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Open AccessArticle

Obsessive Compulsive Symptoms and Psychopathological Profile in Children and Adolescents with KBG Syndrome

1
Department of Neuroscience, Child and Adolescent Psychiatry Unit, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
2
Genetics and Rare Diseases Research Division, Bambino Gesù Children’s Hospital, IRCCS, 00165 Rome, Italy
3
Center for Rare Diseases and Congenital Defects, Fondazione Policlinico Universitario A. Gemelli, IRCCS, 00168 Rome, Italy
4
Istituto di Pediatria, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
5
Fatebenefratelli Foundation for Health Research and Education, San Giovanni Calibita-Fatebenefratelli Hospital, Isola Tiberina, 00186 Rome, Italy
6
Institute of Psychiatry, Fondazione Policlinico Universitario A. Gemelli, Catholic University, 00168 Rome, Italy
*
Author to whom correspondence should be addressed.
These authors contributed equally.
Brain Sci. 2019, 9(11), 313; https://doi.org/10.3390/brainsci9110313
Received: 20 September 2019 / Revised: 29 October 2019 / Accepted: 4 November 2019 / Published: 7 November 2019
(This article belongs to the Section Clinical Neuroscience)
KBG syndrome is a rare multisystem developmental disorder caused by ankyrin repeat domain-containing protein 11 (ANKRD11) gene haploinsufficiency, resulting from either intragenic loss-of-function mutations or microdeletions encompassing the gene. Concerning the behavioral phenotype, a limited amount of research has been focused on attention deficit and hyperactivity disorder, autistic-like features, anxiety and impairments in emotion regulation, and no study has provided a systematic assessment. The aim of the present work is to investigate the psychopathological profile in children, adolescents, and young adults with KBG syndrome. Seventeen subjects with molecularly confirmed diagnoses were evaluated to investigate cognitive abilities and psychopathological features. Parametric and nonparametric indexes were used to describe the patient cohort according to type and distribution of specific measures. The KBG subjects were characterized by a low mean IQ score, with a distribution characterized by a variability similar to that occurring in the general population. Prevalence of neuropsychiatric disorders were computed as well as the corresponding confidence intervals to compare their prevalence to that reported for the general population. The KBG subjects were characterized by higher prevalence of obsessive-compulsive, tic, depressive and attention deficit and hyperactivity disorders. Obsessive-compulsive disorder is a peculiar aspect characterizing the psychopathological profile of KBG patients, which does not seem to be related to the cognitive level. The present study provides new relevant information towards the definition of a psychopathological phenotype of KBG syndromes useful to plan a better treatment for patients. View Full-Text
Keywords: obsessive compulsive symptoms; developmental disorders; ANKRD11 obsessive compulsive symptoms; developmental disorders; ANKRD11
MDPI and ACS Style

Alfieri, P.; Demaria, F.; Licchelli, S.; Santonastaso, O.; Caciolo, C.; Digilio, M.C.; Sinibaldi, L.; Leoni, C.; Gnazzo, M.; Tartaglia, M.; Pasqualetti, P.; Vicari, S. Obsessive Compulsive Symptoms and Psychopathological Profile in Children and Adolescents with KBG Syndrome. Brain Sci. 2019, 9, 313.

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