Evans S, Forester K, Pettiford J M, Morozova O
Department of Physiatry, Childrens National Medical Center, The George Washington University School of Medicine, 111 Michigan Avenue, NW, Washington, DC 20010, USA.
J Inherit Metab Dis. 2009 Jun;32(3):381-6. doi: 10.1007/s10545-009-1190-0. Epub 2009 May 17.
Most children with paediatric neurotransmitter diseases have global functional deficits secondary to central nervous system damage. Paediatric physiatrists, working in conjunction with a multi-disciplinary team, help to improve physical function by normalizing muscle tone and improving body position. Components of spasticity, rigidity, and dystonia may all need to be considered in a comprehensive treatment programme. Complications of disordered tone include skin breakdown, pain, sleep disturbance, and dysphagia. With an integrated approach to use of medications and equipment as well as implementation of therapy and therapeutic exercise, physiatrists can help maximize functional independence for children with this group of disorders. Pharmacological treatment includes GABA-agonists including baclofen and benzodiazepines, alpha-2 adrenergic agonists, L: -dopa and dopaminergic agents, and dantrolene. Intrathecal baclofen may be used in patients refractory to these medications. In addition, physicians may utilize botulinum toxin, phenol, or surgical interventions such as selective dorsal rhizotomy or tendon lengthening. Pharmacological treatment must be used in conjunction with appropriate adaptive equipment in order to maximize therapeutic benefit. Focus on function in an attempt to increase independence is targeted to improve the child's quality of life. We present a framework and rationale to the management of the functional consequences of the paediatric neurotransmitter diseases.
大多数患有儿科神经递质疾病的儿童因中枢神经系统损伤而存在整体功能缺陷。儿科物理治疗师与多学科团队合作,通过使肌张力正常化和改善身体姿势来帮助改善身体功能。在综合治疗方案中可能需要考虑痉挛、僵硬和肌张力障碍等因素。肌张力紊乱的并发症包括皮肤破损、疼痛、睡眠障碍和吞咽困难。通过综合运用药物和设备以及实施治疗和治疗性锻炼,物理治疗师可以帮助患有这类疾病的儿童最大限度地实现功能独立。药物治疗包括γ-氨基丁酸(GABA)激动剂,如巴氯芬和苯二氮䓬类药物、α-2肾上腺素能激动剂、左旋多巴和多巴胺能药物以及丹曲林。鞘内注射巴氯芬可用于对这些药物难治的患者。此外,医生可能会使用肉毒杆菌毒素、苯酚或手术干预措施,如选择性背根切断术或肌腱延长术。药物治疗必须与适当的适应性设备结合使用,以最大限度地提高治疗效果。关注功能以提高独立性旨在改善儿童的生活质量。我们提出了一个针对儿科神经递质疾病功能后果管理的框架和基本原理。