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Acute Otitis Media

Pediatrics In Review

March 1, 2025

There remains a gap in the implementation of the appropriate diagnostic criteria and management of acute otitis media.After completing this article, readers should be able to:List the diagnostic criteria for acute otitis media.Discuss the therapeutic management of acute otitis media.Describe the components of the pediatric otoscopic exam needed for the management of acute otitis media.Describe the important role of the parent/caregiver in the diagnosis and the management of acute otitis media.Acute otitis media (AOM) is the most common cause for antimicrobial treatment in young children, accounting for 56% of antimicrobial prescriptions for children aged 3 to 36 months and 40% of antimicrobial prescriptions for children aged 3 to 6 years.1,2 It is the second most common diagnosis after upper respiratory infection in children. The epidemiology of AOM has international and regional variances and has been impacted by both pneumococcal vaccines and the COVID-19 pandemic.3,4 Approximately 50% of children will have 1 episode of AOM by age 2 years, and by age 3 years, 80% will have had at least 1 episode.5Health disparities have been noted in children with AOM.6 Children who are socially disadvantaged are less likely to receive treatment and more likely to experience complications of misdiagnosed and mismanaged episodes of AOM. Frequent ear infections have been associated with living below the poverty level. In under-resourced countries, chronic supportive otitis media is a leading cause of hearing loss.7The most specific symptom of AOM is acute otalgia. This acute otalgia can manifest in many ways, from the acute onset of ear pain in the school-aged child to nighttime awakening, fussiness, and decreased appetite in younger children. While the acute otalgia is usually constant, it often appears to be worse in the evening. Fever can be associated with AOM but is not a consistent finding associated with AOM. Finally, recent or concurrent upper respiratory infection symptoms such as nasal congestion will often accompany the key symptoms of AOM.8The peak incidence of AOM is between age 6 to 12 months. It is multifactorial in terms of risk factors, with males being at a slightly higher risk than females. Genetic predisposition, passive smoke exposure, conditions associated with decreased immunity, ciliary dysfunction, cochlear implants, daycare attendance, and family history of recurrent AOM in parents or siblings are other risk factors associated with AOM.2AOM can be caused by viruses, bacteria, or a combination of both. Bacterial causes include the most common bacteria that occupy the upper respiratory tract: Streptococcus pneumoniae, non-typeable Haemophilus influenzae, and Moraxella catarrhalis. Since the implementation of conjugate pneumococcal vaccines, AOM is more commonly caused by nonvaccine serotypes. In terms of viral causes of AOM, the most common viral pathogens of otitis media include respiratory syncytial virus, adenovirus, influenza viruses, coronaviruses, human metapneumovirus, and picornaviruses. Infection with both viruses and bacteria is quite common, with one study documenting 66% coinfection rates.9Many practicing clinicians likely noticed a significant decrease in the incidence of AOM during the COVID-19 pandemic. Several studies documented this decrease, along with a decrease in the use of antibiotics and emergency department visits for AOM.10,11 This decrease has largely been attributed to the social distancing and mask use that was in place during the pandemic. The incidence of other viral respiratory infections was also decreased during this time. The presence of SARS-CoV2 in patients with AOM has also been documented, suggesting that the 2 conditions can coexist.12AOM results from the acute inflammation of fluid in the middle ear space, which is dense with bacterial otopathogens. Many factors contribute to the acute inflammatory cycle, including bacterial persistence, viral infections, allergic rhinitis, recent or current upper respiratory infection, and anatomic variation and abnormalities, which set up the genesis of AOM.7 The eustachian tube is particularly essential in protecting the middle ear space from bacterial pathogens and viruses. A properly functioning eustachian tube is essential to the drainage of secretions. The eustachian tube also serves to equalize pressures. In young children under 2 years of age, the eustachian tube is less effective in these functions because it sits in a more horizontal position, leading to a slower drainage of secretions and allowing for more transmission of pathogens into the middle ear space.The diagnosis of AOM rests on the visualization of the tympanic membrane and clinician competence in performing the overall ear exam.13 Therefore, it is important to understand the diagnostic criteria and to be proficient in performing the pediatric ear exam. The accurate diagnosis of AOM facilitates the appropriate usage of antimicrobial therapy for AOM. This also helps to mitigate the unnecessary use of antibiotics, thereby decreasing the overuse of antibiotics in general. Rising health care costs, increasing antibiotic resistance, and the possible need for surgical referrals for recurrent AOM stress the critical importance of an accurate diagnosis of AOM.Understanding the anatomy of the middle ear is essential to making an accurate diagnosis of AOM. The middle ear is the area between the tympanic membrane (which separates the external and middle ear spaces) and the oval/round windows (which mark the beginning of the inner ear). The eustachian tube connects the upper respiratory tract to the middle ear and is more horizontal in infants and children. Several resources are available that physicians can use to explain to parents how AOM may arise in their child.14When examining a normal tympanic membrane (TM), the membrane appears translucent, and the malleus is often seen through the TM in the anterior-superior quadrant (Figure 1). On the other hand, in cases of AOM, the diagnosis is dependent on a bulging TM (Figures 2 and 3). There is often decreased mobility of the TM, and the color is often erythematous with purulent and turbid fluid behind the membrane, suggestive of a bacterial infection.Training in pediatric otoscopy involves learning how to perform a proper ear exam in a child, which includes the use of appropriate techniques for holding and distracting a child, performing pneumatic otoscopy, and removing cerumen. A checklist (Table 1) containing the essential components of a pediatric ear exam has been shown to be helpful in teaching settings.15,16 It is important to remain proficient in pneumatic otoscopy and cerumen removal because it has been shown that these skills can decline over time.17The examiner should perform a deliberate, stepwise exam. When performing the otoscopy exam, it is important to first determine the overall status of the patient, especially with regards to age, development status, and the severity of their illness. This will guide the examiner's approach to the ear exam. Using an appropriate otoscope that provides adequate illumination and resolution to visualize the TM will allow the examiner to make a diagnosis of AOM. It is also important to use an otoscope that allows for the pneumatic otoscopy. Appropriately sized scope tips should be used based on the size of the child and the presence of any craniofacial anomalies. In addition, other developmental needs of the child should be considered; for instance, some children may be afraid of the light from the otoscope, thinking that it may be hot or too bright for their eyes.Next, the examiner should attend to the positioning of the patient. The school-aged child is likely to be on the examining table, and an otoscopy exam can generally be done with appropriate distraction and gentleness without the aid of a parent or caregiver. The younger child should be on the lap of the parent/caregiver or on the examining table with parent/caregiver assistance. Generally, infants younger than 9 months should be on the examining table in a supine position for a safe exam. An effective otoscopy exam does not necessarily require a lot of time. A 'few golden minutes' should suffice in the absence of cerumen; during this time, the child is held in a comforting position that allows the examiner to perform a double-handed exam in which the nondominant hand is used to steady the head, and the dominant hand manipulates the otoscope to visualize the TM.Once the examiner can see the anatomy, the examiner should take note of various characteristics of the TM, including the position of the TM, the color, presence of the bony landmarks, translucency of the TM, and any discharge.18Pneumatic otoscopy is an integral component of the overall pediatric ear exam. It allows for the visualization of the movement of the TM based on pressure changes. It is helpful in determining the presence of effusion in the middle ear space. Performing pneumonic otoscopy requires the examiner to be adept at the basic otoscopy skills described above. It involves attaching a bulb to the otoscope and then applying positive pressure (deflation of the bulb) and negative pressure (inflation of the bulb).Many TM conditions can be associated with abnormal responses to positive and negative pressures. Most often, the reason for impaired mobility of the TM is due to effusion in the middle ear space. As AOM involves the accumulation of fluid in the middle space, impaired movement of the TM as determined by pneumatic otoscopy is associated with AOM. Still, pneumatic otoscopy does not differentiate between serous and purulent effusion. Importantly, it can also be the first step in the evaluation for other more concerning etiologies such as cholesteatoma. A child with impaired mobility of the TM as determined by pneumatic otoscopy should be followed closely.The 2013 American Academy of Pediatrics (AAP) guideline provides a framework for the diagnosis of AOM.8 It is important to recognize that the bulging of the TM is required in each of these criteria in order to make the diagnosis of AOM. Below are excerpts from the AAP guideline regarding the diagnosis of AOM. 'Statement 1A: Clinicians should diagnose acute otitis media (AOM) in children who present with moderate to severe bulging of the TM or new onset of otorrhea not due to acute otitis externa. Evidence Quality: Grade B. Strength: Recommendation.''Statement 1B: Clinicians should diagnose AOM in children who present with mild bulging of the TM and recent (less than 48 hours) onset of ear pain (holding, tugging, rubbing of the ear in a nonverbal child) or intense erythema of the TM. Evidence Quality: Grade C. Strength: Recommendation.'Conditions such as otitis media with effusion (OME) (Figure 4), pharyngitis, and viral upper respiratory infection can mimic some of the symptoms of AOM. However, the acute onset of moderate to severe otalgia is not common with these other conditions. OME is often associated with abnormal findings of the TM such as air-fluid levels or a prominent short process of the malleus, mild otalgia, and often a sensation of