Tics

Tics: Excerpt from The 5-Minute Pediatric Consult

Mohammad M. Qasaymeh, MDJonathan W. Mink, MD, PhD

Tics - BASICS

Tics - description

A tic is a sudden, repetitive, stereotyped, involuntary motor (e.g., blinking, grimacing) or vocal (e.g., throat clearing, grunting, barking) movement. Tics can also be classified into simple (e.g., nose twitching, grunting) or complex (e.g., head shaking, trunk flexion, echolalia, neologism). Tics are stereotypical (repeat same way each time) and also variable, as new tics surface. Often preceded by a premonitory sensation. Patients may be able to suppress tics (partial voluntary control).

• Tourette syndrome (TS): Motor and vocal tics intermittent over ≥1 year
• Chronic multiple motor or vocal tic disorder: Motor or vocal tics, but not both, ≥1 year
• Transient tic disorder: Motor and/or vocal tics that have been present ≥2 weeks, ≤1 year
• Tic disorder not otherwise specified (NOS): Motor and/or vocal tics that do not fit a specific tic disorder
• Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS): Debatable entity described in 1998. In theory, group A β hemolytic Streptococcal (GABHS) infection evokes antibodies that cross-react with the basal ganglia causing tics in some individuals.

Tics - general prevention

Tics cannot be prevented, but educating the child and the family helps minimize tics’ impact. Identification of comorbidities is critical in preventing complications.

Tics - epidemiology

Described in almost all ethnic groups. Affects males > females. Typical onset: Age 5–7 years

Tics - prevalence

The prevalence of chronic tics and TS in school-age children is 3–6% and 0.1–1%, respectively. Transient tics occur in 20–25% of children.

Tics - risk factors

Infectious processes may trigger tic disorders.

Tics - genetics

No single gene has been associated with tics or TS. However, family history often positive for tics; the prevalence of TS in 1st-degree relatives is 10 times that in the general population.

Tics - pathophysiology

The pathophysiology underlying tics and TS is not completely understood. Abnormal dopamine neurotransmission in basal ganglia is probably involved. Evidence also implicates serotonin, norepinephrine, and acetylcholine.

Tics - etiology

Theory: Environmental or hormonal trigger in genetically susceptible individuals

Tics - associated conditions

• ~50% of children with chronic motor tics or TS meet diagnostic criteria for attention deficient hyperactivity disorder (ADHD), and ~50% have obsessive–compulsive disorder (OCD) or obsessive-compulsive behaviors.
• Conduct disorder, anxiety, oppositional defiant disorder, learning disabilities (LD), and rage episodes associated with TS.

Tics - DIAGNOSIS

The diagnosis of tics is clinical. Physical exam and laboratory and imaging studies are typically normal.

Tics - signs & symptoms

Tics - history

• The anatomic location(s), number, frequency, complexity, severity, type, duration, and exacerbating or alleviating factor(s) are important parts of the history. Focus on comorbid conditions: ADHD, OCD and LD.
• The National Institute of Mental Health (NIMH) defined PANDAS as follows:
  • Presence of OCD or a tic disorder
  • Onset between age 3 years and the beginning of puberty
  • Abrupt onset of symptoms, course characterized by dramatic exacerbations of symptoms
  • Onset or exacerbations are temporally related to infection with GABHS.
  • Abnormal results of neurologic examination (hyperactivity, choreiform movements, tics) during an exacerbation
• These diagnostic criteria do not always prove helpful in distinguishing PANDAS from other “standard” tic disorders. The existence of PANDAS remains controversial. The high frequency of GABHS and asymptomatic carriers makes it difficult to prove a link between GABHS infection and tics.

Tics - physical exam

Physical examination is usually normal. Tics may not be seen—may need to depend on history. Videotapes are invaluable in diagnosis and differentiation from other movement disorders. Ask child to try to reproduce the sound(s) or the movement(s) they experience.

Tics - tests

Diagnosis depends largely on history. Diagnostic tests are unnecessary. Psychological testing may delineate comorbidities (ADHD, OCD, LD).

Tics - lab

There is little evidence to support a routine testing for GABHS in children suspected of having PANDAS. Throat cultures should be obtained in children with symptoms of pharyngitis.

