What Medications Should Be Avoided with Long QT Syndrome?

Patient Presentation
An 11-year-old female came to clinic with a hacking cough for the past 4 days. It was bothering her during the day and night. She had no rhinorrhea, fever, nausea, vomiting or shortness of breath. Overall she just didn’t feel well but was attending school and doing her sports activities. She had multiple family members with upper respiratory tract infection symptoms over the past 2 weeks. Her father had been diagnosed with mycoplasma pneumonia 2 days ago after he had had similar symptoms and a chest x-ray that was compatible with the diagnosis. She was fully immunized including a TdaP and influenza vaccine given 4 months previously. Pertussis was circulating in the community, but not influenza. The past and family medical history showed a history of long QT syndrome that was diagnosed after the paternal grandfather had a syncopal incident. Her father and a sibling also had long QT syndrome. She had not had any problems including when taking various antibiotics and antihistamines in the past. She had been diagnosed based on electrocardiogram and genetic testing. She had no history of asthma or other lung disease. The review of systems was otherwise normal.

The pertinent physical exam revealed a tired appearing female with a respiratory rate of 18, normal heart rate and blood pressure. She was afebrile and had normal growth parameters. HEENT was normal. Lung examination had mild end-expiratory wheezing at the base and mid-right lung field. The rest of her examination was normal. The diagnosis of probable mycoplasma pneumonia was made but as she had not had wheezing before a chest x-ray was obtained that showed changes in the posterior basilar segment of the right lower lobe. This confirmed the diagnosis. Because of the history of long QT syndrome the patient was started on doxycycline. Medication for symptoms such as a small amount of codeine to help with sleeping was not encouraged because of the long QT syndrome. The family and patient understood this and said they had been through this before. They would use a humidifier and make sure that she was drinking well. The patient’s clinical course at one week found her to be still coughing, but not as forcefully as before and she was feeling better.

Case Image
Figure 113 – PA and lateral views of the chest demonstrate a faint infiltrate in the posterior basilar segment of the right lower lobe, best seen on the PA view, consistent with bacterial or mycoplasma pneumonia.

Discussion
Mycoplasma pneumoniae is a frequent cause of pharyngitis, pneumonia and acute bronchitis, but acute otitis media is uncommon. Symptoms are usually self-limited and variable and include cough (non-productive usually but later can be productive), fever, fatigue and occasionally headache. Coryza is rare. Cough can continue for 3 to 4 weeks and can be accompanied by wheezing. Radiographic changes include diffuse infiltrates or focal abnormalities but are variable.

Long QT syndrome (LQTS) is a group of disorders that have a prolonged QT interval and a polymorphic ventricular tachycardia. Torsade de pointes (“twisting of the points”) is a particular type of polymorphic ventricular tachycardia that can be associated with LQTS. LQTS can be congenital or acquired. It is increasingly being recognized and may be as common as 1:2500.

Congenital LQTS has many different types. It is associated with chromosomal changes which cause myocardial ion channel problems which interferes with normal cardiac polarization-repolarization leading to arrhythmias. LQT1 accounts for 30-35% of LQTS. LQT2 accounts for 25-40%. LQT3 accounts for 5-10% of patients, and the others account for the remaining percentages. Phenotypically there are two main clinical phenotypes. Romano-Ward syndrome is an autosomal dominant trait that has LQTS without sensorineural deafness. Jervell and Land-Nielsen syndrome is an autosomal recessive trait that has LQTS with bilateral sensorineural deafness. Interestingly, some studies have reported up to 4% of patients who have sensorineural hearing loss to have Jervell and Lange-Nielsen syndrome, but a 2013 study of patients with sensorineural hearing problems identified in statewide newborn hearing screening program found no cases of Jervell and Lange-Nielsen syndrome.

Acquired LQTS usually occurs because of medications but also through various metabolic disorders (particularly hypokalemia or hypomagnesemia), bradyarrhythmias, and other miscellaneous causes.