ear fullness or popping when swallowing (Figure 3). With OME, there is no bulging of the TM or other associated findings of acute inflammation of the middle ear effusions such as a purulent color of the TM. The management of OME is supportive and requires no medications. Still, OME must be followed up until resolution. It is important to document resolution of any abnormal TM finding so as to not overlook chronic OME or other conditions that might present initially in a similar manner such as a cholesteatoma. Of note, despite the distortion of the TM, OME does not represent an acute virulent bacterial infection of the middle ear fluid. Therefore, OME does not necessitate the use of antibiotics.Chronic OME is defined as the persistence of middle ear effusion for more than 3 months. Evaluation by an otolaryngologist is recommended for children with unresolved OME and atypical symptoms accompanying OME such as concern for worsening hearing. Thus, is it important to be able to differentiate between the tympanic findings of OME and AOM, stressing again the importance of competency in the examination of the middle ear.13,19The management and therapies discussed here will be broad based and follow evidence-based criteria. Pediatricians should consider the AAP guideline, studies published following the AAP guideline and the prevalence of pathogens in their community, and should adhere to a strict evidence-based approach in the treatment of their patient.8 It is vital that pediatricians continuously update their knowledge and skills regarding diagnostic criteria, epidemiology, and management plans. Online resources such a peer-reviewed pediatric website offer the pediatrician access to rapid PubMed searches regarding current evidence-based therapeutic regimens.18,20 It is important to reference only peer-reviewed online resources.Some patients with AOM can be safely managed without antimicrobial therapy. The natural history of the condition is such that the pain and discomfort will resolve after the first few days, and the use of antibiotics may result in 1 less day of symptoms. Safety-net antibiotic prescriptions and other similar protocols21,22 have been described, including in the AAP guideline, as a nonantimicrobial management protocol. Criteria for such management usually include older children with non-severe AOM (mild otalgia for less than 48 hours and temperature of less than 38.5°C), no other bacterial infection, and nontoxic status. These children can be followed closely without the use of antimicrobial therapy. Close follow-up is needed, and antibiotics should be started if signs and symptoms worsen or do not improve over 48 to 72 hours. Current studies following publication of the AAP guideline do not support safety-net antibiotic prescriptions and other similar non-antibiotic treatment plans in an evidence-based manner for children younger than 2 years. Studies have shown that withholding antibiotics in children younger than 3 years can result in unfavorable outcomes.23 Also, it is possible that many of the cases of AOM that resolved without antibiotics may have a viral etiology or may not actually have been AOM and rather were OME. This again calls for the need to be competent in the diagnosis of AOM and adhere to the diagnostic gold standard.When managing patients with AOM, a key consideration is the control of pain, recognizing that the acute otalgia of AOM may resolve only after the first 48 hours of antimicrobial therapy. Appropriate nonprescription analgesic medication is the foundation of management of AOM in children. Oral acetaminophen or ibuprofen can be used to treat the otalgia. Aspirin should never be given for pain management. There is no evidence that pain caused by AOM is improved by alternating acetaminophen and ibuprofen in children,24 and this regimen can lead to medication dosing errors. Otic drops for pain can be considered only after a patient has been examined and appropriately diagnosed. Otic drops are contraindicated in some conditions, including the presence or uncertainly of a ruptured TM.Shared decision-making with families is appropriate in almost all cases of AOM. Indeed, educating the parent/caregiver about the diagnosis of AOM and the indications for antimicrobial treatment optimizes the overall management of AOM in the pediatric patient. Table 2 shows a script of talking points that can be used as a guide in the discussion and communication of managing AOM with a parent or caregiver. Additional resources including visual aids appropriate for parents are also available to help support parent/caregiver education about this condition.14,20,25The AAP guideline recommends antibiotic therapy for bilateral AOM in children aged 6 to 23 months even if they do not have severe signs or symptoms;8 more recently, Wald has urged a similar approach (either treatment or close observation) to unilateral or bilateral AOM in younger children.26 Clinicians will usually prescribe antibiotic therapy for children with severe signs or symptoms of AOM such as a temperature above 39°C, moderate to severe otalgia, or otalgia for at least 48 hours. The current AAP guideline recommends amoxicillin as first line of therapy for children who do not have an allergy to penicillin, have not been on penicillin in the past 30 days, and do not have purulent bilateral conjunctivitis.It is recommended that antibiotics with beta-lactamase coverage for AOM should be prescribed if the patient has received amoxicillin in the past 30 days, has concurrent purulent conjunctivitis (suggesting H influenzae infection), and has a history of recurrent