Muscle Tone Assessment under General Anesthesia for Sjögren-Larsson Syndrome and Spasticity

Abstract

Introduction: Study of muscle tone in individuals with severe spasticity (Modified Asworth Scale - MAS:3) under general anesthesia can confirm or rule out the eventual necessity of the impending spasticity relieving ablative neurosurgery by observing the hypertonia reduction and passive range of motion expansion. Therefore, what we measure under muscle relaxants is practically a fixed deformity.

Case presentation: The study was performed on a girl with Sjögren-Larsson syndrome, presenting with icthyosis and spastic diplegia. Proposed intervention was Dorsal Rhizotomy. Under general anesthesia, with and without muscle relaxants, hypertonia was significantly reduced (MAS:1), but the angle of motion did not increase much.

Conclusion: We decided not to perform such a neurosurgical procedure. In ambiguous situations, the proposed study can help in decision-making for spasticity treatment.

Keywords: Dorsal rhizotomy; Muscle relaxants; Muscle tone; Sjögren-larsson syndrome; Spasticity.