LQTS manifestations include syncope, ventricular tachyarrhythmias, torsade de pointes and also cardiac arrest or unexplained sudden death. Patients may also be identified through family history or incidental ECG findings. LTQTS subtypes influence the arrhythmia risk and also the choice of treatment including no specific treatment for some patients. Maintenance of normal electrolytes, avoidance of extreme exertion and many different medications are necessary for many patients. The mainstay of treatment for many patients is beta-blockers. Additionally implantable cardiac defibrillators or permanent pacemakers are also used. Surgical denervation of the heart through ligation of the stellate ganglion is also sometimes used.

Episodes can be precipitated by emotion, stress, sleep, sudden noises, and exertion.

Learning Point
Medication choice always requires careful consideration. Patients with LQTS need particular attention as many medications can interfere with cardiac polarization/repolarization.

There are several categories of common medications that should be avoided in patients with LQTS including:

  • Antibiotics – macrolides, fluoroquinolones, trimethoprim-sulfa
  • Antifungals – some azole antifungals
  • Antihistamines – terfenadine and astemizole have been withdrawn from the market because of this risk
  • Antiemetics and promotility agents – ondansteron, phenergen, cisapride
  • Cough/cold medications – pseudoephedrine
  • Stimulant medications – methylphenidate
  • Pain and sedatives – chloral hydrate, oxycodone, methadone
  • Anti-depressants – including tricyclic antidepressants and some selective serotonin reuptake inhibitors including citalopram, fluoxetine.
  • Other groups include antiarrhythmic drugs, antineoplastic drugs, diuretics (because of electrolyte abnormalities), other psychotropic medications and even some herbs.

The list of medications to avoid changes with new medication availability, and new knowledge about the medications and LQTS itself. CredibleMeds® sponsored by the non-profit organization Azcert.org provides one list of medications that should be avoided with LQTS. It is available here. Consultation with the patient’s cardiologist and pharmacist should also be considered when treating patients. Real-life conundrums occur when patients with LQTS have competing problems and risks that also must be addressed such as the need for sedation or patients with asthma who need beta-agonists. Team management with appropriate personnel in concert with cardiologists and prior planning for such problems can assist other care providers when these situations arise.

Questions for Further Discussion
1. What tests are available for mycoplasma?
2. What is the difference between an implantable pacemaker and an implantable defibrillator?

Related Cases

    Symptom/Presentation: Cough

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Arrhythmia and Pneumonia.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Mikesell CE, Atkinson DE, Rachman BR. Prolonged QT syndrome and sedation: a case report and a review of the literature. Pediatr Emerg Care. 2011 Feb;27(2):129-31.

Collins S, Widger J, Davis A, Massie J. Management of asthma in children with long QT syndrome. Paediatr Respir Rev. 2012 Jun;13(2):100-5.

Mycoplasma pneumoniae and Other Mycoplasma Species Infections. Report of the Committee on Infectious Diseases. Pickering LK, ed., 29th ed. American Academy of Pediatrics, Elk Grove Village, IL, 2012.

Chang RK, Lan YT, Silka MJ, Morrow H, Kwong A, Smith-Lang J, Wallerstein R, Lin HJ. Genetic variants for long QT syndrome among infants and children from a statewide newborn hearing screening program cohort. J Pediatr. 2014 Mar;164(3):590-5.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • How Much Cetirizine Can Be Used?

    Patient Presentation
    A 6-year-old male came to clinic with a history of seasonal allergic rhinitis. Loratadine usually controlled his symptoms well, but this spring seemed to be worse than usual. His mother was giving him the medicine in the morning but by the evening he was having increased sneezing, watery eyes and his mouth was itching. He was having problems sleeping also because of the increased rhinorrhea and was more tired during the day. “I started giving him some Benadryl® at night to try to help him sleep,” she said. “The medicine is just not working.” The past medical history revealed some mild atopic eczema, two episodes of bronchospasm that responded to albuterol, and seasonal allergic rhinitis that was worse in the spring and fall. The family history was positive for exercise-induced asthma and seasonal allergies on both sides of the family. The review of systems was negative.

