ICD-118A05.0	PRIMARY TICS OR TIC DISORDERS Primary tics or tic disorders
ICD-10F95.2	Combined vocal and multiple motor tic disorder [de la Tourette]
DSM-5-TRF95.2	Tourette's Disorder (and other tic disorders F95.0–F95.1)

1. Definition and nosology

Tic disorders (ICD-11: 8A05.0 Primary Tics or Tic Disorders; DSM-5-TR: F95.0–F95.2) — a group of neurodevelopmental disorders characterized by sudden, rapid, recurrent, nonrhythmic, involuntary or semi-voluntary motor movements and/or vocal/sound productions. Tics are typically accompanied by a premonitory urge (an urge or tension) and can be voluntarily suppressed for a short period.

Three main categories in ICD-11 and DSM-5-TR:

• Tourette syndrome (DSM-5-TR F95.2) — multiple motor tics and one or several vocal tics lasting ≥1 year, onset before age 18
• Persistent (chronic) motor or vocal tic disorder (DSM-5-TR F95.1) — motor or vocal (but not both) ≥ 1 year;
• Transient tic disorder (DSM-5-TR F95.0) — tic (motor and/or vocal) < 1 year, onset up to 18 years.

2. History

• Itard J.M.G. (1825) — the first clinical description of motor and vocal tics in Marquise de Dampierre.
• Gilles de la Tourette G. (1885) — Classical description of 9 patients at Salpêtrière hospital under Charcot's direction; name of the syndrome.
• 1960s — psychoanalytic explanation was dominant (‘neurotic disorder’).
• 1961 (Seignot J.N.) — the first clinical use of haloperidol revealed its effect in tics — basis for transition to neurobiological concept.
• DSM-III (1980) and subsequent revisions — formal status of tics as a medical disorder.
• AAN 2019 (Pringsheim T. et al. Neurology 2019;92(19)) — international evidence-based clinical guideline; emphasized that α2-agonists are first-line compared to antipsychotics due to more favorable side effect profile.
• ESSTS — European Society for the Study of Tourette Syndrome — European Clinical Guidelines (2011, updated 2022).

3. Epidemiology

• Tourette syndrome prevalence: in children 0.5–1% (Knight T. et al. systematic review Pediatr Neurol 2012).
• Persistent motor or vocal tic: ~1–2%.
• Transient tics: Up to 20% of school-age children.
• Sex: Higher by 3–4 times in males.
• Age of onset: 5–7 years; peak severity at 10–12 years.
• Persistence: In adulthood, tics significantly decrease in ~50%, persist in ~30%, and severe form remains in ~20% (Bloch M.H., Leckman J.F. J Psychosom Res 2009).
• Comorbidity: ADHD 50–60%, OCD 30–50%, anxiety 30%, depression 20%, learning disorder 20–30%, angry outbursts, sleep disorders.

4. Aetiology and pathogenesis

4.1 Genetic factors

• Heritability: 0.77 (Mataix-Cols D. et al. JAMA Psychiatry 2015 family and twin studies, n>200 000 Sweden) — among the most heritable psychiatric disorders.
• Polygenic nature: GWAS (Yu D. et al. Am J Psychiatry 2019) — risk loci identified; no single gene.
• Rare mutations: SLITRK1, HDC (histidine decarboxylase), CELSR3 — in a small number of families.
• High density of OCD and ADHD in family history — genetic overlap.

4.2 Neurobiological mechanisms

• Cortico-striato-thalamo-cortical (CSTC) circuit dysfunction — disruption of motor inhibition in basal ganglia and related regions (Leckman J.F. et al. Neuron 2000;28(2):349–354).
• Dopaminergic system hyperactivity — mechanism of tic reduction by dopamine antagonists (haloperidol, risperidone).
• Histaminergic system — HDC mutations rare; histaminergic tone low.
• Structural changes: Reduction in caudate volume, sensorimotor cortical thinning (Sowell E.R. et al. Nat Neurosci 2008).

4.3 Environmental factors (modifier)

• Preterm birth, perinatal hypoxia — moderately increases risk.
• Maternal tobacco use during pregnancy.
• Psychosocial stress — is not a cause for the onset of tics, but influences severity.
• PANDAS / PANS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections / Pediatric Acute-onset Neuropsychiatric Syndrome) — rare; most cases are actually typical Tourette/OCD (see expanded §10).

