TICS DR MOHAMMAD A.S. KAMIL CONSULTANT NEUROLOGIST

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.

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.

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.

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 .

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.

TIC-INDUCING OR TIC-EXACERBATING AGENTS

Amphetamines

Cocaine

Heroin

Methylphenidate

Pemoline

Antipsychotics (D2 blockers):(Fluphenazine,Perphenazine,Thiothixene)

Antidepressants

Antiepileptic's: (Carbamazepine,Phenytoin,Phenobarbital,Lamotrigine)

Levodopa

CLINICAL SPECTRUM OF IDIOPATHIC TIC

simple tics Involving a muscle

or group of muscles complex Gilles de la

Tourette’s syndrom

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 .

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.

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.

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.

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.

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.

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).

BODY PARTS AFFECTED BY TICS (APPROXIMATE FIGURES)

Face 90–95 %

Head and neck 90 %

Arms 50 %

Trunk 40 %

Legs 40 %

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.

PHENOMENOLOGY OF THE THREE MAIN TYPES OF ABNORMAL REPETITIVE BEH AVIOR 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.

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.

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.

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.

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.

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.

THANK YOU DR MOHAMMAD A.S. KAMIL CONSULTANT NEUROLOGIST 29-7-2015