11. Shah AB, Chernov I, Zhang HT, et al. Identification and analysis of mutations in the Wilson disease gene (ATP7B): population frequencies, genotype-phenotype correlation, and functional ana- lyzes. Am J Hum Genet 1997;61:317–328. 12. Deguti MM, Genschel J, Cancado EL, et al. Wilson disease: novel mutations in the ATP7B gene and clinical correlation in Brazilian patients. Hum Mutat 2004;23:398. 13. Ravin HA. An improved colorimetric assay of ceruloplasmin. J Lab Clin Med 1961;61:161–168. 14. Członkowska A, Galewicz A, Rodo M, et al. Observations on copper metabolism in Wilson’s disease. Acta Univ Carol 1973;55– 57:175–177. 15. Tsivkovskii R, Efremov RG, Lutsenko S. The role of the invariant His-1069 in folding and function of the Wilson’s disease protein, the human copper-transporting ATPase ATP7B. J Biol Chem 2003;278:13302–13308. Gilles de la Tourette Syndrome: Patient’s Knowledge and Concern of Adverse Effects Katie Kompoliti, MD, * Christopher G. Goetz, MD, Mary Morrissey, ScD, and Sue Leurgans, PhD Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois, USA Abstract: The objective of this study was to assess aware- ness and concern of neuroleptic (NL)-induced side effects in Gilles de la Tourette syndrome (GTS) patients. Al- though NLs are effective tic suppressants, they can be associated with various side effects. Data on patient knowledge and concern about side effects can guide ed- ucational efforts. One hundred consecutive GTS patients or parents in a tertiary referral medical center re- sponded to a standardized, in-person questionnaire. They were given a list of 15 side effects and asked which could be ascribed to NLs (9) or not (6). Side effect concern was rated on a 0 (none) to 10 (extreme) scale. The mean age for the 100 patients was 19.4  14 years; 55 had a history of NL use, and 45 were NL-naive. Less than half the cohort met criteria for being well informed. Only one third of the listed NL side effects were accu- rately identified by at least 75% of the respondents. Patients with past or current NL treatment were more accurate in identifying NL side effects but less concerned about them than NL-naive patients. The side effects of greatest concern were seizures, tardive dyskinesia, think- ing and emotion disturbances, and cardiac irregularities. Overall, patient awareness of NL side effects is insuffi- cient, and although past exposure to NLs enhances knowledge, it decreases concern. © 2005 Movement Dis- order Society Key words: tics; dopamine blockers; tardive dyskinesia; neuroleptics; toxicity Gilles de la Tourette syndrome (GTS) is a childhood- onset disorder characterized by motor and vocal tics. 1 Both the traditional high potency 2 and the newer atypical neuroleptics (NLs) 3,4 are efficacious for the treatment of tics. Despite efficacy, a very high proportion of patients eventually discontinue NL therapy because of side ef- fects. 5 In the GTS population, there are no published reports investigating the patient’s or guardian’s knowledge and concern about potential NL side effects. We hypothe- sized that in a university-based tertiary care medical practice, overall knowledge would be high and concern about medication side effects would be based on accurate information. Furthermore, we considered that patients never exposed to NLs would be less informed and also less concerned about side effects than subjects with cur- rent or past NL treatment. With the overall aim of de- signing pertinent educational programs for tic patients, we conducted a questionnaire study to investigate these hypotheses in a consecutive series of outpatients with GTS. STUDY DESIGN Demographics A questionnaire study was administered in person on consecutive patients with GTS seen in the Movement Disorder Center of Rush University Medical Center. The project was approved by the Institutional Review Board. The data were collected from the guardians for patients under 18 years of age and from the patients themselves if they were over 18. The data included age, gender, age at disease onset, comorbid conditions (attention deficit hy- peractivity disorder, obsessive– compulsive disorder, pervasive developmental disorder, learning disability, af- fective disorders and psychosis), exposure to NLs cur- rently (over the past month before the interview) or in the past (more than a month before the interview), current tic medications, tic medications used in the past, and other current medications. The comorbid conditions were de- fined according to DSM IV criteria. 1 The patient or guardian was asked to define whether motor or vocal tics were primarily the source of disability and to rate tic severity on a 0 (none) to 10 (worst) global severity *Correspondence to: Dr. Katie Kompoliti, Department of Neurolog- ical Sciences, Section of Movement Disorders, 1725 W. Harrison Street, Suite 755, Chicago, IL 60612. E-mail: kkompoli@rush.edu Received 3 February 2005; Revised 22 April 2005; Accepted 5 May 2005 Published online 13 September 2005 in Wiley InterScience (www. interscience.wiley.com). DOI: 10.1002/mds.20680 248 K. KOMPOLITI ET AL. Movement Disorders, Vol. 21, No. 2, 2006