5. Clinical features

5.1 Motor tics

• Simple motor tics: Eye blinking (most frequent onset), nose sniffing, head shaking, shoulder shrugging, lip play.
• Complex motor tics: Sequential movements — hopping, touching, spinning on the spot; rarely an obscene gesture (copropraxia, ~5%).

5.2 Vocal/phonic tics

• Simple vocal tic: coughing, sniffling, wheezing, throat clearing, shouting.
• Complex vocal: word, echolalia (repetition of others' words), palilalia (repetition of own words).
• Coprolalia (obscene word/phrase) — occurs in 19.3% of males and 14.6% of females with Tourette syndrome (Freeman R.D. et al. Dev Med Child Neurol 2009 international Tourette database, n=597), and differs from the portrayal in mass media.

5.3 Premonitory urge and voluntary suppression

Most patients experience a premonitory urge before tics (somatic urge, tension) is noted; the tic temporarily relieves this sensation. Tics can be voluntarily suppressed for a short period (seconds to minutes), but this suppression creates internal tension and discomfort — the subsequent tic episode is usually more intense.

5.4 Course and variability

• tics “Waxing and waning” — severity waxes and wanes; one tic type replaced by another.
• Triggers — stress (positive or negative), excitement, fatigue, specific contexts (e.g., suppressing tics at school).
• Tics decrease during sleep, but do not completely disappear.
• Peak severity at ages 10–12; decreases in most patients during adolescence.

6. Diagnosis

6.1 Unified diagnostic criteria (DSM-5-TR · ICD-11 · AAN 2019 consensus points)

For Tourette syndrome:

1. Multiple motor tics and one or several vocal tics (occurring at different times, not required to be present simultaneously)
2. Tics persist for at least 1 year (they may vary over time).
3. Onset Up to 18 years.
4. Exceptions — not fully explained by substance (e.g., cocaine) or medical condition (Huntington's, postviral encephalitis).

Persistent motor or vocal tic disorder: Same criteria as Tourette, but single type tic (motor or vocal, not both).

Transient tic disorder: Same criteria as Tourette, but duration < 1 il.

6.2 Source-specific clarifications

• DSM-5-TR: Three separate categories (F95.0, F95.1, F95.2). “Functional impairment” and “severity” were not included in the diagnostic requirement — merely meeting the criteria is diagnostically sufficient; however, the intervention decision is based on functional impairment.
• ICD-11 (8A05.0): “Primary tics or tic disorders” — sub-specifications: Tourette, persistent, provisional.
• AAN 2019 (Pringsheim T. et al.): the diagnosis is clinical, video recordings are an adjunct; YGTSS (Yale Global Tic Severity Scale) for baseline measurement and follow-up; comorbidity (ADHD, OCD) must be assessed.
• ESSTS 2022 (Müller-Vahl K.R. et al.): In the European context — same criteria; accepts CBIT as first-line non-pharmacological intervention.

6.3 Diagnostic algorithm

1. Parent and patient interview — onset age, course, triggers, premonitory feeling.
2. Video recordings — episodes captured at home by family (in clinic context tics often diminish)
3. Standardized scale— YGTSS (Yale Global Tic Severity Scale, baseline measures and monitoring).
4. Comorbidity screening — ADHD (Conners, Vanderbilt), OCD (CY-BOCS), anxiety, depression, learning disorder, sleep.
5. Neurological examination — focal signs, exclusion of choreoathetosis.
6. Specific clinical suspicion — Huntington's disease (HTT gene), Wilson's disease (ceruloplasmin, copper), neuroacanthocytosis.
7. PANDAS/PANS suspicion — only if classic clinical picture (rapid onset, OCD + tics + emotional lability post-streptococcal); routine antistreptolysin O tests are not recommended (NIMH 2017).