AOM unresponsive to amoxicillin of if one suspects H influenzae as the cause of AOM. Attention should be paid to local prevalence microbiology data as well.8,19Since the AAP guideline in 2013, well-regarded studies have emerged with recommended management regimens that differ from the guideline. Especially due to the impact of pneumococcal vaccines, there have been significant changes in the prevalence of microorganisms for AOM,27 thus affecting the antimicrobial management of AOM in the pediatric patient. For instance, in 1999, S pneumoniae accounted for 40% to 45% of AOM, with H influenza (25% to 30%), Moraxella catarrhalis (15% to 20%), and Strep pyogenes (3% to 5%) following. In 2017, the pattern was S pneumoniae (15% to 25%), H influenza (50% to 60%), M catarrhalis (12% to 15%), and S pyogenes (3% to 5%). Overall, there has been a decrease in penicillin-resistant S pneumonia. Given these changes since the AAP guideline, Wald recommends a regimen with 'regular dose amoxicillin-clavulanate as the preferred treatment (45 mg/kg/day in 2 divided doses of 400 mg/57 mg) for children with AOM' for 10 days to treat H influenzae and penicillin-sensitive S pneumoniae as initial therapy.27 Pediatric clinicians should continue to monitor changes in microbial prevalence and resistance patterns to continue practicing in an evidence-based manner.The AAP guideline recommends the traditional 10 days of antimicrobial treatment. Since the publication of the guideline, various studies have suggested that a 5- or 7-day course may be just as effective as the traditional 10-day course.28 However, Hoberman et al demonstrated that a reduced duration of treatment with amoxicillin-clavulanate involving children aged 6 to 23 months with AOM was less effective.23 Following the AAP guideline of 10 days of treatment, especially for younger children, seems most prudent currently.According to the current AAP guideline, unless the child has an anaphylactic reaction to penicillin, a course of 80-90 mg/kg/d of amoxicillin for 10 days is recommended. In the case of a non-type 1 allergic reaction, a cephalosporin such as cefdinir is recommended as first-line therapy.29 Macrolides are not indicated in the treatment of AOM due to reported resistance. Further choices of antimicrobial treatment should be based on evidence-based guidance, community prevalence, and resistance patterns, with the advice of a pediatric infectious disease specialist as needed.If the patient does not respond to amoxicillin in a timely manner, an agent such as amoxicillin/clavulanic acid should be prescribed to treat beta-lactamase-producing agents such as non-typeable H influenzae. Recent studies of the pharmacokinetics and pharmacodynamics of specific cephalosporins in respiratory infections suggest against the use of cefdinir in this scenario.29-31 Rather, intramuscular ceftriaxone should be considered. Most importantly, the patient who does not respond in a timely manner needs to be...

Journals & PublicationsOtitis Media, AcuteOtoscopyTympanic Membrane

https://publications.aap.org/pediatricsinreview/article/46/3/139/201120/Acute-Otitis-Media

Tourette Syndrome And Tic Disorders

Pediatrics In Review

February 1, 2024

Improved recognition, education, and consideration of tic phenomenology, comorbidities, and treatment is essential for improved patient and family outcomes. (1)(2)After completing this article, readers should be able to:The early history of Tourette syndrome ranges from witchcraft and Tolstoy to Jean Itard's 1825 description of a French noblewoman (the Marquise de Dampierre) with childhood-onset and progressively worsening movements and vocalizations (including obscenities). (3)(4)(5)(6) In 1885, Georges Gilles de la Tourette described a series of 9 individuals (including the Marquise) with childhood onset, waxing and waning, stereotyped movements and noises, including echolalia (repeating words and phrases from the environment) and coprolalia (profanity), and the presence of an uncomfortable premonitory sensation before onset of the movement or vocalization. (7)(8) Sigmund Freud, a colleague of Tourette's, was interested in patients with complex tics and an array of symptoms labeled as 'hysteria'; therefore, it is not surprising that this disorder initially was linked to neurosis, narcissism, and onanism (self-gratification) following the Freudian psychoanalytic view. (9) In the 1960s, after a case series of tics that were successfully treated with haloperidol was published, there became a greater awareness of this disorder and an initiation of studies seeking a central nervous system pathology as the underlying cause of tic disorders. (10)(11)Our knowledge of tic disorders continues to expand. There is greater understanding of tic characteristics, outcome, and comorbid neuropsychiatric disorders. Although the exact pathophysiology of tics remains under investigation, tics and tic disorders are considered neuropsychiatric disorders with a complex interplay involving genetics, environmental factors, brain circuity, and neurotransmitters. (12)(13) Although there is no known cure for tics, behavioral and pharmacologic treatments can be beneficial in controlling tics.Tics are defined as 'sudden, rapid, brief, recurrent, nonrhythmic, motor movements (motor tics), or vocalizations (phonic/verbal tics).' (13) Both motor tics and phonic tics are subdivided into simple or complex categories (Table 1, Video 1). Patients may have a single tic or, more commonly, a fluctuating pattern of different movements and sounds. Motor tics are also described phenomenologically as clonic (jerklike), dystonic (transiently sustained muscle contraction), tonic (isometric contraction), stereotypic (rhythmic, repetitive), blocking (cessation of movement), and compulsive (driven by an obsession). (13)Tics are quite common in the pediatric population. Up to 27% of children have tics at some point during childhood. The prevalence of Tourette syndrome (chronic motor and phonic tics) is approximately 1%, with estimates varying due to study methods and estimates of unidentified cases. Males are more affected than females (approximately 4:1). (14) The average age at onset for tics is 4 to 7 years, although older and younger ages at onset are common. (15)(16)Tics typically have a waxing and waning pattern, an evolving mixture of new and old sounds/movements, and a fluctuating frequency and intensity. Motor tics typically start in the face/head/neck region and extend to involve the trunk and extremities. Motor tics also tend to start before vocal tics, although this is quite variable. Tics are divided into 'simple tics' involving individual or isolated movements or noises and 'complex tics' involving more orchestrated or sequences of movements or words (Table 1). Simple tics usually precede the appearance of complex tics. (15)(16) Although it is commonly feared, complex vocal tics involving the use of foul language (coprolalia) occur in less than 10% of patients with tic disorders. (13)Brief exacerbations are frequently precipitated by excitement, stress, anxiety, fatigue, discussing one's tics, or infections. In contrast, tics are often reduced when the individual is concentrating or focused on other activities, relaxed, or sleeping. (13) Tics are commonly preceded by a premonitory urge described as a desire to perform the tic or sensation that occurs in 37% of children and 90% of teenagers and adults. (17)(18) This premonitory urge/sensation may be either physically localized to the tic location or more generalized, and the urge typically resolves after the tic is performed. (19)(20) Tics are partially suppressible in many children as they age. (21)(22) The premonitory urge's role as a driving force, diagnostic clue, and/or outcome predictor remains controversial, but it is a major component of behavioral therapy (see later herein). (21)(22)(23)(24)The diagnosis of a tic disorder is made from the clinical history and examination findings. The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) defines 5 separate tic disorders largely based on the type of tics that are present and the duration of time since their origin (Table 2). (25) A provisional tic disorder is defined as the presence of motor and/or vocal tics for less than 1 year. A chronic motor or chronic vocal tic disorder is defined as the presence of solely motor or vocal tics for longer than 1 year. Tourette syndrome is diagnosed by the presence of both multiple motor tics and at least 1 vocal tic for greater than 1 year (although they do not have to occur concurrently). (25) Two other criteria that pertain to all the aforementioned diagnoses include 1) onset before age 18 years and 2) neither substance-induced nor due to a general medical condition. For example, secondary tics are seen in association with a variety of neurodegenerative disorders (Huntington disease, brain iron accumulation, neuroacanthocytosis) or after head trauma, stroke, toxic exposure, or surgery. (13) Other specified tic disorder indicates that the individual has tics but does not fulfill the criteria of the previously listed diagnoses for a specific reason (eg, onset of tics after age 18 years). Last, unspecified tic disorder indicates that the individual has tics but, in contrast to the preceding diagnosis, the reason why the individual does not meet the criteria for the first 3 diagnoses is not provided.Although widely used, the DSM-5 criteria have a variety of intrinsic concerns. For example, the selection of required time (1-year cutoffs) is arbitrary, and the distinction between motor and vocal tics is unclear (eg, a vocalization could be secondary to a diaphragmatic contraction). (26) Furthermore, some authors have suggested that these disorders exist along a spectrum rather than as individual diagnoses. (26)(27)In addition to the formal tic classifications described previously herein, following the onset of the COVID-19 pandemic and associated international lockdowns in early 2020, there has been a worldwide explosion of severe, atypical, fulminant-onset ticlike behaviors. (28) These behaviors, frequently occur in teenagers and are similar to previously described functional (psychogenic) tics. Etiologically, they are thought to be linked to the underlying stress of the pandemic and exacerbation of coexisting anxiety and mood issues. (28) Formal criteria for the diagnosis of 'functional ticlike behaviors' or a 'functional tic disorder' have been proposed. (29)(30) In addition, because a large percentage of affected individuals initially viewed similar movements/sounds via the internet before the onset of their symptoms, it has also been labeled a social media disorder triggered by the stress of the pandemic in a vulnerable population with a tendency toward suggestibility. (31)(32)(33)(34)(35) A similar 'mass psychogenic illness' has been previously reported in LeRoy, New York, in 2011-2012. (36)Increasing evidence points toward a spectrum of tic severity, variability in outcome, and an important role for tic-related comorbidities in determining quality of life. (27)(37) A specific individual's course is not easy to predict because tics tend to have a natural waxing and waning pattern over time. When