Tics - differencial diagnosis

Usually, diagnosis is straightforward. Complex or dystonic tics are more difficult to diagnose—may resemble purposeful, normal movements or other abnormal movements. Tics are stereotypical, nonrhythmic, variable, preceded by a premonitory sensation, and under partial voluntary control. These features help distinguish tics from other movement disorders.

• Hemifacial spasm (HFS; rare): Frequent involuntary contraction of one side of the face. Initially, the spasm involves few muscles. It spreads to involve all muscles on one side of the face and become continuous. Early cases of HFS may be difficult to distinguish from motor tics. However, HFS is limited to one side of the face, and the spasm lasts longer than a tic.
• Chorea is an excessive, spontaneous movement, irregularly timed, nonrepetitive, randomly distributed and abrupt. Tics are repetitive and stereotypical, whereas chorea is not repetitive or stereotypical.
• Myoclonus is a sudden, brief, shock-like movement. “Sleep starts” that some people experience while drifting off to sleep is a typical example. Myoclonus is not suppressible, no premonitory sensations.
• Stereotypy is a patterned, episodic, repetitive, purposeless, rhythmic movement. The movements in stereotypy are constant in pattern and location and do not vary over time.
• Fasciculations are randomly occurring spontaneous single muscle twitches. They result from deinnervation and represent single, spontaneously firing motor unit. Both are easily distinguished clinically and by electrophysiological studies (EPS).
• Myokymia is a fine repetitive twitching of muscle bundles. It results from hyperexcitability of peripheral nerve motor axons, readily distinguished clinically and by EPS.
• Tremors: Oscillatory appearance
• Dystonia is an involuntary, sustained, and sometimes repetitive contraction of opposing muscles causing an abnormal posture and/or twisting movements. A dystonic tic (a tic that is transiently sustained in posture) can be difficult to distinguish from dystonia. The lack of premonitory sensation in dystonia helps in distinguishing both.
• Periodic limb movement disorder (PLMD) is a repetitive, often stereotyped limb movement during non–rapid eye movement (non-REM) sleep. PLMD is usually restricted to lower limbs, occurs only during sleep, and has limited variability over time. Diagnosed via polysomnography. PMLD is associated with restless leg syndrome (intense creeping sensation in the legs, relieved by movement).
• Partial seizures may be confused with tics. The automatisms accompanying partial seizures may look like a tic, but there is no premonitory sensation or voluntary control over these movements. EEG is usually normal in tics and may be abnormal in seizures.

Tics - TREATMENT

Many tics do not interfere with the child’s life and do not require specific treatment. Thus, educating the child and the family about tics is often sufficient. Treatment decisions must take comorbid symptoms into account: Aim at the most bothersome symptoms. The waxing and waning nature of tics confounds treatment. It may take weeks to identify benefits of the medication.

Tics - general measures

Tics - diet

There is no evidence that dietary modifications alter the course of tics.

Tics - activity

There is no evidence that lifestyle changes or restriction of activities modifies the course of tics.

Tics - special therapy

• Focal motor (or vocal) tics may be treated with injections of botulinum toxin into the affected muscles (especially for localized dystonic tics).
• Habit reversal (behavioral) therapy (HRT) has gained wider recognition after promising outcomes were demonstrated in a few randomized controlled trials. It is considered as a useful treatment particularly when pharmacotherapy is unsuccessful or results in side effects.

Tics - medication

• Mild/Occasional tics: Medication not needed.
• Moderate or severe: α-2 or dopamine antagonist may reduce severity/frequency.
• With OCD: Selective serotonin reuptake inhibitors can be helpful. Fluoxetine, fluvoxamine, and sertraline appear to be equally effective.
• With ADHD: Guanfacine (or clonidine) useful initial medicine; adding or switching to stimulant medications in refractory cases
• PANDAS: Similar treatment. Evidence for treatment with immunomodulatory therapy or long-term antibiotics is lacking.

Tics - first line

• Clonidine and guanfacine are considered as 1st-line medications.
• Clonidine: Start with 0.05 mg at bedtime. Increase weekly as needed and as tolerated by 0.05 mg to a maximum dose of 0.3–0.4 mg/d divided 3 or 4 times a day. Sedation and orthostatic hypotension are common initial adverse effects. Abrupt discontinuation: Risk of rebound hypertension. Transdermal clonidine patch is an alternative to oral preparations.
• Guanfacine: Start with 0.5 mg at bedtime. Increase as needed and as tolerated by 0.5 mg every week to a maximum dose of 3–4 mg/d, divided twice a day; less sedating/less hypotensive effect compared to clonidine.

