.

Chronic vocal tic disorder – It is rare & is characterized by the persistent

exclusive vocal tics. Comorbidities are just as frequent as in Tourette

syndrome.

Transient tic disorder – Transient tic disorder is seen only in children and

is characterized by tics that disappear within one year. These are

usually mild, simple motor tics that the children themselves may not

even notice.

EPIDEMIOLOGY

The tics of Tourette syndrome begins in childhood.

10–15% of children in elementary school have transient simple motor tics.

Tics usually appear with gradually increasing intensity between the ages of 6 and 8 years & are at their most severe, on average, between the ages of 10 and 12.

Is found among all social, racial and ethnic groups and has been reported in all parts of the world.

Three to four times more frequent among males than among females.

Up to 1% of the overall population experiences tic disorders, including chronic tics and transient tics of childhood. Chronic tics affect 5% of children, and transient tics affect up to 20%.

DIAGNOSIS

According to DSM-IV-TR, Tourette’s may be diagnosed when a person

exhibits both multiple motor and one or more vocal tics over the period

of a year, with no more than three consecutive tic-free months.

Tic disorders are clinically diagnosed on the basis of a detailed history

and a neurological and psychiatric examination.

There are no specific medical or screening tests that can be used in

diagnosing Tourette's. it is frequently misdiagnosed or underdiagnosed,

partly because of the wide expression of severity, ranging from mild or

moderate, to severe.

Coughing, eye blinking, and tics that mimic unrelated conditions such

as asthma are commonly misdiagnosed.

DIFFERENTIAL DIAGNOSIS Dissociative movement disorders

Compulsive behaviors

Generalized hyperactivity

Mannerisms

Stereotypies

(Less commonly) dystonia and myoclonus

In rare cases, tics can arise as a manifestation of another disease (e.g.,

Wilson’s disease, neuro- acanthocytosis, Fragile X syndrome,

Sydenham’s chorea, Huntington’s disease); they can also be

iatrogenic or substance-induced (e.g., by carbamazepine, phenytoin,

lamotrigine, amphetamines, dopaminergic drugs, or cocaine). Tardive

tics are a rare complication of neuroleptic use.

CORMORBIDITIES 80–90% of patients with tourette syndrome have not only tics, but also psychiatric

manifestations.

Common comorbidities in childhood that often impair psychosocial functioning are the following:

1. Obsessive-compulsive behavior and anxiety

2. Impulse-control disorders

3. Emotional dysregulation

4. Disorders of social behavior

5. Autism spectrum disorders (ASD)

6. Disorders of individual skills

Children are the most likely (50–90%) to suffer additionally from attention deficit–

hyperactivity disorder (ADHD)

Adults often have obsessive-compulsive symptoms or behavior as well as auto-aggression, depression, and sleep disorders

ETIOLOGY

The exact cause of Tourette’s is unknown.

Structural and functional abnormalities in the motor and somatosensory

portions of the corticostriatal-thalamocortical circuit:

Exact mechanism is unknown but it is believed to result from dysfunction

in cortical and subcortical regions, the thalamus, basal

ganglia and frontal cortex.

Dopamine dysfunction is considered a prime abnormality in TS based on

tic suppression with the use of dopamine antagonists

(antipsychotics). An abnormality of presynaptic regulation exists in

combination with phasic dysfunction of dopaminergic transmission.

Genetic and environmental factors:

It is currently estimated that the first degree relatives of a patient with

Tourette syndrome have a 5% to 15% risk of developing the disease

themselves and a 10% to 20% risk of developing any sort of tic.

Prenatal Perinatal Postnatal

• smoking during pregnancy

• psychosocial stress during pregnancy

• intrauterine growth retardation, low birth birth weight

• premature birth• perinatal hypoxia

• infection, especially Group A β-hemolytic streptococci streptococci (GABHS) with PANDAS (“pediatric autoimmune neuro- psychiatric disorders associated with associated with streptococcal infections”

• psychosocial stress (often worsens tics)

Non- genetic risk factors:

TREATMENT

Thorough patient education.

Psycho-education should always be the first step of treatment.

Tics cannot be cured. . Tics should be treated symptomatically when

they produce marked physical or psycho- social impairment.

Choices of treatment include Pharmacotherapy, Behavior therapy,

Deep brain stimulation and treating the comorbidities associated with

Tourette syndrome.

PHARMACOTHERAPY

Dopamine-receptor antagonists (neuroleptic drugs). Haloperidol is the only

medication that is approved in germany for the treatment of tourette syndrome.

Agents of first choice used in children:

a. tiapride,

b. risperidone,

c. ripiprazole

For adults:

a. tiapride,

b. sulpiride,

c. risperidone (recommendation level A for each)

d. aripiprazole(recommendation level B)

Alternatives: other atypical neuroleptic drugs, pimozide, combinations with

tetrabenazine, topiramate, and local botulinum-toxin injections and cannabinoids.

Clonidine is also used, even though its tic-suppressing effect is rather weak.

BEHAVIOR THERAPY

Habit reversal training (HRT): This method lessens the frequency of tics by

about 30%.

Exposure and response prevention (ERP): Interrupting the automatism

described by many patients in which a premonitory urge is necessarily

followed by a tic.

It is recommended that behavior therapy should always be tried before drug

treatment.

DEEP BRAIN SIMULATION

It is for adult patients with severe, medically intractable Tourette

syndrome.

Deep brain stimulation not only lessens the frequency of tics but also

improves comorbid psychiatric disorders, including obsessive-

compulsive symptoms, depression, anxiety, and auto-aggression.

The most common stimulation-induced side effects are fatigue, lack of

energy, visual disorders, and dizziness and occasionally by infection

and rarely by intra-cerebral hemorrhage.

TREATMENT OF CORMORBIDITIES

Alongside drug therapy, psycho-therapeutical interventions are an

important component of the multimodal treatment concept,

particularly for children.

Selective serotonin reuptake inhibitors (SSRI) are the mainstay of drug

treatment for obsessive-compulsive symptoms, anxiety, and

depression;

Fluoxetine is the only one approved for use in children aged 8 or older.

Methylphenidate is the drug of first choice for treatment of ADHD in

patients with comorbid tics.

CONCLUSION

The general knowledge of tic disorders is deficient.

Many patients with chronic tic disorders and Tourette syndrome have

such mild tics that they need no treatment.

To prevent stigmatization, psycho-education of the patient, his or her

family, and other persons who interact with the patient in everyday life

is essential.

The available scientific evidence on the pharmacotherapy of Tourette

syndrome is still of poor quality.