persistent, tics statistically significantly reach their greatest severity between 9 and 11 years of age. Nevertheless, every individual can be quite different. Several predictors of tic severity have been suggested, but none have been confirmed. (13) Up to two-thirds of children tend to improve during adolescence, and up to one-third of children may eventually have their tics resolve completely. (16)Up to 86% of patients with chronic tic disorders will develop at least 1 comorbid psychiatric disorder. In addition, in many patients, the negative impact of these coexisting problems can be more significant than the tics. (12)(38)(39)(40)Obsessive-compulsive behaviors (OCBs) occur in approximately 50% of children with Tourette syndrome, but pure obsessive-compulsive disorder (OCD) (as defined by the DSM-5) may be less common. (12)(41) Differentiating between tics and OCBs can be difficult due to overlapping symptoms. (42) Both tics and compulsions are frequently associated with an uncomfortable sensation that precedes the behavior. The premonitory urge associated with tics is typically a physical sensation or desire. In contrast, the sensation preceding compulsions is typically driven by anxiety, a sense of inner discomfort, or a feeling that something bad may happen if the behavior is not performed correctly. (42) Another distinguishing feature is that patients with tic disorders rarely have contamination-related obsessions (such as an extreme fear of germs with resultant excess hand-washing behavior). An intermediate phenotype between tic disorders and true OCD has been defined as Tourettic OCD. (42)(43) Differentiating between tics, pure OCD, and Tourettic OCD is especially important because the latter is less likely to respond to medical and behavioral therapies solely targeted at either tics or OCD individually. (42)Other common comorbidities in patients with tic disorders include attention-deficit/hyperactivity disorder (54% of all children, 59% of males), anxiety disorders (36% of all children, 48% of females), mood disorders (30% of all children, 39% of females), and disruptive behaviors (30% of all children). (12) Sudden and explosive outbursts of anger, termed rage attacks, occur in up to 58% of children with Tourette syndrome. Rage attacks, when present, can be the most impairing symptom in up to two-thirds of children with tics. (12)(44)(45)(46) Other commonly occurring comorbidities include impulse control issues, self-injurious behavior, suicidality, sleep disorders, academic problems, and sensory-processing issues. (47)(48)(49)(50)(51)The etiology of tics is believed to depend on a complex interplay involving genetics and environmental interactions. In his case series, Gilles de la Tourette noted that tics tended to have a hereditary predisposition. (7)(8) Studies have subsequently shown that monozygotic twins have a higher concordance rate than dizygotic twins (77%-90% versus 23%), and individuals with closer genetic links (first-degree relatives versus second-degree versus third-degree) have a higher risk of developing tics. (52)(53) Nevertheless, although multiple international genome-wide studies have identified several susceptibility genes and loci, to date, no individual causative gene has been linked. (13)(54)Complex and integrated cortical-basal ganglia-thalamo-cortical circuits have been strongly implicated in the pathogenesis of tic disorders and their comorbidities. (13) Anatomic and functional imaging studies, as well as data obtained in animal models, support dysregulation in several cortical and basal ganglia circuits. Deficiencies in various brain regions contribute to the development of tic-related psychiatric comorbidities, and insular dysregulation is believed to be partially responsible for generation of the premonitory urge. (55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65)(66) Although many investigators frequently focus on specific pathways (eg, direct and indirect, habitual and goal-directed, inhibitory, limbic, etc), scientific advances continue to demonstrate an expanding complexity of interactive brain circuits. Furthermore, although the improvement of tics in patients receiving typical and atypical antipsychotics supports a role for dopamine, it should be recognized that multiple neurotransmitter systems are involved in the transmission of messages through interconnected brain circuits. (67)(68)Two controversial disorders have attributed the onset of tics to autoimmune mechanisms. Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS), initially described in a case series in 1998, included prepubertal children with the sudden, fulminant onset and recurrence of tics and/or OCD symptoms in association with group A β-hemolytic streptococcal (GABHS) infections (Table 3). (69) Its proposed autoimmune mechanism includes streptococcal-induced polyreactive antibodies, which, via molecular mimicry, recognize and disrupt either neuronal extracellular or intracellular antigens similar to the mechanism in Sydenham chorea. (69)(71)Numerous concerns exist regarding the existence of a PANDAS diagnosis. For example, studies have shown that children who have been previously diagnosed as having PANDAS frequently do not meet the established diagnostic criteria (Table 3). (72) Recent well-designed, prospective, controlled, longitudinal studies have failed to confirm either a temporal association between GABHS infection and the development of new tics or an infection-associated dramatic exacerbation of an underlying tic disorder. (73)(74)(75)(76)(77)(78)(79)(80)(81)(82)(83)(84) Furthermore, GABHS infection