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

STUDY

DESIGN

A case report.

SETTING

Department of Orthopaedic Surgery, Shiga University of Medical Science, Japan.

PATIENT: 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.

RESULTS AND

CONCLUSION

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.