    The pertinent physical exam showed a healthy boy with normal growth parameters. His eyes had allergic shiners and cobblestoning of the palpebral conjunctiva. There was mild redness of the bulbar conjunctiva bilaterally. His nose showed copious clear rhinorrhea with boggy turbinates without polyps. His lungs were clear. His skin had some mild keratosis pilaris on the upper arms. The rest of his examination was negative.

    The diagnosis of seasonal allergic rhinitis and conjunctivitis not responding to his regular antihistamine treatment was made. The physician recommended changing to cetirizine and to use more medicine than what was on the label. He said, “The allergists like this medicine because it seems to work without many side effects, and you can use alot of this medicine. We can increase it to help his symptoms.” He went on, “For your son, I would start with 20 mg or two tablets in the morning, but after a few days if he is still having problems, add another tablet at night. If that isn’t working then you can add another tablet. That would be 40 mg a day. If you look at the label it will tell you only 10 mg a day. It’s a very safe medicine, but let’s start low and see how much he needs to help his symptoms. As things improve with the summer then you can decrease the amount of the medicine too.” The physician also prescribed antihistamine eye drops to help control the eye symptoms, but his mother did not want to use nasal steroids at that time. “Call me in about 5 days or so and let me know how things are going, and we can decide if he needs more of the medicine and also about continuing the eye drops,” he instructed. The patient’s clinical course over the next two weeks showed him needing 20 mg BID to control his symptoms but he was now not using his eye drops. He was not having any medicine side effects and was able to sleep and play. Over the next few months, the boy was able to decrease the cetirizine to 10 mg a day in the summer, but needed 20 mg BID of cetirizine again in the fall.

    Discussion
    Antihistamine medications have been available for over 70 years. The original H1-antihistamines, while effective in treated allergic rhinitis, urticaria and other allergic problems, easily penetrated the blood-brain barrier and caused somnolence. The newer, second generation H1-antihistamines have much less somnolent side effects because of poor penetrance.

    Fexofenadine (Allegra®), desloratadine (Claritin®) and levocetirizine (Zyrtec®) are commonly used H1-antihistamines in the US today. Previously Astemazole (Hismanal®) and Terfanadine (Seldane®) were used but were withdrawn because of problems with cardiac arrhythmias with a propensity to prolong the QT interval. Fexofenadine in studies has the same somnolence producing effect as placebo. Desloratadine and cetirizine both can have some sedative effects.

    Cetirizine is extensively and rapidly absorbed by the gut allowing a rapid onset of action. It interacts with target receptors at effective concentrations, and organs where it would not be effective or toxic are avoided. There is no hepatic metabolism (thus avoiding many potential drug-drug interactions) and the drug is excreted unchanged by the kidney. The half-life is about 10.5 hours in adults. Cetirizine has higher binding affinity for receptors than other similar drugs thus it has “…a more potent, faster onset and longer duration of action…” when used for seasonal allergic rhinitis. “…[T]here is evidence that continuous treatment with cetirizine is more effective than on-demand treatment in achieving significantly greater inflammatory and clinical control in [allergic rhinitis] patients.” Cetirizine does not have cardiotoxic side effects, and is a pregnancy category B medicine ( = without harm to animal fetus and no human studies available). It is excreted in breast milk therefore is not recommended during lacatation. Cetirizine is contraindicated for people with known hypersensitivity to its components or hydroxyzine.

    Learning Point
    Cetirizine is a safe and effective medication, and because of this some allergists will use it off label in higher doses. As with any time a medicine is used off-label, it is important to monitor the patient closely for efficacy and especially for potential side effects. It is the human metabolite of hydroxyzine (Atarax®) that is used for severe urticaria/pruritis and seasonal allergic rhinitis.
    Normal dosing for cetirizine is:
    6 months-2 years, 2.5 mg daily to BID
    2-5 years, 2.5 mg daily to BID
    6-11 years, 5-10 mg daily to BID
    > 12 years, 5-10 mg daily to BID

    Hydroxyzine is often used at doses of 2 mg/kg/day up to 75 mg BID. Hydroxyine to cetirizine as an equivalent is 25 mg of hydroxyzine to 10-20 mg cetirizine equivalent (personal communication with pediatric allergist).

