Tourette syndrome
Tourette syndrome is a childhood-onset tic disorder defined by multiple motor tics plus at least 1 vocal tic present for more than 1 year, with onset before age 18 and near-constant psychiatric comorbidity such as obsessive-compulsive disorder and ADHD. Diagnosis is clinical; first-line management is psychoeducation and habit reversal training, escalating to an alpha-2 agonist or a VMAT2 inhibitor such as deutetrabenazine for disabling tics.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
What this article covers
- Overview
Definition: Tourette syndrome is a childhood-onset tic disorder defined by multiple motor tics plus 1 or more vocal (phonic) tics, present for more than 1 year, with onset before age 18 and not…
- Epidemiology
Sex distribution: Tourette syndrome is more common in males and shows a genetic predisposition, with family history of tics or Tourette syndrome raising risk.
- Risk factors
Family history: a genetic risk for tics or Tourette syndrome in relatives is the strongest recognized risk factor.
- Pathophysiology
Basal ganglia circuit dysfunction: tics are grouped with chorea, dystonia, myoclonus, and akathisia as hyperkinetic movement disorders arising from basal ganglia and corticostriatal circuit…
- Clinical features
Diagnostic core: the defining features are multiple motor tics (for example excessive blinking, shoulder shrugging, grimacing, head shaking) plus 1 or more vocal tics (grunting, throat clearing…
- Differential diagnosis
Provisional or persistent (chronic) tic disorder: provisional tic disorder has motor and/or vocal tics present for less than 1 year; persistent (chronic) tic disorder has either motor or vocal tics…
- Diagnosis
The diagnosis is clinical: Tourette syndrome is confirmed by history and direct observation (or video) of multiple motor tics plus 1 or more vocal tics, present for more than 1 year with onset before…
- Management
First action for every patient: psychoeducation and habit reversal training.
- Complications
Social and functional impairment: embarrassment about involuntary movements or vocalizations, including coprolalia when present, commonly leads to social withdrawal and academic or occupational…
- Prognosis
Tics wax and wane: tic type, frequency, and intensity fluctuate from one period to the next, which is expected and not itself a sign of treatment failure.
- Prevention
No primary prevention: Tourette syndrome has a genetic predisposition and no modifiable cause to target for primary prevention.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Obsessive-compulsive disorder
Obsessive-compulsive disorder (OCD) is defined by ego-dystonic obsessions and/or compulsions that are time-consuming or cause significant distress and functional impairment, distinguishing it from the ego-syntonic perfectionism of obsessive-compulsive personality disorder. Diagnosis is clinical. First-line treatment combines cognitive behavioral therapy using exposure and response prevention with a high-dose selective serotonin reuptake inhibitor, with clomipramine as an effective second-line tricyclic and an OCD trial that can take up to 10 weeks to judge full response.
- Huntington disease
Huntington disease is an autosomal dominant CAG trinucleotide repeat disorder of the huntingtin gene causing striatal and cortical neurodegeneration, presenting in mid-adulthood with a triad of chorea, cognitive decline, and psychiatric disturbance. Diagnosis is clinical plus confirmatory genetic testing; management is symptomatic, using VMAT2 inhibitors for chorea and psychiatric drug therapy, since no disease-modifying treatment exists.
- Acute rheumatic fever
Acute rheumatic fever is a nonsuppurative, immune-mediated multisystem illness that follows group A streptococcal (GAS) pharyngitis, driven by antibodies against the streptococcal M protein that cross-react with cardiac, joint, skin, and CNS tissue. It is diagnosed using the JONES major criteria with evidence of antecedent GAS infection, and treated with antibiotics to eradicate the pharyngitis plus salicylates or corticosteroids for inflammation, followed by secondary penicillin prophylaxis to prevent rheumatic heart disease.
- Functional neurologic symptom disorder
Functional neurologic symptom disorder (conversion disorder) causes real, involuntary weakness, sensory loss, movement abnormality, or non-epileptic attacks that are incompatible with recognized neurologic disease on careful examination. Diagnosis rests on positive bedside signs of incongruity (Hoover sign, tremor entrainment) rather than exclusion alone; first-line treatment is diagnosis explanation plus physiotherapy or cognitive behavioral therapy, not antiseizure or other disease-specific drugs.
- Antipsychotics
Antipsychotics block the dopamine D2 receptor, with second-generation agents adding serotonin receptor blockade, and are first-line treatment for schizophrenia and other psychotic and manic states; a second-generation agent such as risperidone 2 to 8 mg/day or olanzapine 10 to 20 mg/day is chosen first, clozapine is reserved for treatment-resistant disease after two adequate trials fail, and treatment requires baseline and ongoing metabolic, cardiac, and, for clozapine, hematologic monitoring.