Surgical Treatment of Upper Extremity Segmental Myoclonus in an Adolescent with Chiari Malformation and Cervicothoracic Syrinx

Abstract

<b><i>Background:</i></b> Myoclonus is an involuntary movement disorder characterized by semirhythmic jerking movements of muscle groups but is rarely seen in association with Chiari malformation type I (CM-1). CM-1 is a frequently encountered clinical entity in pediatric neurosurgery characterized by caudal displacement of the cerebellar tonsils with or without syringomyelia. We report a pediatric patient who presented with upper extremity myoclonus and was found to have CM-1 and a complex septated cervicothoracic syrinx eccentric to the left. <b><i>Case Presentation:</i></b> A 12-year-old female presented with 6 months of headaches and upper extremity paresthesias who subsequently developed a left upper extremity segmental myoclonus after a fall. MRI demonstrated a CM-1 and a large complex cervicothoracic syrinx with a midline and left paracentral cavities. Her myoclonus was nonepileptic and refractory to clonazepam, cyclobenzaprine, and gabapentin. She underwent an intradural Chiari decompression and duraplasty. Postoperatively, she had complete resolution of her segmental myoclonus. <b><i>Discussion:</i></b> This case demonstrates a durable resolution of posttraumatic upper extremity segmental myoclonus after surgical decompression of a CM-1 with syringomyelia. Thus, Chiari decompression should be considered in cases of myoclonus with CM-1 and syringomyelia.