A case of Arnold-Chiari syndrome with flaccid paralysis and huge syringomyelia.

Abstract

A case report. Department of Orthopaedic Surgery, Shiga University of Medical Science, Japan. A 13-year-old woman presented progressive weakness in the lower extremities, with predominance on the right. Magnetic resonance (MR) imaging revealed a huge syrinx. The patient also showed scoliosis, cleft palate, hearing impairment, excessive sweating, hairiness, dural ectasia, and malformation of the skull. METHOD AND OBJECTIVES: We treated a very rare case of Arnold-Chiari syndrome, which presented with flaccid paralysis. Methods of differential diagnosis and suitable treatment are discussed. Both the syrinx and muscle strength were quickly improved following placement of a syringo-peritoneal (S-P) shunt, after which the patient recovered the ability to walk. However, transient hypesthesia in the right hand occurred after the operation. The syrinx around the conus was thought to play a crucial role in the etiology of the patient case, which showed unique symptoms.