Pharmacological treatment of behavioral disorders in Cornelia de Lange Syndrome. A case report

Downloads

Published

2019-09-30

How to Cite

Fernández-Natal, C., & Carballo, J. J. . (2019). Pharmacological treatment of behavioral disorders in Cornelia de Lange Syndrome. A case report. Revista De Psiquiatría Infanto-Juvenil, 36(3), 32–35. https://doi.org/10.31766/repsij.v36n3a4

Issue

Vol. 36 No. 3 (2019): July-September

Section

Case reports
Crossref
Scopus
Google Scholar
Europe PMC

Authors

DOI:

https://doi.org/10.31766/repsij.v36n3a4

Keywords:

Cornelia de Lange Syndrome, behaviour disorder, drug therapy, risperidone

Abstract

Cornelia de Lange Syndrome (CdLS) is a genetic disorder characterized by different somatic impairments and retarded psychomotor development. 8-year-old male diagnosed with CdLS comes to Children’ Psychiatry assesment presenting behavioural disorder as well as delayed motor and cognitive development. He cannot talk and only pictograms are used for communication. Severe cognitive rigidity, specially to manage changes and new contexts. In these cases, he shows aggressiveness against objects and self-injurious behaviour. A trial test of risperidone is started based on similarities betweeen these behavioral disorders and those present in autism spectrum. He shows a remarkable improvement, decreasing three points in the Clinical Global Impression. Scientific evidence mostly derives from case reports studies. That implies the quality of scientific evidence is low. Though the treatment with risperidone may be effective and well tolerated, more studies are required to move forward on the pharmacological treatment of behavioral disorders in CdLS.

Downloads

Download data is not yet available.

Author Biographies

Cora Fernández-Natal, Hospital Universitario de La Princesa

Departamento de Psiquiatría

Juan J. Carballo, Hospital General Universitario Gregorio Marañón

Servicio de Psiquiatría del Niño y del Adolescente

References

Srivastava S, Landy-Schmitt C, Clarck B et al. Autism traits in children and adolescents with Cornelia de Lange Syndrome. American Journal of Mental Genetics part A. 2014; 164A: 1400-1410. https://doi.org/10.1002/ajmg.a.36573 DOI: https://doi.org/10.1002/ajmg.a.36573

Ajmone P, Rigamonti C, Dall’Ara F et al. Communication, cognitive development and behavior in children with Cornelia de Lange Syndrome (CdLS): Preliminary results. American Journal of Mental Genetics Part B. 2014; 165B: 223-229. https://doi.org/10.1002/ajmg.b.32224 DOI: https://doi.org/10.1002/ajmg.b.32224

Andrisk F, Ollendick TH, Turner SM, Hughes JR. Pharmacologiac treatment of aggresive behavior and emesis in the Cornelia de Lange Syndrome. J Nerv Ment Dis. 1979; 167 (12): 764-6. https://doi.org/10.1097/00005053-197912000-00010 DOI: https://doi.org/10.1097/00005053-197912000-00010

Stevic M, Milojevic I, Bokum Z, Simic D. Unpredictable drug reaction in a child with Cornelia de Lange syndrome. Int J Clin Pharm. 2015; 37: 1-3. https://doi.org/10.1007/s11096-014-0050-7 DOI: https://doi.org/10.1007/s11096-014-0050-7

Sargent WW. Anaesthatic management of a patient with Cornelia de Lange syndrome. Anesthesiology. 1991; 74: 1162-3. DOI: https://doi.org/10.1097/00000542-199106000-00041

Watchel LE, Hagopian LP. Psychopharmacology and applied behavioral analysis: tandem treatment of severe problem behaviors in intellectual disability and a case series. Isr J Psychiatry Relat Sci. 2006; 43(4): 265-274.

Wierzba J, Mazurkiewicz-Beldzinska M, Jablonska-Brudlo J. Challenges of caring for a patient with a rare disease – as demostrated by Cornelia de Lange syndrome. Developmental Period Medicine. 2015; XIX 4: 511-515.

La Malfa, G; Lassi, S; Bertelli M; Castellani A. Reviewing the use of antipsychotic drugs in people with intellectual disability. Hum Psychopharmacol Clin Exp. 2006; 21: 73–89. https://doi.org/10.1002/hup.748 DOI: https://doi.org/10.1002/hup.748

McDougle CJ, Scohill L, Aman MG. Risperidone for the core symptom domains of autism: results from a study by the autism network of the research units on pediatric psychopharmacologie. Am J Psychiatry. 2005; 162:1142–1148 DOI: https://doi.org/10.1176/appi.ajp.162.6.1142

McCracken JT, McGough J, Shah B, Cronin P, Hong D. Risperidone in children with autism and serious behaviorall problems. N Engl J Med. 2002 Aug 1; 347(5): 314-21. https://doi.org/10.1056/NEJMoa013171 DOI: https://doi.org/10.1056/NEJMoa013171

Kline AD, Moss JF, Selicorni A, Bisgnetwork et al. Diagnosis and management of Cornelia de Lange Syndrome: First international consensus statement. Nat Rev Gen. 2018 Jul 11; 19: 649-666. https://doi.org/10.1038/s41576-018-0031-0 DOI: https://doi.org/10.1038/s41576-018-0031-0