6.4 Differential diagnosis

7. Examination and assessment

7.1 Clinical scales

• YGTSS (Yale Global Tic Severity Scale, Leckman J.F. et al. 1989) — separate for motor and vocal tics; frequency, intensity, complexity, interference, number on a 0–5 scale; global impairment 0–50. Baseline measures and treatment monitoring.
• PUTS (Premonitory Urge for Tics Scale).
• CY-BOCS — comorbid condition assessment.
• Conners-3, Vanderbilt — comorbid ADHD.

7.2 Laboratory investigations

No specific laboratory indication for routine tics. Based on clinical suspicion:

• Thyroid function (TSH) — in hyperthyroidism context.
• Ceruloplasmin, 24-hour urine copper — suspicion of Wilson's disease (especially hepatic or movement adjunct).
• HTT — suspected Huntington (family history).
• Streptococcal antibodies (ASO, anti-DNase B) - only if classic PANDAS clinical picture present; not recommended routinely.

7.3 Instrumental investigations

• EEG — only if seizure suspected.
• Brain MRI — focal neurological signs, atypical clinical picture.
• Routine neuroimaging for tics is not recommended.

8. Treatment

8.1 General principles (AAN 2019 · ESSTS 2022 consensus)

1. Intervention decision based on functional impairment — mild tics may be observed; aggressive intervention not required.
2. Psychoeducation for family and school — the first step — nature of tics, ‘waxing and waning’, premonitory urge.
3. Behavioral intervention — CBIT (Comprehensive Behavioral Intervention for Tics) AAN 2019 first-line recommendation (age 8+). HRT (Habit Reversal Training) + functional intervention + relaxation.
4. Pharmacotherapy — when functional impairment is present and CBIT is unavailable or insufficiently effective:
   • First-line: α2 adrenergic agonists — clonidine, guanfacine (ER) — side effect profile superior to antipsychotics; particularly beneficial in comorbid ADHD.
   • Second-line: Atypical antipsychotic — aripiprazole (FDA-approved for Tourette syndrome, ages 6–18, 2014), risperidone, ziprasidone. Stronger anti-tic effect, but metabolic and EPS side effects.
   • Third-line: typical antipsychotic — haloperidol, pimozide (FDA approval for Tourette, but EPS and QTc prolongation risk).
   • In refractory cases — VMAT2 inhibitors (tetrabenazine off-label; deutetrabenazine and valbenazine under investigation for tics), botulinum toxin for focal tics.
5. Comorbidity treatment — ADHD (stimulants do not significantly worsen tics – meta-analysis: Bloch M.H. et al. JAACAP 2009; but patients should be monitored), OCD (SSRI + CBT), anxiety, sleep.
6. Refractory severe in Tourette's — Deep Brain Stimulation (DBS) — thalamic centromedian-parafascicular or GPi (globus pallidus internus) targets; multidisciplinary selection in certified center (Schrock L.E. et al. Mov Disord 2015).

8.2 Pharmacotherapy — First-Line (α2-Agonists)

8.3 Second-Line (Atypical Antipsychotic)

8.4 Third-Line (Typical Antipsychotic)

• Haloperidol 0.5–4 mg/day — FDA approved for Tourette, but side effects: EPS, tardive dyskinesia, sedation.
• Pimozide 1–4 mg/day — FDA approval for Tourette; QTc prolongation, EKG mandatory.

8.5 Approach in Comorbid Conditions

• ADHD + tic: α2 agonist + stimulant or atomoxetine. Stimulants had a controversial history as a ‘tic enhancer’, but Bloch 2009 meta-analysis and the TACT (Tourette's Syndrome Study Group. Neurology 2002;58(4):527–536) — moderate-dose methylphenidate does not significantly exacerbate tics; monitor.
• OCD + tic: SSRI + ERP; adjunctive aripiprazole or risperidone.
• Sleep disorder: melatonin; clonidine also assists in the sleep component.

8.6 Source-Specific Clarifications

• AAN 2019 (Pringsheim Neurology 2019): CBIT first-line non-pharmacological; α2-agonists before antipsychotics (favorable side effect profile); dual benefit in comorbid ADHD.
• ESSTS 2022 (Müller-Vahl): In the European context; choice of CBIT and pharmacotherapy is individualized based on patient characteristics.
• Piacentini J. et al. JAMA 2010 (CBIT pivotal RCT): n=126, ages 9–17 — CBIT significantly superior to placebo (supportive therapy); reduction of 7.6 vs 3.5 points on YGTSS; effect size g = 0.68.
• Tourette Association of America Behavioral Sciences Consortium — Implementation of CBIT program and therapist training.

