Rapidly progressing monoparesis caused by Chiari malformation type I without syringomyelia

Patients with Chiari malformation type I (CM-I) can manifest neurological symptoms, such as headache, neck pain, dysesthesia, swallowing disturbance, and paresis, which are usually stable or slowly progressive even if syringomyelia is coexistent. In some instances, however, acute onset of neurological symptoms has been reported but the pathogenetic mechanism and subsequent clinical course have not been explained. In those cases, it was reported that urgent treatment of foramen magnum decompression (FMD) was very effective. This work reports that an 11-year-old girl with CM-I subacutely developed unique symptoms and that urgent treatment of FMD was very effective.

We present here an 11-year-old girl with CM-I who subacutely developed dysphagia, left upper extremity monoparesis and sensory dysesthesia, with the limb assuming a peculiar posture at rest, with the wrist in extension and the elbow joint in flexion. Although her symptoms were assumed to be due to previously diagnosed CM-I without syringomyelia, no differences on magnetic resonance imaging (MRI) could be found except for slight change in the shape of tonsils compared with the previous ones. FMD and C1 removal with duraplasty was performed and resulted in an excellent neurological recovery.

This case is a reminder that the presence of a new neurological deficit referable to nuclei within, or tracts that traverse, the cerebromedullary junction is a firm surgical indication for FMD in a patient with CM-I. MRI was nearly identical during the asymptomatic and symptomatic periods in this case, and did not explain the timing of symptom onset.