Prevention and Management of Limb Contractures in Neuromuscular Diseases

Abstract

Synopsis Limb contractures are a common impairment in neuromuscular diseases (NMD). They contribute to increased disability due to decreased motor performance, mobility limitations, reduced functional range of motion, loss of function for activities of daily living (ADL), and increased pain. The pathogenesis of contractures is multifactorial. Myopathic conditions are associated with more severe limb contractures in comparison to neuropathic disorders. Although the evidence supporting the efficacy of multiple interventions to improve ROM in NMD in a sustained manner is lacking, there are generally accepted principles with regard to splinting, bracing, stretching, and surgery that help minimize the impact or disability from the contractures.

Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders

“…Two factors might contribute to the 28% increase of VC in our cohort of NMD patients: First MI-E serves as ''internal'' physiotherapy releasing contractures and adhesions of the thoracic cage in accordance to the therapy of limb contractures. 15 Second especially the mechanical exsufflation in the MI-E maneuver might clear microatelectasis and augment reduced lung compliance reported to be present in NMD with severe lung volume restriction. 16,17 The release of microatelectasis might be especially due to the mobilization of airway secretions by the application of exsufflation.…”

Aquatic therapy for children with Duchenne muscular dystrophy: a pilot feasibility randomised controlled trial and mixed-methods process evaluation

“…34 Although evidence to support interventions aimed at improving the range of movement is lacking, there are generally recognised principles that should be carried out to delay or, where possible, prevent the development of contractures. 35 These include the prescription of a regular targeted stretching regime and the use of specific orthotics (e.g. resting or night splints are generally recommended).…”

Role of stretch therapy in comprehensive physical habilitation of patients with Charcot–Marie–Tooth hereditary neuropathy

“…Ско-рость прогрессирования заболевания широко варь-ирует: в одних случаях состояние стабильное в течение нескольких десятилетий, в других -про-грессирование заболевания довольно быстрое. Основная цель лечения пациентов с БШМТ -за-медление естественных процессов болезни и улуч-шение качества жизни и трудоспособности пациен-тов [6][7][8][9]. В настоящее время основным методом лечения БШМТ является программа эффективной абилитации, которая может предотвратить развитие деформаций конечностей и тем самым улучшить качество жизни пациента.…”

“…Стрейч-те-рапия показана при любой стадии БШМТ, как у взро-слых, так и у детей [15][16][17]. Основные цели примене-ния стрейч-терапии при БШМТ заключаются в улучшении подвижности суставов, профилактике и / или лечении укорочения ахилловых сухожилий, камбаловидной мышцы, улучшении статического позиционирования в течение длительного периода времени [3,6,18,19] и, как следствие данных измене-ний, улучшении равновесия пациентов с БШМТ [4,[20][21][22].…”