Neuropathic arthropathy caused by syringomyelia

J Neurosurg Spine. 2013 Mar;18(3):303-9. doi: 10.3171/2012.11.SPINE12860. Epub 2013 Jan 4.


Object: Neuropathic arthropathy (Charcot joint) caused by syringomyelia is rare and commonly misdiagnosed. Few cases have been reported by neurosurgeons. The aims of this study were to analyze the clinical and imaging presentations of neuropathic arthropathy and to discuss the effect of surgical management of the primary neurological deficits on neuropathic arthropathy.

Methods: The authors retrospectively reviewed clinical and imaging data of 12 patients with neuropathic arthropathy caused by syringomyelia who were referred to the department of neurosurgery between January 2003 and September 2012. Radiographs revealed destruction, dislocation, disorganization, and increased density or debris in the joints. Magnetic resonance imaging showed a syrinx of the spinal cord in all patients, with Chiari malformation in 11 patients and tethered spinal cord in 1 patient. Neurosurgical operations were performed in 5 of 12 patients, including posterior fossa decompression in 4 patients and syrinx-subarachnoidal shunt placement in 1 patient. Surgical management of the neuropathic joints was not performed in any of the patients. All patients were followed up, with a mean duration of 39 months.

Results: Sixteen joints were involved, including 10 elbows, 3 shoulders, 2 interphalangeal joints, and 1 wrist. The side of the syrinx on cervical axial MRI was consistent with the side of the affected limb in every patient. Five patients who underwent neurosurgical treatments stated improvement in neurological dysfunctions and no deterioration in symptoms related to neuropathic arthropathy. In the 7 patients without neurosurgical treatments, 5 reported aggravation of neuropathic arthropathy manifestations, with deterioration of neurological symptoms in 4 of the 5 patients. The condition of the other 2 patients remained stable.

Conclusions: The elbow is the most frequently involved joint in neuropathic arthropathy caused by syringomyelia, followed by the shoulder. The authors speculate that the side of the syrinx determines the side of the neuropathic arthropathy. A detailed medical history and a careful physical examination are crucial for differentiating neuropathic arthropathy from other joint lesions. This study suggests that early management of the primary neurological condition may play an important role in preventing the development of neuropathic arthropathy and avoiding disease progression.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Arthropathy, Neurogenic / etiology*
  • Arthropathy, Neurogenic / surgery*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neurosurgical Procedures / methods*
  • Retrospective Studies
  • Syringomyelia / complications*
  • Syringomyelia / surgery*
  • Treatment Outcome