Aktuelle Strategien der konservativen und operativen Therapie der häufigsten Muskelerkrankungen

Abstract

Though up to now no causal treatment for the majority of neuromuscular disorders is available, their disease progress and above all the quality of life of these patients can be decisively improved by established medical procedures. The main symptom is variably rapid, progressive muscle weakness, which leads to muscular imbalance and differently manifested impairment of motor functions. This results in the essential orthopaedic problems in these patients, e.g. contractures and deformities of the lower and upper extremities, foot deformities and sitting instability due to progressive scoliosis. Since the affected muscles have no physiological adaptability, they cannot be trained like healthy muscles. The orthopaedic treatment includes conservative methods, e.g. physiotherapy, orthotic devices and aids as well as surgical spine stabilisation and correction of the contractures of the lower extremities. Very important in the care of patients with neuromuscular disorders are the early prophylaxis and treatment of respiratory insufficiency by regular respiratory therapy to learn breathing and coughing techniques and self-exercises as well as the timely initiation of assisted ventilation.

Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients

“…The course of the disease and the patient’s quality of life can be greatly improved by established medical procedures [3,5,6]. Orthopaedic treatment of scoliosis and pelvic obliquity is of great importance for SMA patients.…”

Proximal spinal muscular atrophy: current orthopedic perspective

“…For patients with SMA type IIIb, preserved standing ability can decelerate the progression of scoliosis, and well-regulated strength training seems to have a slightly positive effect 31. Early physiotherapy, as well as walking and standing ability (including passively with technical support), have an important prophylactic relevance with benefit for all patients 30,31…”

“…Early physiotherapy, as well as walking and standing ability (including passively with technical support), have an important prophylactic relevance with benefit for all patients 30,31…”

“…For SMA type II and IIIa, corset therapy does not halt the progression of scoliosis 29–35. Nevertheless, it has its value as treatment before possible operative spine stabilization, especially for young children in the growth period,31 or if surgery is not possible due to poor general conditions or parental refusal.…”

“…Additional training to improve coordination and proprioception and physical procedures like massages, balneotherapeutic measures, heat applications, and electro-therapy can be added. The intensity of the activities should be adapted to the individual constitution of the patient 12,15,31,52…”