Introduction

Persistent (Chronic) Tic Disorders (PTD), including Tourette Syndrome (TS), are developmental neuropsychiatric disorders characterized by multiple motor and vocal tics present for at least one year beginning before the age of 18 years [1]. It is estimated that PTDs affect up to 3% of children [2]. Approximately 90% of individuals with PTD have at least one co-occurring neuropsychiatric condition, such as attention deficit hyperactivity disorder (ADHD), obsessive-compulsive disorder (OCD), or anxiety [3–7]. The clinical spectrum of PTD is heterogeneous. For most individuals with PTD, tics improve through adolescence; however, persistent moderate-to-severe tics or worsening in adulthood can occur [8–11]. PTD can be associated with poorer quality of life (QOL) and interference in daily living [12–14]. Both tic severity and psychiatric comorbidities can lead to psychosocial, functional, and physical difficulties that can disrupt educational attainment, employment, and social interactions [12, 15–18]. As a result, these patients require not only treatment for their tics but also their comorbidities.

Both the American Academy of Neurology (AAN) [19] and the European Society for the Study of Tourette Syndrome (ESSTS) [20–23] have published guidelines regarding the clinical evaluation and management of PTD. Although these guidelines considered cost, feasibility, and acceptability in their recommendations, there is marked diversity in clinical assessment and management geographically, even amongst movement disorder specialists [24]. For example, these guidelines recommend evaluating tic severity with validated scales; however, only 10% of movement disorder clinicians use standardized scales in clinical practice and vary in which scales. Additionally, although both guidelines recommended behavioral therapy as the primary treatment, accessibility to behavioral therapists globally remains challenging.

These barriers to guideline implementation are reported by healthcare professionals, patients, and families alike. The financial coverage of treatments, accessibility to and cost of specialized PTD care, and additional neuropsychiatric symptoms are the most frequently reported barriers to optimal PTD care [24]. Some studies have specifically assessed patients’ and families’ perspectives on the existing care model for tics. A systematic review [25] of these studies found several significant barriers to appropriate care identified by people with tics: difficulty in diagnosing patients with TS; fear of stigmatization leading to delays in seeking treatment; concerns about adverse effects of the commonly used medications; concern for potential rebound effect of behavioral therapy leading to worsening of tics or onset of new tics; financial cost of, the time commitment for, and travel distance to behavioral services. In addition, parents of children with tics would have preferred to have more support to help cope with the diagnosis, including active collaboration with school officials, teachers, and pediatricians, which could lead to reduced barriers to receiving optimal care [25].

There is a need to assess the degree to which these clinical guidelines are being followed, contextualize them within diverse practices, and identify barriers encountered so that strategies can be developed to achieve a standard of care consistently. This article presents the rationale and methodology for a survey of healthcare professionals routinely involved in the clinical care of patients with persistent tic disorders, aimed at identifying existing barriers to the practical implementation of published guidelines into routine clinical care.

Published guidelines

The AAN and the ESSTS have published guidelines regarding the clinical evaluation and management of PTD based on evidence-based literature, randomized controlled trials (RCTs), and expert opinion. Although these guidelines focus on evidence-based treatments through RCTs, there are scientific and ethical limitations to these studies with the PTD population [26]. For example, limited inclusion or recruitment of individuals with PTD and comorbid neuropsychiatric conditions or concomitant medications in RCTs and the heterogeneity of PTD may limit generalizability to the general PTD population. Additionally, there is limited guidance on combination therapies, optimal duration or the dose of treatment, and treatment indications, all necessary considerations for individualized treatment in routine clinical practice.

