Spinal muscular atrophy (SMA) is characterized by the degeneration of spinal alpha motorneurons. Nusinersen demonstrated good efficacy in the early disease phases. The feasibility of transcutaneous spinal cord stimulation (tSCS) in motor rehabilitation of patients with spinal cord injury has been demonstrated. We hypothesize that tSCS may activate intact and restored by nusinersen motorneurons and slow down the decline in motor activity, and may contribute to the development of motor skills in children with SMA. A case series is presented. Five children (6–13 years old) with SMA type II or III participated in the study. They were treated with nusinersen for ~2 years. Application of tSCS was carried out during physical therapy for 30–40 min per day in the course of 10–14 days. Outcome measures were goniometry of joints with contracture, forced vital capacity (FVC), RULM and HFMSE scales. The participants tolerated the stimulation well. The reduction of the contracture was ≥5 deg. RULM and HFMSE increased by ~1–2 points. Predicted FVC increased by 1–7% in three participants. Each participant expanded their range of active movements and/or learned new motor skills. Spinal cord stimulation may be an effective rehabilitation method in patients treated with nusinersen. More research is needed.
The review addresses modern methods of electrical stimulation
used to regulate the function of external respiration in humans.
The methods include abdominal functional stimulation of respiratory
muscles, diaphragmatic stimulation, phrenic nerve stimulation, epidural
and transcutaneous spinal cord stimulation. The physiological rationale
of their application is described along with the examples of their
use in clinical practice, including stimulation parameters and electrode
placement diagrams for each of the methods. We analyze the effectiveness
of each of the methods in patients with respiratory muscle paresis
and the features of their use depending on the level of spinal cord
injury. Special attention is paid to the method of epidural spinal
cord stimulation because this technique is widely used in electrophysiological
studies on animal models, providing deeper insight into the spinal
levels of the functional control of external respiration. The review
substantiates the great potential of using the method of transcutaneous
electrical spinal cord stimulation both in fundamental studies of
external respiration and in clinical practice.
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