Portal de Periódicos da CAPES

STEREOTYPIES IN RETT SYNDROME – ANALYSIS OF 65 PORTUGUESE PATIENTS

2006; Thieme Medical Publishers (Germany); Volume: 37; Issue: S 1 Linguagem: Inglês

10.1055/s-2006-945700

1439-1899

Teresa Temudo, M. Paula Souza Dos Santos, Karin Ziliotto Dias, Ana Moreira, JP Vieira, G Olivieira, E Calado, Clara Barbot, A Lévy, Inês Carrilho, M. A. S. Fonseca, Agenor Dias, Nuno Lobo Antunes, Pedro Cabral, J Perdra Monteiro, Regina Lúcia Elia Gomes, Carlos César Barbosa, Gabriela Andrada,

Child Nutrition and Feeding Issues

Objectives: Rett syndrome (RS) was discovered by Andreas Rett, when he noticed that two girls who were waiting for his consultation presented the same movement disorder: hand stereotypies. Hand stereotypies are the hallmark of RS and they emerge when purposeful hand use is lost. Behind hand stereotypies there are also other type of stereotypies involving other parts of the body (personal observation): head rolling, eye rolling, trunk rocking, intermittent leg elevation and tapping of the floor, toe walking, and swaying movements of all the body with shifting weight from one leg to the other, feet wringing. Stereotypies can also be very complex in the beginning of the disease, some girls exhibiting like a “stereotyped dance” (personal observation). Methods: We analised stereotypies in 65 patients who fullfill the revised diagnostic criteria for RS. All the patients were videotaped and stereotypies were classified based on the video and the information registered on the database.