RT Journal Article
SR Electronic
T1 Cauda Equina Syndrome in Ankylosing Spondylitis: Challenges in Diagnosis, Management, and Pathogenesis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.181259
DO 10.3899/jrheum.181259
A1 Chen Tang
A1 Franklin G.  Moser
A1 John Reveille
A1 Jane Bruckel
A1 Michael H.  Weisman
YR 2019
UL http://www.jrheum.org/content/early/2019/08/08/jrheum.181259.abstract
AB Objective Cauda equina syndrome (CES) is a rare neurologic complication of longstanding ankylosing spondylitis (AS). It is unclear what causes CES, and no proven or effective therapy has been reported to date. We have encountered 6 patients with longstanding AS diagnosed with CES. We set about to study their features, review the literature, and generate hypotheses regarding pathophysiology, as well as to speculate on the possibilities of early recognition and prevention. Methods We obtained permission from 6 patients with longstanding AS and CES to access their medical records and imaging studies for research purposes related to this paper. We collected and reviewed each patient’s medical history, imaging studies, disease duration, past therapies especially those that relate to AS, laboratory data, as well as any treatment they received for CES and followup results of each case to the present time. Results The 6 cases of CES with AS have remarkable similarity to each other in that several decades of the disease had passed before neurologic symptoms and later signs appeared. All cases have fused spines and facet joints without spinal fractures, spinal stenosis, or disc herniation. Conclusion CES is a rare yet debilitating neurologic complication of longstanding AS. The pathophysiology and treatments are far from clear. We postulate that chronic enthesitis of the vertebral column initiates the process that results in dural stiffening and formation of ectasias, causing downstream nerve root damage.