Rett syndrome (RS) is a neurological disorder resulting from an X-linked dominant mutation. It is characterized by a variety of physical and perceptual disabilities, resulting in a need for continuous intervention programs to be administered on a regular basis throughout life. Many of these individuals with RS show fear of movement and, therefore, find it hard to accept external facilitation (so common in physical therapy intervention). In a search for novel intervention techniques that might improve their ability to cope with difficulties in daily situations, while also reducing their difficulty in handling motion inflicted by an external physical facilitator, we examined the use of the Snoezelen room. The Snoezelen, also known as the controlled multisensory environment, can provide a soothing atmosphere that appeals to the individual with RS, while at the same time it can improve physical, sensorial, and functional abilities. This article suggests various intervention goals that are appropriate for individuals with RS at different stages of the disorder. Since the management of young children with RS in the multisensory environment has been discussed at length in the past, this article will mainly describe intervention with adults with RS, focusing on three case stories. The present article reviews the available scientific materials on the topic of Snoezelen, incorporating clinical knowledge in the field of RS and suggesting this approach as an appropriate intervention method for this population.

译文

Rett综合征 (RS) 是一种由X连锁显性突变引起的神经系统疾病。它的特点是各种各样的身体和知觉残疾,导致需要在一生中定期执行持续的干预计划。这些患有RS的人中的许多人表现出对运动的恐惧,因此很难接受外部便利 (在物理治疗干预中很常见)。为了寻找新颖的干预技术,这些技术可以提高他们在日常情况下应对困难的能力,同时还可以减少他们处理外部物理促进者造成的运动的难度,我们研究了Snoezelen房间的使用。Snoezelen,也称为受控的多感官环境,可以提供舒缓的氛围,吸引个人RS,同时可以改善身体,感官和功能能力。本文提出了各种干预目标,这些目标适用于处于疾病不同阶段的RS患者。由于过去已经详细讨论了在多感官环境中对患有RS的幼儿的管理,因此本文将主要描述对患有RS的成年人的干预,重点是三个案例故事。本文回顾了有关Snoezelen主题的现有科学资料,结合了RS领域的临床知识,并建议将这种方法作为该人群的适当干预方法。

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