    Thus for a hydroxyzine dose of 75 mg/dose, the cetirizine dose would be 30-60 mg/dose. Using an average of 20 kg weight for the 6 year old above, the dose would be 16-30 mg/dose. Thus the physician above starting at 20 mg once a day was within this range and the amount the child needed to control his symptoms (40 mg/day) was also within this range.

    A reported overdose in an adult of 150 mg caused somnolence but no other problems. An overdose in an 18 month old patient of ~180 mg caused restlessness, irritability and then drowsiness. There were no other problems.

    Questions for Further Discussion
    1. What are indications for use of intranasal steroids for seasonal allergic rhintis?
    2. What are indications for use of antihistamine eye drops for allergic conjunctivitis?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Allergies and Cough and Cold Medicines.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Cetirizine. RxList. Available from the Internet at http://www.rxlist.com/zyrtec-drug.htm (rev. 5/16/2007, cited 5/26/2014).

    Zhang L, Cheng L, Hong J. The clinical use of cetirizine in the treatment of allergic rhinitis. Pharmacology. 2013;92(1-2):14-25.

    Church MK, Church DS. Pharmacology of antihistamines. Indian J Dermatol. 2013 May;58(3):219-24.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What Causes Neck Stiffness?

    Patient Presentation
    A 14-year-old female came to clinic with a 3-day history of increasing neck stiffness. The pain was mainly left-sided and got worse as the day went on. She was also having generalized headaches in the evening that would resolve with sleep. She denied fevers, chills, nausea, emesis, or photophobia or other pain. She denied numbness or tingling in shoulders or arms. The past medical history showed that she was fully vaccinated and had 3 migraine headaches since the age of 12 that were controlled with sleep and ibuprofen. The family history was positive for migraine headaches and a paternal grandfather with a replaced knee. The review of systems was non-contributory.

    The pertinent physical exam showed a female in no distress who was alert and oriented x 4. Vital signs were normal including temperature. HEENT was normal except for pain of the left trapezius and sternocleidomastoid muscles. This became worse with stretching of these muscles, but there was complete range of motion in the neck. Muscle palpation showed tense, spasmed muscles. The left occipitalis muscle also had some minor pain near the posterior insertion. Movement of the shoulder also caused mild pain when these muscles were engaged. Neurological examination was normal with good tone, strength and normal sensation in the face, neck, arm and shoulder. The diagnosis of muscle spasms of the left neck muscles was made. The physician noticed a large backpack in the room and asked the patient about it. She had started the school year that week and was carrying several large textbooks all day with her. She carried the backpack on her left shoulder only. The physician herself could barely lift the backpack, and talked with the patient about ways to decrease the weight (e.g. use online books if available, take only the books necessary at one time) and to wear the backpack on both shoulders or to use a pull-type, roller backpack on the ground if excessive weight was necessary. The patient was told how to use anti-inflammatory medications, heat and gentle exercise and massage to help eliminate the spasm. “You should be careful about your posture too.” she said. “People sitting in chairs or working at computers for a long time can make this worse. You need to get up and move frequently and stretch even for a minute. Then come back and do your work.”

    Discussion
    The complaint of neck stiffness always makes the clinician a little concerned until he/she understands the whole history because of the potential diagnosis of meningitis/encephalitis. While this potential is always concerning, there are many other causes of neck stiffness or pain to consider that are much more common. Normal wear and tear, injury or overuse that occur in daily activities and work can cause neck stiffness or pain. Often, even in adults, the cause of the pain is not recognized. Good examples are the adolescent above, or an innocent stumble, particularly if carrying something that may cause a person to be off-centered, twist their body to regain balance and only later cause a stiffness or soreness. The little stumble is not recognized as the cause of the neck stiffness.

