Coprophenomena Associated With Worse Individual and Family Function for Youth With Tourette Syndrome: A Cross-Sectional Study.

Background and Objectives: Tourette syndrome (TS) is defined by multiple motor tics and one or more phonic tics with a symptom duration of >1 year. Coprophenomena are uncommon tics characterized by obscene sounds, words, or gestures. Youth with TS commonly have psychiatric co-occurring conditions such as attention-deficit hyperactivity disorder or obsessive-compulsive disorder and have reported lower scores on measures of individual and family functioning than youth without TS. This study aimed to determine associations among co-occurring condition symptoms, tic severity, and function in youth with TS and coprophenomena compared with those with TS without coprophenomena. Methods: Data were collected through a multicenter, cross-sectional study. Youth with TS were recruited from 2 referral centers, and data were collected from youth and their parents or caregivers. Tic severity was assessed using the Yale Global Tic Severity Scale, and individual function was measured with the Children's Global Assessment Scale. Family impact was measured using the Family Impact Module in domains of parent health-related quality of life (HRQOL), family functioning, and total family impact. We compared individual and family function in youth with TS with coprophenomena (TS+copro) and without coprophenomena (TS-copro). Wilcoxon rank-sum tests were used to compare scores on individual function and family function measures. Results: Of 169 participants, 17 (10.1%) reported coprophenomena. Participants with TS and coprophenomena had higher tic severity scores than those without coprophenomena (TS+copro mean = 36.9, TS-copro = 20.8). Youth with coprophenomena had lower scores for global function (TS+copro median = 51, TS-copro = 60), family functioning (TS+copro = 43.8, TS-copro = 59.4), parent HRQOL (TS+copro = 57, TS-copro = 72), and total family QOL (TS+copro = 50.7, TS-copro = 65.3). Discussion: Youth with TS and coprophenomena had lower individual function, family function, and parent HRQOL than youth without coprophenomena. Coprophenomena presence may indicate that youth have a more severe phenotype of TS, and youth with copropheneomena may benefit from additional caregiver or family supports.

Performance of a Tic Screening Tool (MOVeIT) in Comparison to Expert Clinician Assessment in a Developmental-Behavioral Pediatrics Clinic Sample.

Youth with intellectual and developmental disabilities typically have higher rates of tics and stereotypies compared to children with otherwise typical development. Differentiating between these two pediatric movement disorders can be challenging due to overlapping clinical features, but is relevant due to distinct treatment modalities. The current study evaluated sensitivity and specificity of a tic screening measure, the Motor or Vocal Inventory of Tics (MOVeIT) in a pediatric sample enriched for stereotypy and tics. Children (n=199, age 2-15 years old) receiving care in a developmental-behavioral pediatrics clinic underwent a gold-standard diagnostic assessment by a tic expert; these evaluations were compared to the MOVeIT. The MOVeIT demonstrated good sensitivity (89.8%) and relatively lower specificity (57.1%) compared to tic expert for detecting tics in the overall sample. Specificity of the MOVeIT to identify tics improved to 75% when excluding children with co-occurring stereotypy. For children with tics and co-occurring stereotypy, sensitivity remained high (91.9%) but specificity was low (39.1%). The area under the curve (AUC) value to detect tics on the MOVeIT compared to the tic expert gold standard was significantly higher for children without stereotypy (AUC=85.7%) than those with stereotypy (AUC=64.3%, p <0.01). Overall, the ability to detect tics was better in those without co-occurring stereotypy symptoms. Further work is needed to establish the utility of the MOVeIT in populations where there is a high likelihood of co-occurring tics and stereotypy and in general population settings. Accurate distinction between tics and stereotypy will guide choices for intervention and anticipatory guidance for families.

Diagnosis and treatment of attention deficit hyperactivity disorder.

Attention-deficit hyperactivity disorder (ADHD) is a neurobehavioral disorder characterized by signs and symptoms of inattention, hyperactivity, and impulsivity that typically begin in childhood. ADHD can persist into adulthood, causing impairments in occupational performance and peer and family relationships. This article reviews the epidemiology, diagnosis, and treatment of ADHD.

Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease): A Pilot Study of Feasibility and Reliability.

Remote technology provides an opportunity to extend the reach of clinical care and research for pediatric rare disease. This pilot study evaluated the feasibility and reliability of neuropsychological evaluation, using remote audiovisual technology, in the assessment of children with juvenile Batten disease. Three children with Batten disease and 1 healthy sibling completed a standardized cognitive assessment. Results indicated high agreement between an in-person and a remote evaluator when comparing the subjects' cognitive test scores. This initial test of remote cognitive assessment suggests it is feasible and reliable in children with pediatric neurodegenerative disease, for whom disease burden may limit travel and access to expert care and/or clinical trials.

Pilot Testing Behavior Therapy for Chronic Tic Disorders in Neurology and Developmental Pediatrics Clinics.

Comprehensive Behavioral Intervention for Tics (CBIT) is an efficacious treatment with limited regional availability. As neurology and pediatric clinics are often the first point of therapeutic contact for individuals with tics, the present study assessed preliminary treatment response, acceptability, and feasibility of an abbreviated version, modified for child neurology and developmental pediatrics clinics. Fourteen youth (9-17) with Tourette disorder across 2 child neurology clinics and one developmental pediatrics clinic participated in a small case series. Clinician-rated tic severity (Yale Global Tic Severity Scale) decreased from pre- to posttreatment, z = -2.0, P < .05, r = -.48, as did tic-related impairment, z = -2.4, P < .05, r = -.57. Five of the 9 completers (56%) were classified as treatment responders. Satisfaction ratings were high, and therapeutic alliance ratings were moderately high. Results provide guidance for refinement of this modified CBIT protocol.

Utility of the diagnostic interview schedule for children for assessing Tourette syndrome in children.

OBJECTIVE: The Diagnostic Interview Schedule for Children IV (DISC) has been used extensively in research and screening. Despite wide use, little information exists on the validity of the DISC for diagnosing tic disorders. METHODS: Participants were 181 youth with expert clinician-diagnosed Tourette syndrome (TS). RESULTS: Using expert clinician-diagnosed TS as the gold standard, the sensitivity of the DISC-Y (youth, 0.27) and DISC-P (parent, 0.44) was poor. The DISC-Y identified 29.7% of youth with diagnosed TS whereas the DISC-P identified 47.4% of cases. Only 54% of cases of TS were detected by either the DISC-Y or -P. Diagnostic agreement between the DISC and expert clinician diagnosis was poor. The DISC-Y/P results did not differ as a function of tic severity. CONCLUSIONS: Despite utility for assessing child psychiatric disorders, the sensitivity of the DISC for detecting TS appears poor. This study suggests that DISC has low agreement with expert clinician diagnosis of TS. Findings highlight the need for modification of the DISC and/or the identification and development of more sensitive measures for TS screening.