Tics and Tourette Syndrome

Harvey S. Singer, MD, FAAN p. 936-958 August 2019, Vol.25, No.4 doi: 10.1212/CON.0000000000000752
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PURPOSE OF REVIEW: The purpose of this article is to present current information on the phenomenology, epidemiology, comorbidities, and pathophysiology of tic disorders and discuss therapy options. It is hoped that a greater understanding of each of these components will provide clinicians with the necessary information to deliver thoughtful and optimal care to affected individuals.

RECENT FINDINGS: Recent advances include the finding that Tourette syndrome is likely due to a combination of several different genes, both low-effect and larger-effect variants, plus environmental factors. Pathophysiologically, increasing evidence supports involvement of the cortical–basal ganglia–thalamocortical circuit; however, the primary location and neurotransmitter remain controversial. Behavioral therapy is first-line treatment, and pharmacotherapy is based on tic severity. Several newer therapeutic agents are under investigation (eg, valbenazine, deutetrabenazine, cannabinoids), and deep brain stimulation is a promising therapy.

SUMMARY: Tics, defined as sudden, rapid, recurrent, nonrhythmic motor movements or vocalizations, are essential components of Tourette syndrome. Although some tics may be mild, others can cause significant psychosocial, physical, and functional difficulties that affect daily activities. In addition to tics, most affected individuals have coexisting neuropsychological difficulties (attention deficit hyperactivity disorder, obsessive-compulsive disorder, anxiety, mood disorder, disruptive behaviors, schizotypal traits, suicidal behavior, personality disorder, antisocial activities, and sleep disorders) that can further impact social and academic activities or employment.

Address correspondence to Dr Harvey S. Singer, Professor of Neurology and Pediatrics, Johns Hopkins Hospital, Rubenstein Child Health Building, 200 N Wolfe St, Ste 2141 Baltimore, MD 21287, hsinger@jhmi.edu.

RELATIONSHIP DISCLOSURE: Dr Singer serves as a consultant for Abide Therapeutics, Inc; Cello Health BioConsulting; ClearView Healthcare Partners; Teva Pharmaceutical Industries Ltd; and Trinity Partners, LLC. Dr Singer receives publishing royalties from Elsevier and research/grant support from the Tourette Association of America.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Singer discusses the unlabeled/investigational use of baclofen, botulinum toxin, cannabidiol, clonazepam, clonidine, deutetrabenazine, ecopipam, fluphenazine, guanfacine, nabiximols, risperidone, sulpiride, tetrabenazine, Δ-9-tetrahydrocannibinol, tiapride, topiramate, and valbenazine.

© 2019 American Academy of Neurology.