testing, using either throat cultures or antistreptococcal antibody titers, has potential diagnostic flaws. For example, 1) a positive throat culture can identify a benign carrier state for the bacterium and may remain positive for nearly a year in 20% of children and 2) serum antistreptococcal antibodies merely demonstrate that a patient was exposed at some point without signifying when such infections occurred. There are also significant concerns regarding the proposed autoimmune molecular mimicry hypothesis for PANDAS. For example, 1) studies investigating a causative link between autoantibodies and disease have had inconsistent findings (85)(86)(87)(88)(89) and 2) a commercially available diagnostic test for these antibodies has been shown to have high variability in sensitivity and specificity, a negative predictive value, and poor test-retest accuracy. (90)(91)Several treatment options have been suggested for PANDAS, including antimicrobial and immunomodulatory treatments, with the latter ranging from 'relatively' benign treatments (corticosteroids and intravenous immunoglobulin) to high-risk treatments (rituximab, mycophenolate, and plasmapheresis). (92)(93) Although some studies reported clinical improvement, most were scientifically flawed by either a failure to demonstrate statistically significant improvement (94)(95) or methodological problems (small sample size, absence of a control population, or other). (96)(97)(98)(99)(100)(101)(102)Several years after PANDAS was proposed, the criteria were modified to create pediatric acute-onset neuropsychiatric syndrome (PANS) in an effort to broaden acute-onset neuropsychiatric symptoms to include cases not associated with GABHS infection. (70) Proposed criteria are presented in Table 3. Several important distinctions exist between the proposed criteria for PANDAS and PANS, including 1) the presence of tics is not included in the primary diagnostic criteria, with the latter requiring an 'abrupt and dramatic onset of OCD or severely restricted food intake'; 2) the presence of 'sensory or motor abnormalities' such as tics is 1 of 7 potential supporting criteria (of which 2 must be met) (70); and 3) a PANS diagnosis does not require a relapsing course and could instead be diagnosed at onset of symptoms. (102) If abrupt and severe OCD and/or restricted food intact has not occurred, a diagnosis of PANS cannot be made, but a diagnosis of PANS could be made exclusively in the setting of psychiatric symptoms. (70)(102) It is essential to note that, unlike PANDAS, no specific etiological agent has been proposed for PANS, although an autoimmune pathology is frequently suggested.Several scales exist to rate the severity of tic disorders and their related symptoms, and 5 have been 'recommended.' (103) The Yale Global Tic Severity Scale (YGTSS) is the most commonly used rating scale and has been recently revalidated. (104) It contains a scale for determining tic severity (Total Tic Score) based on the number, frequency, intensity, complexity, and interference of the tics (subdivided into motor tic and vocal tic components). In addition, there is a separate measurement of the impact of the patient's tics on overall quality of life. (103) The YGTSS has demonstrated clinical utility in defining meaningful response to treatment and can be used to guide treatment decisions. (105)(106) The Tourette's Disorder Scale assesses comorbid psychiatric symptoms, and the Premonitory Urge for Tics Scale specifically evaluates premonitory urges. The 2 other 'recommended' scales (Shapiro Tourette Syndrome Severity Scale and Tourette Syndrome-Clinical Global Impression) are quicker to administer but less comprehensive compared with the YGTSS. (103)The American Academy of Neurology (AAN) and the European Society for the Study of Tourette Syndrome recently updated their recommendations for the treatment of tic disorders. (1)(2)(107)(108)(109) Both groups highlight the multidimensional factors involved in treating these patients, including the variability of the tics, their psychosocial and physical impact, the presence and impact of comorbid psychiatric disorders, and effects on quality of life. All of these factors are important to consider when deciding how to treat a patient with a tic disorder.Given the waxing and waning pattern of tics, as well as the overall tendency toward long-term improvement, 'watchful waiting' is appropriate when tics are not causing an impairment (eg, psychosocial problem; physical discomfort; disruption at home, school, or work; or affecting other activities). (1) It is especially important to address comorbid neuropsychiatric issues, recognizing that they can affect one's quality of life, even in the presence of mild tic-related impairment. (110)(111)When an impairment of any type exists, available treatments include behavioral, medical, and/or procedural therapies and should be tailored to the child's individual needs (Fig). Referral to a subspecialist should be considered based on the primary physician's knowledge, comfort, and experience dealing with the existing problem(s).The use of complementary and alternative medication (CAM) is common in patients with tics, with up to 87% of patients and families reporting having tried at least 1 type of CAM. (112)(113)(114)(115) Commonly used agents include vitamin D, vitamin A, pyridoxine, magnesium, l-theanine, N-acetylcysteine, omega-3 fatty acids, gluten-free diet, and traditional Chinese medicine. Although many individuals report benefit, evidence for CAM-related benefit on tics is limited. (112)(113)(114)(115) Thus, because efficacy data