Tics - second line

Typical and atypical antipsychotic medications are considered as 2nd-line medications.

• Atypical neuroleptics have fewer extrapyramidal side effects and better tolerability. Usually they are tried 1st. Weight gain is a common side effect:
  • Risperidone: Start with 0.01 mg/kg/dose once a day; dose may be increased by 0.02 mg/kg/d at weekly intervals, up to 0.06 mg/kg/dose once a day.
  • Ziprasidone or olanzapine: Reasonable alternative.
• Standard antipsychotic medications such as haloperidol or pimozide are the most potent. However, these medications commonly cause bothersome side effects such as sedation, weight gain, and galactorrhea. More serious side effects (less common) include extrapyramidal reactions, neuroleptic malignant syndrome, and tardive dyskinesia. Pimozide is associated with QT prolongation, which may predispose the patients to ventricular arrhythmias. The use of typical antipsychotic should be limited to refractory and disabling tics.

Tics - surgery

Recent experimental data have shown deep brain stimulation (DBS) as a potential treatment for adults with severe and refractory tics.

Tics - FOLLOW UP

Tics - prognosis

Although common, tics cause impairment in a minority of children. Peak severity occurs in preadolescent stage. Most patients have partial or complete resolution of tics as adults. Long-term outcome depends on associated comorbidities.

Tics - complications

Although harmless, tics can be distressing and can result in social disability. Injuries, due to complex tics, compulsions, impulsivity, inattention and other factors, can be more frequent in patients with TS. Chronic, repetitive, and forceful tics can cause musculoskeletal symptoms (e.g., cervical spine arthritis, disc herniation).

Tics - bibliography

1. Deckersbach T, Rauch S, Buhlmann U, et al. Habit reversal versus supportive psychotherapy in Tourette’s disorder: A randomized controlled trial and predictors of treatment response. Behav Res Ther. 2006;44:1079–1090.
2. Erenberg G, Cruse R, Rothner A. The natural history of Tourette syndrome: A follow-up study. Ann Neurol. 1987;22:383–385.
Kurlan R, ed. Handbook of Tourette’s Syndrome and Related Tic and Behavioral Disorders, 2nd ed. New York: Marcel Dekker; 2005.
3. Kurlan R. The PANDAS hypothesis: Losing its bite? Mov Disord. 2004;19(4):371–374.
4. Mink JW. Neurobiology of basal ganglia and Tourette syndrome: Basal ganglia circuits and thalamocortical outputs. Adv Neurol. 2006;99:89–98.
5. Mink JW, Walkup J, Frey KA, et al. Patient selection and assessment recommendations for deep brain stimulation in Tourette syndrome. Mov Disord. 2006;21(11):1831–1838.
6. Snider LA, Seligman LD, Ketchen BR, et al. Tics and problem behaviors in schoolchildren: Prevalence, characterization, and associations. Pediatrics. 2002;110:331–336.
7. Tourette Syndrome Study Group. Treatment of ADHD in children with tics: A randomized controlled trial. Neurology. 2002;58:527–536.

Tics - CODES

Tics - icd9

• 307.20 Tic disorder
• 307.21 Transient tics
• 307.22 Chronic tics
• 307.23 Tourette syndrome

Tics - PATIENT TEACHING-MED

The Tourette Syndrome Association is a valuable resource for information. There are many local chapters. www.tsa-usa.org

Tics - FAQ

• Q: Can a child with tics and ADHD be treated with stimulant medication?
• A: Although there have been concerns of stimulants making tics worse, there is no evidence that stimulants cause chronic tics. Furthermore, several recent studies have shown that treatment of ADHD with stimulants does not worsen tics and may lead to improvement.
• Q: Should mild tics be treated if they lead to teasing?
• A: The best approach is to educate the child, teacher, and parents about tics. The child can be armed with a response to questions such as “Those are tics. They are just something I do and I can’t help it.”

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

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More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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