Treatment methods

1. Comprehensive Behavioral Intervention for Tics (CBIT) — Woods D.W., Piacentini J. — First-line non-pharmacological intervention (AAN 2019). Three core components: (1) Habit Reversal Training (HRT — recognition of premonitory sensation, learning competing behavior); (2) functional intervention (modifying contexts that reinforce tics); (3) relaxation training. Standard course — 8 individual sessions, 10 weeks; ongoing booster sessions. Evidence: Piacentini J. et al. JAMA 2010 RCT — pivotal study; Wilhelm S. et al. Arch Gen Psychiatry 2012 RCT in adults; effect size moderate. tourette.org.
2. Habit Reversal Training (HRT — Habit Reversal Training) — Azrin (Azrin N.H.), Nunn (Nunn R.G.) — Core component of CBIT — awareness training + competing response. Separate competing behavior for each tic (e.g., for neck jerking, holding neck centered).
3. Exposure and Response Prevention (ERP) for Tics — Alternative behavioral approach to HRT — patient learns to tolerate premonitory urge without responding with tic. Verdellen C.W. et al. Behav Res Ther 2004. ESSTS — alternative evidence-based behavioral method.
4. Yale Global Tic Severity Scale (YGTSS — Yale Global Tic Severity Scale) — Leckman (Leckman J.F.) et al., 1989 — Gold standard severity scale for tic disorders; separate counts for motor and vocal tics: frequency, intensity, complexity, interference level (0–5 points); global impairment 0–50. Baseline indicators and monitoring.
5. Premonitory Urge for Tics Scale (PUTS) — Woods D.W., Piacentini J — 9-item self-assessment; intensity of premonitory urge and its effect on intervention behavior.
6. Deep Brain Stimulation (DBS) — Surgical intervention for refractory severe Tourette; typical targets — thalamus (centromedian-parafascicular) and globus pallidus internus (GPi). Multidisciplinary selection; Schrock L.E. et al. Mov Disord 2015 international consensus.
7. Botulinum Toxin — Injection into target muscle in focal motor (e.g., neck, lip, eye) and vocal tics; 3–4 month effect. Marras C. et al. Neurology 2001 RCT.

9. Prognosis

Good prognostic factors

• Typical tic disorder (distinct from rapid functional tic-like behavior).
• Mild-moderate YGTSS severity.
• Active management of comorbidity (ADHD, OCD).
• Early psychoeducation and family-school support.
• CBIT availability.

Poor prognostic factors

• Severe comorbidity (severe OCD, ADHD, anxiety, depression).
• Self-injurious tics (e.g., head banging).
• Social and academic limited adaptation.
• Refractory pharmacotherapy response.

Follow-up targets

• YGTSS every 3–6 months.
• Comorbidity (ADHD, OCD, anxiety, depression) — annual.
• Monitoring side effects (in those taking antipsychotics — metabolic, EPS, prolactin, QTc).
• Social and academic support.
• In adolescence, possibility of spontaneous remission; possibility of gradual reduction of pharmacotherapy.
• Support for occupation and relationships in adult patients.

10. Myths and misconceptions

10.1 Aetiology myths

Myth 1: “Tourette syndrome is always accompanied by coprolalia (swear words)”

Why it is widespread: In mass media (film, television), the portrayal of Tourette is almost always presented with coprolalia; sensational media choice.

Clinical and biological rationale: Coprolalia is the rare complex vocal tic form; neither motor nor vocal tics are obscene in most Tourette patients.

Evidence: Freeman R.D. et al. Dev Med Child Neurol 2009 — International Tourette Syndrome Association database n=597: coprolalia occurs in 19.3%, of males and 14.6% of females.

Real clinical step: Correct information to family and school; reducing patient's social stigma.

Myth 2: “Tourette's is a result of poor upbringing or parental mistakes”

Evidence: heritability 0.77 (Mataix-Cols JAMA Psychiatry 2015) — highest among psychiatric disorders. Genetic and neurobiological basis; parental experience not etiology.