The 2019 AAN guidelines provided forty-three total recommendations for optimal care of PTD. A multidisciplinary panel performed a systematic review of RCTs [27]. The panel included nine physicians, two psychologists, and two patient representatives with expertise in child and adult neurology, psychiatry, pediatrics, behavioral treatments, and methodology. For all non-evidence-based factors, including value, availability, risk, and cost, the panel assigned levels of obligation to each recommendation and voted independently and anonymously online using a modified Delphi process. Statistical analysis synthesized the responses, and a consensus was obtained in three rounds of voting. Of the forty-three total recommendations, there were twenty-two level A (must), fifteen level B (should), and nine level C (may) recommendations for the assessment and management of people with PTD [19].

The 2021 ESSTS guidelines consist of four articles summarizing recommendations for assessment [22], psychological interventions [23], pharmacological treatment [20], and deep brain stimulation [28] for PTD. In addition, the ESSTS working group updated and summarized evidence-based literature from the original 2011 guidelines. An online survey of clinical practices and expert opinions completed by 59 ESSTS members in 17 European countries was incorporated into the updated guidelines [21].

Despite these guidelines and the high prevalence of PTD, many patients experience a substantial delay in diagnosis and limited access to effective first-line interventions. For example, a US patient survey by the Tourette Association of America (TAA) found that 78% of adult respondents reported more than a 2-year delay in diagnosis from symptom onset. Several factors include high reliance on specialists to make the diagnosis, difficulty in diagnosing patients with TS, or fear of stigmatization leading to delays in seeking treatment [25, 29].

Both guidelines suggest the importance of proper counseling about the natural history of tics, screening for psychiatric comorbidities, psychosocial education, and the importance of behavioral intervention [23, 30]. Both guidelines recommend using Cognitive Behavioral Intervention for Tics (CBIT) as first-line therapy after several high-quality studies showed a beneficial impact on tic disorders [23, 30]. However, only 36% of children and 25% of adults included in the TAA survey had participated in CBIT. In addition, awareness, accessibility to, the financial cost of, the time commitment for, and travel distance to behavioral services as well as skepticism, were commonly cited barriers to trying CBIT [25, 31].

Regarding medications, the AAN practice guidelines include alpha-agonists, particularly for those with comorbid ADHD; antipsychotic medications were recommended by the AAN when the benefits outweighed the risks [30]. In addition, the ESSTS recommended aripiprazole as an effective therapy based on two large-scale randomized controlled trials [20]. medication is commonly prescribed, only 44% of children and 47% of adults reported that their symptoms were adequately controlled [13].

Surgical treatments for refractory PTD are an evolving field. For example, with regards to deep brain stimulation (DBS), ESSTS described this therapy as having limited evidence; AAN recommended the use of a multidisciplinary care model for evaluation to confirm the diagnosis, assess psychiatric comorbidities and ensure at least two standard medication trials have failed to produce adequate benefits [28, 30].

Despite the publication of AAN and ESSTS guidelines, the variability of clinical practices and barriers to guideline implementation impede standardized PTD care, Fig 1. Therefore, the authors propose a survey to evaluate how clinical practices diverge from existing guidelines and identify barriers to guideline implementation in PTD. Understanding these barriers provides the initial steps to evaluating and designing interventions to elevate community care of PTD to the existing guidelines.

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Fig 1. Conceptualized barriers to guideline implementation in persistent tic disorder care.

AAN: American Academy Neurology; ESSTS: European Society for the Study of Tourette Syndrome; PTD: Persistent Tic Disorder.

https://doi.org/10.1371/journal.pone.0288408.g001

Methods

Data management and oversight

All centralized study data are stored and managed using an External REDCap database and related tools hosted at Wake Forest University Health Sciences (WFUHS) on a secure server [44]. Data will be maintained in a study database on REDCap. Access to study data will be available to research personnel.

Discussion

Given the high prevalence of PTD, many patients will not get their care from PTD experts. A significant portion of individuals will receive care from primary doctors, neurologists, and psychiatrists who may have limited PTD expertise. These clinicians face the challenge of limited resources in healthcare communities and patient financial access. This circumstance leads to disparity in care delivered.

