Chiari malformation type I associated with familial spastic paraplegia: report of a surgically treated case

Case report: We describe the case of a 7-year-old girl with Chiari malformation type I (CMI) and concomitant familial spastic paraplegia (FSP), who exhibited spastic gait disturbance. She also showed poor results on the standing tolerance test using thermography, which evaluates autonomic nerve function. The patient was subjected to posterior fossa decompression and cervical 1 (C1) laminectomy for CMI based on the result of the standing tolerance test. After the operation her gait disturbance and autonomic nerve dysfunction improved. Discussion: Although no cases of CMI and concomitant FSP have been reported, due to the fact that autonomic nerve dysfunction is one of CMI symptoms but never FSP, the diagnosis of autonomic nerve dysfunction of CMI using the standing tolerance test played an important role in selecting the surgical procedure for this case.