    Meningitis is an inflammation of the meninges. The most feared causes are rapidly growing bacteria such as meningococcus. Aseptic meningitis is usually caused by nonbacterial organisms and other diseases including enteroviruses, measles, mumps, and mycoplasma. Organisms colonize the person usually in the nasopharyngeal mucosa, spread to the blood stream and eventually reach the meninges by the blood-brain barrier and cerebrospinal fluid after evading the person’s immunological defenses. Lumbar puncture is needed to help determine if meningitis is present and the potential organism. To review what are the initial cerebrospinal fluid findings for meningitis, click here.

    Meningismus that is associated with meningitis is neck pain with flexion of the neck, not lateral movement. In a seated upright position with the neck fully extended, the neck is flexed and resistance may be felt throughout the movement or just at the end of the movement.

    Learning Point
    The differential diagnosis of stiff neck includes:

    • Infectious
      • Abscess – retropharyngeal or peritonsilar
      • Lymphadenitis, cervical
      • Encephalitis
      • Meningitis
      • Discitis
      • Herpes zoster
      • Osteitis
      • Poliomyelitis
      • Tetanus
    • Neurological
      • Cerebral palsy
      • Epidural hematoma
      • Intracranial hemorrhage
      • Post-lumbar puncture
      • Vertebral anomaly
    • Arthritis – with prominent symptoms in the neck joints
    • Deconditioning or overuse of muscles
    • Drugs
    • Trauma to neck – whiplash where the muscles and ligaments are stretched with pain and inflammation
    • Torticollis – spasm of the sternocleidomastoid muscle or hemorrhage
    • Tumor – primary or metastatic

    Questions for Further Discussion
    1. What bacterial organisms cause meningitis?
    2. How much weight is recommended to be carried in a backpack?
    3. What are indications for radiological evaluation of a patient with neck stiffness?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Neck Injuries and Disorders

    .

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Sheldon SH, Levy HB. Pediatric Differential Diagnosis. 2nd Edit. Second Edition. Raven Press: New York. 1985:153-154.

    Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1988:250-252.

    Curtis S, Stobart K, Vandermeer B, Simel DL, Klassen T. Clinical features suggestive of meningitis in children: a systematic review of prospective data. Pediatrics. 2010 Nov;126(5):952-60.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

  • What are the Most Common Motor and Vocal Tics?

    Patient Presentation
    A previously healthy 10-year-old female came to clinic with new onset of eye blinking that the mother had noticed for 2 weeks. The patient initially didn’t notice it, but was becoming more aware and said that she noticed it occurred more when she was upset or excited. Her friends had not noticed it. The eye blinking didn’t seem to bother her in general and the mother said that she saw only increased eye blinking in the evenings particularly when she was tired. They both agreed that she was only having eye blinking and denied any abnormal motor or vocal movements despite extensive examples. The patient denied any visual changes, photophobia, changes in tearing, or erythema of the eye structures. The past medical history was positive for seasonal allergic rhinitis, but the patient was not having any other allergic symptoms. The family history was positive for eye blinking and cheek puffing tics in her mother as a child that resolved before middle school. There was no neurological, psychiatric or school problems in the family. The review of systems was negative.

    The pertinent physical exam showed a cooperative female with increased eye blinking throughout the examination. Her growth parameters were at the 90%. Her vital signs were normal. Visual acuity was 20/25 in the left eye and 20/20 in the right. Eye examination was normal as was the rest of her examination. The diagnosis of a simple or provisional motor tic was made. The family was counseled that most of these resolve within a few weeks to months, but it could be persistent. It could also herald the beginning of other tics. As the current tic was not bothering her, the family was told to continue to monitor it and if it became more of a problem or if more complex motor or vocal movements began, to contact the office. The patient’s clinical course showed an increase in the eye blinking for about 2 months, then it resolved. At two years later she had no other tics.

