Systematic evaluation of variables that contribute to noncompliance: a replication and extension.

The effects of time-out and escape extinction were examined with 2 preschoolers after we identified variables that may have resulted in noncompliance. Results of a functional analysis showed that noncompliance was highest in the escape condition for both participants. During the treatment evaluation, escape extinction resulted in greater reductions in noncompliance relative to time-out.

Intervenção fonoaudiológica em esclerose tuberosa / Speech language intervention in tuberous sclerosis

A Esclerose Tuberosa e caracterizada come uma desordem degenerativa causadora de nódulos em diversos órgãos, particularmente o cérebro. Os déficits que podem ser apresentados pelos pacientes com Esclerose Tuberosa São manifestados nos níveis biológico, cognitivo e social, fazendo com que o fonoaudiólogo seja membro da equipe de reabilitação. Assim, este trabalho teve come objetivo descrever aspectos da intervenção fonoaudiológica em uma paciente com Esclerose Tuberosa, atendida no Serviço de Atendimento Fonoaudiológico da Universidade Federal de Santa Maria - UFSM, que apresentava, de acordo com a avaliação neurológica, um quadro de retardo global do desenvolvimento relacionado a Esclerose Tuberosa, com manifestações epilépticas e comportamentos do aspecto autista como manifestação secundária. Foram relatados a avaliação multidisciplinar, a terapia fonoaudiológica e seus resultados. Observou-se, na avaliação fonoaudiológica, presença dos reflexos patológicos de sucção e de mordida, comunicação não-intencional com comportamentos reativos e nenhuma intencionalidade na interação. Como objetivos gerais da terapia fonoaudiológica, buscou-se auxiliar o desenvolvimento das funções atrasadas ou inexistentes, tais come cognição, linguagem, sociabilidade, e desenvolvimento psicomotor. As melhoras foram evidenciadas per meio da inibição dos reflexos patológicos, pela evolução das habilidades cognitivas visíveis na forma de manipulação dos objetos, e pelo aumento do contato visual. O relate de case, considerando o prognostico reservado e a complexidade das manifestações clinicas, proporciona mais perspectivas de estudo e pesquisas sobre a intervenção fonoaudiológica em pacientes com Esclerose Tuberosa.

Tuberous Sclerosis is characterized as a degenerative disorder that causes nodules in many organs, particularly the brain. Tuberous Sclerosis patients' deficits may occur in biological, cognitive and social levels, involving the speech language therapist in the rehabilitation staff. Thus, the aim of this study was concisely describe speech therapy intervention aspects of a patient with Tuberous Sclerosis, from the Speech Language and Hearing Clinic of Federal University of Santa Maria, who had a global development delay resulting from Tuberous Sclerosis, with seizures and autistic spectrum behavior as secondary expressions, accord¬ing to neurological assessment. Multidisciplinary assessment, the speech language therapy and its results were described. In speech language assessment, pathologic sucking and bite reflexes, non intentional communication with reactive behaviors, and no deliberate interaction were observed. As general aims of speech language therapy, the development of delayed and inexistent functions, like cognition, language, social skills and psychomotor development, was emphasized. The patient's improvement was evidenced by pathologic reflexes inhibition, evolution in cognitive skills revealed in objects manipulation, and better ocular contact. This case report offers more study approaches and researchs about the Tuberous Sclerosis patients intervention, considering her/his restrict prognosis and the complexity of clinic manifestations.

Seguimiento a largo plazo de angiofibromas tratados con láser de CO2 en 23 pacientes con esclerosis tuberosa / Long-term follow up of angiofibromas treated with CO2 laser in 23 patients with tuberous sclerosis

