This is the second installment of a double issue on Tourette syndrome (TS) for CNS Spectrums. This issue highlights recent research findings in the field and provides a state-of-the art update on the relationship between TS and obsessive-compulsive disorder (OCD), as well as on the genetics, functional imaging and neurocircuitry, and neuropathology of TS.

I would again like to express my appreciation to the guest editor of this superb issue—Neal Swerdlow, MD, PhD, associate professor of psychiatry at University of California, San Diego, and scientific director of the Tourette Syndrome Association (TSA)—as well as Sue Levi-Pearl of the TSA.

The issue begins with a first-person account of TS by Major League baseball player Jim Eisenreich. His .300 lifetime batting average in professional baseball is a reflection of the superior focus he achieves in sports. In his work as a spokesman on behalf of TS, he serves as a role model, and his work will certainly help to raise awareness about TS so that others may avoid his experience of a 17-year gap between onset of symptoms and correct diagnosis.

Seventeen years after professional baseball player Jim Eisenreich first developed symptoms of Tourette syndrome (TS), the correct diagnosis was finally made.

At age 6, his rapid eye blinking led to a diagnosis of hyperactivity. “I was told I would grow out of it,” says the 39-year-old Eisenreich, who may have played his last game as a Los Angeles Dodger. “I knew I was different even then.”

In the early 1980s, Eisenreich's condition first became public. Since then, his achievements as a professional athlete have made him a role model for other TS patients.

Sports were always a haven for him as he grew up. “I found peace, comfort, and security in sports. Whatever the season, I played the sport—football, baseball, hockey,” says Eisenreich. “Socially, I didn't go to the movies or go out much with girls.”

After 2 years in the minor leagues, he was called up by the Minnesota Twins in 1982 as an outfielder. During the season, a TS specialist recognized his grunting and sniffling as signs of the disease. “I had no idea what the specialist was talking about, and the Twins doctors dismissed it because I didn't have copralalia,” he says. The Twins team physician (an internist) prescribed a sedative. Then Eisenreich tried Inderal, which caused hyperventilation, and Catapres, which caused depression.

There is clear overlap in the clinical symptoms of obsessive-compulsive disorder (OCD) and Tourette syndrome (TS). As a result, OCD (with or without tics) and TS (with or without obsessive-compulsive symptoms [OCS]) have been conceptualized to form a disorder spectrum—an overlapping set of phenotypes reflecting presumed commonality at the level of the underlying genetics and neuropathology. We identified the characteristics of a research sample of patients with OCD or TS, based on information obtained in semistructured clinical interviews, to examine the similarities and differences in the clinical symptoms across this spectrum. This sample conformed to known age-of-onset and sex distribution patterns for OCD and TS. Previously reported patterns of predominant aggressive and sexual obsessions and touching compulsions were observed in subjects with tic-related OCD, compared with non—tic-related OCD (ie, OCD alone). The majority of patients with tic-related OCD experienced horrifically violent obsessions that were less common in OCD alone and much less common in TS. Nonetheless, symptomatic and functional impairment in TS subjects was clearly related to the intensity of their OCS. The specific obsessions and compulsions associated with clinical impairment in TS differed from those associated with impairment in OCD. These results suggest that, despite the many overlapping dimensions of these disorders, the symptoms and associated impairment in “pure” OCD, tic-related OCD, and TS do not form a simple continuous spectrum.

The familial nature of Tourette syndrome (TS) was first described by Georges Gilles de la Tourette in 1885. Formal investigations of TS since the 1970s have consistently demonstrated an increased risk for TS among relatives of patients with TS. Family studies, twin studies, and segregation analyses all indicate that TS has significant genetic determinants. The mode of transmission, while mildly controversial, is generally believed to be due to at least one major locus inherited either as an autosomal dominant trait with reduced penetrance, or as a trait with intermediate inheritance in which some heterozygotic persons manifest the disorder. There is evidence for a TS spectrum of symptoms that includes chronic tics and obsessive-compulsive disorder. Systematic genome linkage studies of TS are progressing. To date, however, no significant linkage findings exist, although the search has included many neurologically relevant candidate genes.

CNS Spectrums is a peer-reviewed journal that publishes original scientific literature and reviews on a wide variety of neuroscientific topics of interest to the clinician. CNS Spectrums publishes 12 issues yearly. As the immense prevalence of comorbid diseases among patients seen by psychiatrists and neurologists increases, these physicians will jointly diagnose and treat the neuropsychiatrically ill. Our mission is to provide these physicians with an editorial package that will enhance and increase their understanding of neuropsychiatry. To this end, manuscripts that address crossover issues germane to neurology and psychiatry will be given immediate priority.

Tourette syndrome (TS) is a complex neurobehavioral disorder principally characterized by motor and vocal tics. However, features of obsessive-compulsive and attention-deficit/hyperactivity disorders are often present. The basal ganglia and associated brain structures have been implicated in the pathophysiology of TS, as well as in these related conditions. Specifically, it is believed that the neuroanatomically and functionally defined basal ganglia thalamocortical loops are involved in TS. These loops are composed of a sequence of connections originating in the cortex and passing in series through the striatum (caudate and putamen), globus pallidus, and thalamus before returning to the cortical areas of origin. This review concentrates on the neuroimaging findings in Ts, particularly as they relate to alterations in components of the basal ganglia thalamocortical circuits. These neuroimaging data suggest that the major abnormalities in TS involve striatal or cortical dysfunction, as well as dysfunction of dopaminergic systems that regulate basas ganglia neurotransmission.

The unique clinical presentation of Tourette syndrome (TS) and its symptomatic response to dopamine antagonists are widely cited as evidence for the central role of the limbic-motor interface in the pathophysiology of TS. Nonetheless, the true neuropathology of TS remains elusive, even though significant advances have been made in understanding complex interconnected circuitries within the limbic system and basal ganglia. Neuropathologic and neuroimaging studies—plagued by small samples, clinical heterogeneity, and a number of interpretative problems—are generally supportive of pathology within the orbitofrontal cortex, striatum, and their efferent projections in TS. The specific patterns of abnormalities vary widely across these studies, clouding attempts to define a unifying neuropathology for this disorder. Converging yet circumstantial evidence for frontal cortical, and basal ganglia pathology in TS comes also from studies infields ranging from neuroimmunology to neuropsychology, and from the clinical overlap between TS and disorders such as obsessive-compulsive disorder, attention-deficit/hyperactivity disorder, and Sydenhams chorea. As a “model” neuropsychiatric disorder, TS has stimulated advances in several areas of neurobiology research, yet we still await a real understanding of its pathophysiology in order to move from empirically driven therapeutics to the development of targeted effective treatments.

The psychosis, intermittent hyponatremia, and polydipsia (PIP) syndrome is a prevalent, medically serious, and difficult to treat condition in psychiatry, especially common in hospitalized chronic schizophrenic patients. In this paper, the pathophysiology and clinical manifestations of the PIP syndrome are presented as an overview, and a program of medical/pharmacologic, nursing, behavioral, and psychiatric/psychological interventions is presented and discussed. The emphasis throughout this paper is on multidisciplinary treatment approaches for patients with PIP syndrome, including medical, psychiatric, psychosocial, behavioral, and nursing contributions to the evaluation and care of these often difficult patients.