The purpose of this survey will be to clarify how current practice differs from the evidence-based AAN and ESSTS guidelines. While the AAN and ESSTS guidelines consider all the practical elements for both physicians and patients in the generation of its recommendations—cost, feasibility, acceptability to patients and expert input, and the real world clear, practical applicability—we do not know empirically the degree to which many of these recommendations are practiced. Practitioner knowledge, available time, and other practical factors likely influence the degree to which these guidelines have been implemented since publication. By discerning how current practice across multiple settings diverges from guideline recommendations, the healthcare community can focus on optimizing education and care to areas where approach diverges from recommendations. This will have widespread implications in focusing the direction of and improving the care provided.

Limitations

There are some inevitable limitations to this approach. Current evidence to guide treatment in PTD is hampered by inconsistencies in outcomes used, doses of medication employed, and limitations in the duration of studies. As a result, physician responders develop their treatment protocols considering the available data and their experience, which may be influenced by local resource availability and other factors. Still, this allows some framework to guide the best treatment within the current limitations. Furthermore, the questionnaire will be able to deduce additional categories that present barriers to care delivery. Still, more work may be needed to reach a granular level of understanding of the factors that interplay and how these may be overcome. We hope that the effort to identify barriers to guideline implementation will result in opportunities for education, advocacy, and strategy development to improve consistency in care delivered from diverse treatment environments. Furthermore, incorporating a broader range of perspectives, including primary care clinicians, general neurologists, patients, and patient advocates, into developing these strategies would provide valuable insight.

Supporting information

Acknowledgments

In addition to the authors, the Movement Disorder Society Tic and Tourette Study Group includes the following members: 1) Mark Hallett, National Institute of Neurological Disorders and Stroke, Bethesda, MD; 2) Andreas Hartmann, Department of Neurology, Hôpital de la Pitié-Salpêtrière, Paris, France; 3) Mariam Hull, Pediatric Movement Disorders Clinic, Section of Pediatric Neurology and Developmental Neuroscience, Texas Children’s Hospital and Baylor College of Medicine, Houston, TX; 4) Andrea Lee, Department of Neurology, University of Kansas Medical Center, Kansas City, KS; 5) Pablo Mir, Unidad de Trastornos del Movimiento Servicio de Neurología y Neurofisiología Clínica, Instituto de Biomedicina de Sevilla Hospital Universitario Virgen del Rocío/Universidad de Sevilla, Seville, Spain; 6) Kirsten Muller-Vahl, Clinic of Psychiatry, Social Psychiatry and Psychotherapy, Hannover Medical School, Carl-Neuberg-Straße, Hannover, Germany; 7) Alexander Muenchau, Institute of Systems Motor Science, University of Lübeck Lübeck Germany; 8) Michael Okun, Department of Neurology, Norman Fixel Institute for Neurological Diseases, Gainesville, FL; 9) Mered Parnes, Pediatric Movement Disorders Clinic, Section of Pediatric Neurology and Developmental Neuroscience, Texas Children’s Hospital and Baylor College of Medicine, Houston, TX; 10) Kallol Set; Department of Pediatric Neurology Dayton Children’s Hospital, Boonshoft School of Medicine, Wright State University Dayton Ohio; 11) David Shprecher; Banner Sun Health Research Institute, Sun City, AZ; 12) Harvey Singer, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, 13) Katarzyna Smilowska, Department of Neurology, Regional Specialist Hospital of St. Barbara in Sosnowiec, Poland; 14) Natalia Szejko, Department of Neurology, Medical University of Warsaw, Warsaw, Poland; 15) Daniel van Wamelen, Department of Neuroimaging, King’s College London Institute of Psychiatry, Psychology & Neuroscience, London, United Kingdom; 16) Yulia Worbe, ICM, Inserm, CNRS, Department of Neurophysiology, Hôpital Saint Antoine (DMU 6), AP-HP, Sorbonne University, Paris, France.