[Strategy of physiotherapy in dysphagia associated with spinal muscular atrophy type Ib: Case study]

Neuromuscular diseases progress to pathologic conditions which reveal structural or functional lesions of the elements forming the motor unit of the body. Typical clinical symptoms include muscle weakness, muscle flaccidity, pareses, and partial or total loss of reflexes. Excessive intensity of these symptoms leads i.a. to motor dysfunctions and ultimately to problems with physical development of children and youth. Treatment of such conditions is based on physiotherapy. The aim of this article is to expose the growing problem of dysphagia in patients with neuromuscular diseases and to systematize therapeutic activities during the process of rehabilitation.

We present the case of a 25-year-old male with SMA Ib diagnosed at birth, with full-blown symptoms of mechanical and oropharyngeal dysphagia. The patient complained of jaw rigidity, reduced oral aperture, difficulties in moving food in the oral cavity, loss of masticatory force, weakness and quickly progressing fatigue of jaw muscles during chewing, and problems with swallowing. Prior to the physiotherapeutic test, we performed a qualitative and quantitative assessment of temporomandibular joint (TMJ) mobility. Quantitative assessment of movement range was done in accordance with principles for functional examination of the masticatory organ in dentistry with the use of a millimeter ruler. We also recorded the subjective assessment by the patient of eating and swallowing. Assessment was done at the start and end of therapy (after 8 weeks). The patient underwent an 8-week course of therapy which consisted of stretching of cervical muscles according to Anderson, massage of soft tissues in the TMJ region, acupressure of the attachment of the masseter muscle (on the zygomatic bone), postisometric relaxation ofmasseter muscles (in the direction of abduction), active TMJ movements, active tongue exercises, and swallowing maneuvers. Rehabilitation was performed according to the sequence above during 20-minute sessions three times per week.

We observed objective and subjective improvement after our physiotherapy of the patient. TMJ mobility increased and the patient reported subjective improvement in the quality of life manifested with greater comfort during eating, moving, chewing, and swallowing food.

Therapy ofdysphagia in SMA Ib patients should be included as early as possible in the rehabilitation program in anticipation of problems with food ingestion. Therapy of dysphagia should be comprehensive, i.e. should not be limited to exercises stimulating TMJ mobility but should also address tongue movements and therapy of soft tissues. Therapy needs to be continuous and systematic regardless of the severity of dysphagia. It is particularly important to start dysphagia prevention measures in children with neuromuscular disorders by including exercises of the masticatory organ in the rehabilitation program.