Is Telerehabilitation as Effective as the Traditional Hospital-based Rehabilitation in Patients With Proximal Muscle Weakness? - A Randomized Controlled Study

Some neuromuscular diseases with myogenic or neurogenic origin present with proximal muscle weakness. These diseases preferentially affect muscles around shoulders and hips and hence are called limb girdle neuromuscular diseases (LG-NMD). Patients have difficulty in climbing stairs or hills, and reaching high shelves. The vast majority of these diseases are X-linked recessive, autosomal recessive or dominantly inherited muscular dystrophies and are called limb girdle muscular dystrophies (LGMD). Spinal muscular atrophy (SMA), a disease of motor neurons, is also characterized by progressive proximal muscle weakness. Although the diseases are progressive in both conditions, regular and proper doses of exercise starting from the early stages may delay the progression of muscle weakness and may prevent joint stiffness, and contractures.

The strengthening and aerobic exercise program increases overall wellness, condition and muscle strength in patients with neuromuscular diseases. The aerobic exercise program under supervision enhances oxidative capacity and muscle function without causing fatigue. Patients performing regular exercise program report an increase in their physical endurance, leg muscle strength and walking distance. Submaximal strengthening exercises combined with aerobic exercise are highly effective.

Regulation of the exercise program according to the needs of the patient is the most important step. Appropriate individualized exercises should be programmed by a physical medicine and rehabilitation physician and performed under the supervision of a physiotherapist in the hospital environment in order to be effective. Otherwise, success of rehabilitation and patient compliance might be reduced. With the development of technology, patients can perform exercises at their home under remote supervision by a physiotherapist via electronic connection. Therefore, the aim of our study is to investigate the effectiveness of two exercise programs supervised by a physiotherapist, performed in the hospital or at their home via electronic connection in a group of LGMD and SMA patients.

Fourteen consecutive patients from the Koç University Center for Muscle Diseases (KUCMD) diagnosed with LGMD or SMA are enrolled.

Patients followed by KUCMD outpatient clinic with proximal muscle weakness who held LGMD or SMA diagnoses will be evaluated by a physical medicine and rehabilitation physician. Demographic characteristics, Vignos scale, Brooke scale, Barthel index, upper extremity functional index, Nottingham Health profile, short form-36, 6 minute walk test and muscle thicknesses measured by ultrasound of certain muscles will be recorded. Patients will be randomized into two groups.