    Discussion
    Tics are usually single repetitive, non-rhythmic, non-purposeful movements or utterances. Tics can be multiple and complex however. Tics may be preceeded by a premonitory urge where the person has a feeling that a certain type of tic is going to occur and then this feeling goes away after the tic is produced.

    Tics, especially simple motor tics, are very common with ~10-15% of elementary age children having a tic at some time. In one community-based study, the overall prevalence was 3-9% with an overall frequency of 24% of elementary school children during one school year. Tics are more common in boys and present around age 6-12 years, but may be not recognized until as late as 21 years. They are usually most severe around 10-12 years of age. Motor tics usually appear 2-3 years before vocal tics but vary with the individual. Tics can last for a few weeks, months or be chronic (> 1 year of symptoms). Simple tic or provisional tics last less than 1 year. Chronic or persistent tics last more than 1 year. Note that the definitions are based on timing of symptoms not the severity or the complexity of the tics themselves.

    Tourette syndrome (TS) is a particular type of tic but for the lay public may be the most well known. It is a chronic tic disorder where patients have both motor and vocal tics, although these may occur at different times. Patients with TS also have psychiatric symptoms such as obsessive-compulsive disorder, attention deficit disorder, depression, anxiety and others. Patients often have complex tics. The tics must have started before age 18 and they cannot be due to medications or other medical conditions to be diagnosed with TS.

    Treatment is education and watchful waiting mainly with patients with complex, chronic or tics that are disturbing using a variety of alpha-2 agonists, anti-psychotics and botulinum toxin. Habit reversal training or different types of electrical brain stimulation have also been used.

    The prognosis in most children is generally good with resolution for many patients. Unfortunately, despite research at this time, there are no specific predictive signs, symptoms or tests which can determine the prognosis for an individual.

    The differential diagnosis of other involuntary movement disorders includes:

    • Chorea and choreoathetosis
    • Hiccough
    • Myoclonus
    • Reflexes – Moro, startle
    • Spasmus nutans
    • Tics
    • Tremor
    • Torticolis, spasmodic
    • Torsion spasms
    • Other
      • Ballismus
      • Hysteria
      • Rett syndrome
      • Stereotypical movements with autism

    Learning Point
    The most common motor tics involve the face and head with ocular tics being particularly common. The most common vocal tics are throat clearing and sniffling which can be misdiagnosed as allergic symptoms or asthma. Patients with Tourette Syndrome (TS, discussed above) and those with other types of tics have other unusual behaviors such as echolalia (repeating someone else’s words), pallilalia (repeating one’s own words), coprolalia (obscene speech), echopraxis (repetitive gestures), copropraxis (obscene gestures), and coprographia (obscene writing). These behaviors are considered complex tics.

    Questions for Further Discussion
    1. What is PANDAS and how is it related to tics?
    2. What is the Yale Global Tic Severity Scale?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Movement Disorders and Tourette Syndrome.

    To view current news articles on this topic check Google News.

    To view images related to this topic check Google Images.

    To view videos related to this topic check YouTube Videos.

    Illingworth RS, Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1988:227-234.
    Snider LA, Seligman LD, Ketchen BR, et. al. Tics and Problem Behaviors in Schoolchildren: Prevalence, Characterization, and Association. Pediatrics. 2002;110;331-336.

    Shprecher D, Kurlan R. The management of tics. Mov Disord. 2009 Jan 15;24(1):15-24.

    Siniatchkin M, Kuppe A. Neurophysiological determinants of tic severity in children with chronic motor tic disorder. Appl Psychophysiol Biofeedback. 2011 Jun;36(2):121-7.

    Ludolph AG, Roessner V, Munchau A, Muller-Vahl K. Tourette syndrome and other tic disorders in childhood, adolescence and adulthood. Dtsch Arztebl Int. 2012 Nov;109(48):821-288.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.

    Author

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital