Treating Tourette Syndrome: A Guide for Practitioners

All three editors of this book are prominent clinicians and researchers in the area of Tourette Syndrome (TS). The purpose of the book is to inform practitioners on comprehensive treatment of patients with TS. Each chapter of the book offers something unique, and though all of them could be pulled out and used separately, the authors recommend an integrated treatment approach afforded by the use of the whole book.

In the Forward, Dr. Peter Hollenbeck describes the progress since the description of TS by Gilles de la Tourette. He highlights the relatively recent development of Habit Reversal Therapy (HRT). HRT arose after decades of studies of the basal ganglia, which showed that the basal ganglia encode motor sequences, and that they can change and modify their circuitry in response to appropriate types of conditioning. In HRT, the therapist elicits in fine detail from the patient the premonitory urges, the circumstances around which the tics arise, and the cues warning the patient that the tics are coming. The patient is then instructed to develop and practice a competing response, usually an action opposite to, or incompatible with, the tic. This usually significantly reduces tic severity and functional impairment.

In chapter one, the editors report on the evolution in thinking about Tourette Syndrome, starting with Gilles de la Tourette’s neurological and genetic view, to a psychodynamic view, and, as of the late 1960’s, a medical view. As of 1999, the emphasis has been on an integrated biological, psychological and social approach. Based on this integrated conceptual model of TS, the Tourette Syndrome Association formed the Behavioural Sciences Consortium (BSC) in 2002.

Chapter two, Characteristics of Tourette Syndrome, mentions that Gilles de la Tourette’s description of TS was accurate. We now see the disorder as a relatively common neurobehavioural disorder. A clinical description of tics is given, followed by the differential diagnosis of tic disorders, as well as the diagnostic criteria for the disorders. A brief summary of the course and prognosis, epidemiology, phenomenology and comorbidity follow. The chapter ends with a brief explanation of current explanatory models, from a neurological and environmental perspective.

Chapter three covers the differential diagnosis of tics and other movement disorders; assessment of tic severity; premonitory sensations; and assessment of current functioning. Movement disorders which may resemble tics include myoclonus, dystonia, Sydenham or Huntington Chorea and restless leg syndrome. Practitioners not familiar with these disorders are advised to obtain a neurological consultation. A medical evaluation may help elucidate the cause of eye blinking, sniffing and throat clearing. Stereotypic movement disorders are usually single, do not wax and wane, and do not vary in anatomical location. If there is no presence nor history of simple tics of the head, and if the patient has a development disorder, then the disorder is more of a stereotypic nature. Tic severity includes factors such frequency, discomfort, pain, complexity, intensity and noticability. The Yale Global Tic Severity Scale (Leckman et al. 1989) is useful in assessing severity. Self-reporting scales, such as the Yale Tourette Syndrome List- Revised (Cohen, Detlor, Young, & Shaywitz, 1980), and parent reporting scales (the authors’ questionnaire is included in Appendix 3.1) as well as direct observation and video recording are also useful. The latter can be deceiving because tics may be suppressed during interviews. Premonitory sensations can be assessed with the Premonitory Urges for Tics Scale (included in Appendix 3.3).

Chapter four describes the assessment of co-occurring psychiatric disorders, e.g. Attention Deficit Hyperactivity Disorder (ADHD), Obsessive-Compulsive Disorder (OCD), anxiety disorders and pervasive developmental disorders.

Chapter five elaborates on the genetic and neurobiological bases for TS. Genetic studies remain inconclusive; this could be caused by failure to include epigenetic factors. Neurological studies support the concept of an abnormality in the corticostriatal circuitry.

Chapter six focuses on neurocognitive deficits in TS. There appears to be a consensus that Verbal IQ is significantly greater than the Performance IQ in many patients with TS, suggestive of problems in visuospatial, perceptual, and motor abilities. Studies also suggest difficulties with executive functions; for example, poorer divided attention abilities, problem-solving abilities, and response inhibition. There may be problems with procedural memory. The most consistent finding is visuomotor integration impairment. Fine motor skill dependent on visuoperceptual processes are consistently impaired in patients with TS, regardless of the presence of ADHD. Mild neurocognitive abnormalities are associated with TS from childhood to adulthood. Girls are slower than boys in Letter Word Fluency. Mounting evidence suggests that comorbidities such as ADHD and OCD exacerbate the cognitive impairments found in TS. Deficits in visuomotor integration and response inhibition may be specific to TS. These deficits impact on symptom presentation, such as aggressiveness, impulsiveness, mood disturbances and poor social skills, and on the treatment response. Treatment may be targeted toward the specific deficits.

Part II of the book covers the medical management of TS. Chapter 7 discusses the medical management, which consists of treatment of the most impairing conditions. Tics are very apparent and can distract from important comorbidities, especially internalizing ones. Tics were traditionally treated with typical neuroleptics, like haloperidol, pimozide or fluphenazine, though these medications are gradually being replaced by the atypical neuroleptics, like risperidone, olanzapine, ziprasidone, quetiapine, and aripiprazole, The use of other dopamine-modulating agents (pergolide, sulpiride, tiapride, tetrabenazine, levodopa, talipexole, rapinirole, metoclopromide), as well as alpha-adrenergic agents (clonidine and guanfacine) are discussed. Other tic suppression agents (baclofen, nicotine, mecamylamine and delta-9-tetrahydrocannibinol) are seen as not promising. The uses of penicillin and intravenous immunoglobulin in infection and in autoimmune-based treatments have not been proven effective. The authors then discuss the treatment of associated comorbid disorders —ADHD and OCD — and end with the discussion of treatment refractory cases and of complex cases.

Chapter eight discusses the psychosocial treatment of tics and of intentional repetitive behaviors associated with TS: contingency management, function-based interventions (examines under which circumstances the tics arise), massed practice (consists in voluntarily rapidly producing the tics), relaxation training, hypnosis, self-monitoring, exposure with response prevention, and habit reversal. Particular attention was given to the latter. Medications rarely eliminate tics, and they have side effects which result in poor compliance; therefore the treatments discussed in this chapter can be useful.

Chapter nine discusses the management of comorbid internalizing disorders in TS; for example, OCD, anxiety disorder, depressive disorder, and the use of cognitive behavioral therapy for these disorders.

Chapter ten discusses the assessment, phenomenology and treatment (psychosocial and medical) of disruptive behaviour in patients with TS. Anger and non-compliance are a large part of this problem. The chapter reported on a large number of studies dealing with the issues. It is unclear whether tic severity contributes to difficult behaviour. The presence of ADHD worsens it.

Part three, Clinical Management of Secondary Problems, chapters eleven, twelve and thirteen deal with family issues, school issues, and social and occupational difficulties of persons with TS, and discusses when and how to intervene.

I enjoyed reading this book. It is thorough, and it gave a very good up to date review and critique of the literature on every aspect of TS. It is written in an authoritative manner, giving up to date methods for assessment and treatment of every aspect of this disorder and of its complications. I highly recommend it for all practitioners who deal with patients who suffer from TS.