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Hemodialysis-Related Cervical Spondyloarthropathy: An Unusual Cause of Neck Pain 2014

Category 
General Spine
 Mougnyan Cox
Danielle Fortuna
Ian Hayden
David Friedman
Upasana Joneja

Purpose 
This is an 84 year old woman with diabetes and end-stage renal disease on hemodialysis who presented to her primary care doctor with progressive neck pain. Physical exam did not reveal any neurologic deficits. Imaging showed an erosive soft tissue mass centered at C1 and C2, with mass effect on the cervicomedullary junction. 
Materials & Methods She was taken to the operative room for posterior cervical spine fixation and intraoperative biopsy. Intraoperative biopsy showed nodular amyloid deposition on Congo Red staining consistent with amyloidosis, likely B-2 microglobulin. Postoperatively, she had no neurologic deficits, and her pain improved over a course few weeks. Results 
Amyloidosis is characterized by deposition of abnormally folded proteins into tissue, resulting in organ dysfunction. Amyloid deposits typically show apple-green birefringence on Congo Red staining. Dialysis-related amyloidosis is caused by deposition of beta-2 microglobulin. This form of amyloidosis has a particular affinity for the muscles and joints, and may cause spondyloarthropathy, arthritis or pseudohypertrophy of muscles. In one prospective postmortem study, 50% of patients on dialysis for more than 7 years had amyloid deposition in shoulder, knee or sternoclavicular joints. 

Conclusion
 Cervical neck pain is usually the first symptom of amyloid-related cervical spondyloarthropathy. Soft tissue mass with erosion of cervical vertebral bodies is commonly seen. In severe cases, paralysis may occur due to spinal cord compression from adjacent amyloid soft tissue mass. Imaging shows a soft tissue mass with bony destruction. On MR, amyloid deposits are isointense or mildly hypointense on T1 and hypointense on T2 with variable enhancement. In cases with neurologic deficits, intractable pain or spinal instability, surgical fixation is often performed. 

References 
 1. Imaging of focal amyloid depositions in the head, neck, and spine: Amyloidoma. Parmar et al. AJNR 2010; 31:1165-70 2. Primary amyloidoma of the cervical spine. Hwang et al. AJNR 2000; 21:601-603 3. Fatal destructive cervical spondyloarthropathy in two patients on long-term dialysis. Allard et al. Am J Kidney Dis 1992; 19:81 4. Dialysis-related amyloidosis. Koch. Kidney Int 1992;41:1416