Tratamiento farmacológico de las alteraciones de conducta en el Síndrome de Cornelia de Lange, a propósito de un caso
- Cora Fernández-Natal 1
- Juan J. Carballo 2
- 1 Departamento de Psiquiatría, Hospital Universitario de La Princesa. Madrid, España
- 2 Servicio de Psiquiatría del Niño y del Adolescente, Hospital General Universitario Gregorio Marañón, CI-BERSAM, Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM) Madrid, España
ISSN: 2660-7271, 1130-9512
Year of publication: 2019
Volume: 36
Issue: 3
Pages: 32-35
Type: Article
More publications in: Revista de Psiquiatría Infanto-Juvenil
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.
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