Myth 3: “Tourette's is caused by emotional trauma or stress”

Clinical logic: Stress is not a cause of tic onset — tics are genetic and neurodevelopmental in origin. However, stress influences the severity of existing tics.

Myth 4: “All tics are PANDAS and must be treated with antibiotics”

Why it is widespread: In the 1990s, PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) hypothesis by Susan Swedo and colleagues, and marketing by alternative clinics.

Clinical and biological rationale: Classic PANDAS clinical picture — rapid (hours-days) onset OCD/tics + emotional lability + post-streptococcal infection — is a rare event. Most tic patients show a typical course (gradual onset, waxing and waning).

Evidence: NIMH 2017 PANDAS/PANS clinical statement: routine antistreptolysin O testing or antibiotic prophylaxis in general tic disorders is not recommended; assessed only if a classic clinical picture is present. Routine use of IVIG and plasmapheresis is not evidence-based (only within clinical trials).

10.2 Harmful or erroneous methods

Myth 5: “Scolding or punishing a child harshly when they tic will make the tics go away”

Clinical logic: Tics can be partially voluntarily suppressed, but prolonged suppression leads to an increase in internal tension and subsequent “rebound” tic outbursts. Punishment does not reduce tics; on the contrary, it increases stress-related intensity.

Evidence: AAN 2019, ESSTS 2022 — tics are not voluntary behavior; punitive approach is ineffective and harmful.

Myth 6: “Antipsychotic haloperidol should be first-line in all tic patients”

Evidence: AAN 2019 (Pringsheim) — α2-agonists (clonidine, guanfacine) Before antipsychotics. It is recommended due to its favorable side effect profile. Haloperidol and pimozide are FDA-approved, but they have third-line status because of risks for EPS, tardive dyskinesia, sedation, prolactin, and QTc prolongation.

Myth 7: Stimulants cause tic explosions — stimulants are contraindicated for a Tourette patient with ADHD

Evidence: Bloch M.H. et al. JAACAP 2009 meta-analysis (n=9 RCTs, n=477) and TACT (Treatment of ADHD in Children with Tics; Tourette's Syndrome Study Group. Neurology 2002;58(4):527–536) — moderate-dose methylphenidate does not significantly exacerbate tics; in some patients even reduce tic severity. Comorbid ADHD constitutes the major part of functional impairment — considering stimulants as contraindicated is incorrect.

Real clinical step: In comorbid ADHD, start stimulant, monitor patient for tic severity; if exacerbation observed, reduce dose or α2-agonist + stimulant combination.

10.3 Ineffective or scientifically unfounded methods

Myth 8: Cannabis (medical marijuana) is an effective treatment for Tourette syndrome

Why it is widespread: anecdotal reports on social media, cannabis industry marketing, modest effects shown in some pilot studies.

Evidence: Müller-Vahl K.R. studies (small RCTs) — THC may partially reduce tics, but evidence base is insufficient for clinical recommendation. AAN 2019 — cannabinoids are not standard first-line; risk profile (psychosis risk in adolescents, cognitive effects, dependence) is significant. May be considered only in refractory adult cases and where national legislation permits.

Myth 9: “Special diet (gluten-free, casein-free) or avoidance of food additives eliminates Tourette's”

Evidence: No specific clinical evidence; AAP, AAN do not recommend.

Myth 10: “EEG neurofeedback reduces tics”

Evidence: Evidence base is limited; AAN 2019 — not first-line; CBIT is significantly superior.

Myth 11: “Vitamin mega-doses, omega-3, magnesium resolve tics”

Evidence: Efficacy of these supplements for Tourette has not been proven; AAN 2019 and ESSTS 2022 do not recommend.

Myth 12: “Acupuncture, homeopathy, craniosacral therapy cure Tourette's”

Evidence: Cochrane and systematic reviews — the effect of these complementary interventions for tic disorders has not been proven.

Myth 13: “Hypnotherapy or psychoanalytic therapy resolves tics”

Evidence: Was dominant until the 1960s, but shows no efficacy in modern evidence base; CBIT or pharmacotherapy is superior.

11. Sources

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