for each of these treatments is either limited, equivocal, or without evidence for tic suppression, (109)(116)(117)(118)(119)(120)(121)(122)(123)(124) there is no scientific evidence to support their use.Behavioral therapies have been recommended as first-line treatment for tics by major medical societies. Although several behavioral therapies exist, Comprehensive Behavioral Intervention for Tics (CBIT) has the greatest evidence supporting its efficacy in reducing tics in both children and adults. (22)(125) CBIT consists of several components, including awareness training, competing response training, relaxation training, functional-based interventions, and social support (Table 4). (127) Awareness training teaches the patient to be aware of the premonitory urge associated with tics. Competing response training focuses on having the patient perform a voluntary action that physically competes with the tic. A hierarchy of tics from most disabling to least disabling is initially created, with tics subsequently treated in that order. Social support encourages continued use of the techniques.Meta-analyses have confirmed the effectiveness of CBIT. (128)(129) It is recommended with 'high confidence' by the AAN and is considered first-line therapy by the European Society for the Study of Tourette Syndrome. (1)(107)(109) Unfortunately, access to CBIT is often limited due to the scarcity and location of trained providers. Newer delivery methods for behavioral therapies (including CBIT and exposure-response prevention) have included group outpatient visits, videoconferencing/telehealth, self-directed and parent-guided DVD-based therapy, and Internet-based platforms, most with proven efficacy. (130)(131)(132)(133)(134)Exposure-response prevention decreases tics by increasing the tolerance to the exposures and premonitory urge. It targets all the patient's tics simultaneously by enhancing the patient's ability to suppress their tics. More specifically, therapy consists of a patient voluntarily suppressing all their tics during increased exposures to triggers and the premonitory urge. Although CBIT is typically considered the first-line behavioral therapy, exposure-response prevention has been used with good effect. CBIT, exposure-response prevention, and cognitive behavioral therapy (not to be confused with CBIT) are frequently used in combination for patients with significant OCBs. (42)(107)(109)Pharmacotherapy should be considered for tic suppression in patients who do not respond to behavioral therapies, who lack access to behavioral therapies, or who have significant psychosocial and/or physical issues secondary to their tics. (108) Several classes of medications are available to treat tics, and the choice of the initial agent is typically based on the severity of the clinical impairment of the tics balanced against the potential for adverse effects (Table 5). The presence or absence of various psychiatric comorbidities should also be considered when choosing a medication. (109)α-Agonists (clonidine and guanfacine) are frequently used as first-line (Tier 1) agents, especially in children whose tics are relatively mild or in those with comorbid attention-deficit/hyperactivity disorder. (1)(109) Common adverse effects of α-agonists include sedation, hypotension, dry mouth, headache, and bradycardia. Clonidine may be more sedating than guanfacine and so may be beneficial in children with comorbid sleep issues. (135) Clonidine is also available as a transdermal patch, which has shown benefit compared with placebo patch. (136) Although studies are promising for equivalency with oral administration, randomized controlled trials are lacking. The evidence for guanfacine's effectiveness in tic reduction may be less robust, and a randomized controlled trial of extended-release guanfacine showed no improvement compared with placebo. (137)(138) Thus, although both clonidine and guanfacine can be used in the treatment of tics, clonidine has a higher confidence level in the AAN's recent recommendations. (1)(109) Nevertheless, both clonidine and guanfacine are used by experts around the world. Topiramate, an antiseizure medication, has good efficacy in tic reduction with a favorable adverse effect profile compared with antipsychotics. In general, topiramate is commonly used as an agent in patients who have failed α-agonist trials before escalating to antipsychotic therapy. Common adverse effects of topiramate include decreased appetite and weight loss, cognitive and language difficulties, and, rarely, nephrolithiasis. (139)Second-tier medications are primarily typical (dopamine receptor antagonists) and atypical (dopamine and serotonin receptor antagonists) antipsychotics. Currently, haloperidol, pimozide, and aripiprazole are the only US Food and Drug Administration (FDA)-approved medications for the treatment of tic disorders; haloperidol is also approved for Tourette syndrome in Europe. (108) In addition to these agents, other typical and atypical antipsychotics have shown efficacy for treating tics, and the agent is frequently chosen based on adverse effect profiles and the prescriber's previous experiences.Haloperidol, a dopamine D2 receptor (D2R) antagonist, was the first medication with proven high efficacy in treating tic disorders. (140) Its use, however, has declined over the years due to a high incidence of adverse effects, including extrapyrami...

Journals & PublicationsTourette Syndrome

https://publications.aap.org/pediatricsinreview/article/45/2/85/196473/Tourette-Syndrome-and-Tic-Disorders

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