Introducción. La esclerosis tuberosa es una enfermedad autosómica dominante que determina la formación de hamartomas en múltiples órganos. Los cambios cutáneos son una de las características primarias de esta enfermedad. Dentro de éstos, los angiofibromas constituyen una forma común de presentación que causa importantes problemas cosméticos y médicos. El láser de CO2 se ha utilizado satisfactoriamente en el tratamiento de estas lesiones, pero existen escasos estudios que evalúen sus resultados a largo plazo. El objetivo de nuestro estudio es valorar la respuesta del tratamiento de los angiofibromas en el largo plazo. Métodos. Se realizó un estudio retrospectivo en 23 pacientes con angiofibromas tratados con láser de CO2. Los pacientes fueron tratados entre los años 1991 y 2000 inclusive con láser de CO2 continuo o superpulsado. Clasificamos los angiofibromas de acuerdo a su tamaño, resultados iniciales del tratamiento y edad de los pacientes (< 20 años y 20 años o más). Resultados. El rango de edad varió entre 12 y 39 años, con una media de 22,5 años. Después del tratamiento los pacientes fueron controlados durante un periodo de 6 meses a 10 años. En el análisis a largo plazo encontramos que un 30,1 % mantuvo el resultado inicial y el 60,9 % mostró diferentes grados de recidiva con un tiempo medio de recurrencia de 3 años. Cuando analizamos los resultados a largo plazo según tamaños de angiofibromas, resultado inicial y edad de los pacientes, no encontramos diferencias estadísticamente significativas entre los diferentes grupos. El análisis de supervivencia, con curvas de Kaplan-Meier, de los grupos de edad mostró que los pacientes más jóvenes (< 20 años) recayeron antes que los de mayor edad (rango logarítmico 4,01 y p < 0,05). Conclusiones. El tratamiento con láser de CO2 logra buenos resultados a corto plazo. Por otro lado, uno de los mayores problemas es la recaída en el largo plazo, ya que probablemente debido a la naturaleza de las lesiones, éstas no se pueden eliminar permanentemente. Este trabajo coincide con estudios previos que no han encontrado factores que permitan predecir la recurrencia de las lesiones. Sin embargo, podemos concluir que los pacientes de mayor edad recaen más tardíamente y tienen por tanto mejores resultados cosméticos a largo plazo

Introduction. Tuberous sclerosis is an autosomal dominant disease in which hamartomas form in multiple organs. Cutaneous changes are one of the primary characteristics of this disease. These include angiofibromas (AF), a common form of presentation that causes significant cosmetic and medical problems. The CO2 laser has been used satisfactorily in treating these lesions, but there are few studies that evaluate its long-term results. The aim of our study is to assess the long-term response of the treatment of angiofibromas. Methods. A retrospective study was carried out on 23 patients with angiofibromas treated with CO2 laser. The patients were treated between 1991 and 2000, inclusive, with continuous or superpulsed CO2 laser. We classified the angiofibromas by size, initial treatment results and patients' ages (< 20 years and 20 years or older). Results. Ages ranged from 12 to 39 years, with a median age of 22.5 years. After treatment, patients were followed up for a period of six months to 10 years. In the long-term analysis, we found that 30.1 % maintained the initial result, and 60.9 % showed different degrees of recurrence, with a mean recurrence time of 3 years. When we analyzed the long-term results by the size of the angiofibromas, initial result and patients' ages, we found no statistically significant differences among the different groups. The survival analysis of the age groups, with Kaplan-Meier curves, showed that the youngest patients (< 20 years) had earlier recurrences than the older ones (logarithmic range 4.01 and p < 0.05). Conclusions. CO2 laser treatment achieves good short-term results. On the other hand, one of the biggest problems is recurrence over the long term; this is probably due to the fact that, because of their nature, these lesions cannot be eliminated permanently. This work coincides with earlier studies which found no factors that would make it possible to predict the recurrence of the lesions. However, we can conclude that recurrence takes place at a later date in older patients, and therefore they have better cosmetic results over the long term

Gaining new understanding of tuberous sclerosis.

Although tuberous sclerosis is associated severe learning disability, it is also a physical disorder with growths that affect any part of the body. Recent genetic research explains the diversity of the condition. The behavioural phenotype of tuberous sclerosis has shown the autism and attention deficit disorder associated with the brain lesions.

Swallowing difficulties and early CNS injuries: correlation with the presence of axial skeletal deformities.

We reviewed the neurological findings in 36 adults with history of perinatal brain injury that required gastrostomy due to progressive swallowing difficulties in a population of 624 institutionalized patients. A stereotypic pattern of progression of deficits was observed. Risk factors that correlated with increased risk of swallowing disorders included: presence of multiple handicaps; progressive deformities of the spine and extremities; decreased range of motion of mandible, laterognathism and other mandibular asymmetries. These findings suggest that spinal deformities and swallowing difficulties are part of a spectrum of impaired function of paired axial muscles in this population. The identification of these risk factors may help in the long-term care plan of severely impaired patients whose survival is extended by the use of feeding tubes.