title: Quantification of functional abilities in Rett syndrome: a comparison between stages III and IV.
abstract: We aimed to evaluate the functional abilities of persons with Rett syndrome (RTT) in stages III and IV. The group consisted of 60 females who had been diagnosed with RTT: 38 in stage III, mean age (years) of 9.14, with a standard deviation of 5.84 (minimum 2.2/maximum 26.4); and 22 in stage IV, mean age of 12.45, with a standard deviation of 6.17 (minimum 5.3/maximum 26.9). The evaluation was made using the Pediatric Evaluation of Disability Inventory, which has 197 items in the areas of self-care, mobility, and social function. The results showed that in the area of self-care, stage III and stage IV RTT persons had a level of 24.12 and 18.36 (P=0.002), respectively. In the area of mobility, stage III had 37.22 and stage IV had 14.64 (P<0.001), while in the area of social function, stage III had 17.72 and stage IV had 12.14 (P=0.016). In conclusion, although persons with stage III RTT have better functional abilities when compared with stage IV, the areas of mobility, self-care, and social function are quite affected, which shows a great functional dependency and need for help in basic activities of daily life.
authors: School of Arts, Sciences and Humanities, University of São Paulo, São Paulo, Brazil.
authors: Carlos Bm
authors: CB
authors: Monteiro
authors: MOVE Research Institute Amsterdam, Faculty of Human Movement Sciences, VU University, Amsterdam, the Netherlands.
authors: Geert Jp
authors: GJ
authors: Savelsbergh
authors: Burke-Cornell Medical Research Institute, White Plains, NY, USA.
authors: Ana Rp
authors: AR
authors: Smorenburg
authors: Department of Neurology, University of São Paulo, São Paulo, Brazil.
authors: Zodja
authors: Z
authors: Graciani
authors: Motor Behavior Laboratory, University of São Paulo, São Paulo, Brazil.
authors: Camila
authors: C
authors: Torriani-Pasin
authors: Laboratory of Scientific Writing, Department of Morphology and Physiology, School of Medicine of ABC, Santo Andre, Brazil.
authors: Luiz Carlos
authors: LC
authors: de Abreu
authors: Department of Speech Language and Hearing Therapy, Faculty of Philosophy and Sciences, UNESP, Marilia, Brazil.
authors: Vitor E
authors: VE
authors: Valenti
authors: Department of Neurology, University of São Paulo, São Paulo, Brazil.
authors: Fernando
authors: F
authors: Kok
keywords: Rett syndrome; disability evaluation; mobility limitation; self-care