Back Pain in Rare Diseases: A Comparison of Neck and Back Pain between Spinal Cord Ischemia and Spinal Dural Arteriovenous Fistula

Abstract

Neck and back pain may be noted like a first symptom in rare diseases: spinal cord ischemia and spinal dural arteriovenous fistula (SDAVF). Spinal cord ischemia is a rarer pathology, compared with cerebral ischemia, yet the morbidity and mortality are comparable in both cases; furthermore, classifying the acute loss of function in the spine, encountered in spinal cord ischemia as an important neurological entity. SDAVF presents the same clinical symptoms as spinal cord ischemia, but even though it has a progressive character, the impact in the quality of patients’ lives being equally as important. Between August 2012–August 2017 we admitted through the hospital emergency department 21 patients with spinal cord ischemia and 11 patients with SDAVF (only self-casuistry). Demographic (age, gender), clinical, imagistic (Magnetic Resonance Angiography, Magnetic Resonance Imaging), paraclinical data as well as history, time to diagnosis, the visual analogue scale for pain (VAS score), risk factors, surgical and medical treatment, evolution, neurorehabilitation, were all used to compare the two lots of patients. The aim of this study was to observe potential differences in the demographics, symptomatology, VAS scores and treatment in comparison for spinal cord ischemia and SDAVF, to facilitate the further recognition and management in these diseases. In group A we have 21 patients with spinal cord ischemia (14 females, 7 males). The median age was 41.3 years (range 19–64). The median time to diagnosis was 7 h. The most frequent symptoms were acute neck or back pain at onset (100%), motor deficits (95.24%), sensory loss (85.72%), and sphincters problems (90.48%). The most common location was the lumbosacral spine (14 cases; 66.67%; p-value = 0.03) for spinal cord ischemia and the thoracic spine (7 cases, 63.64%; p-value = 0.065) for SDAVF. The treatment of spinal cord ischemia was medical. In group B we included 11 patients (6 females, 5 males). The median age was 52.6 years (range 28–74). The median time to diagnosis was 3 months (range 2 days–14 months). Patients have progressive symptoms: neck or back pain (100%), gait disturbances (100%) and abnormalities of micturition (100%). The treatment of SDAVF was surgical occlusion of fistula. The proportion of severe VAS score (7–10) in patients with spinal cord ischemia was significantly higher than that in patients with SDAVF (100% vs. 18, 19%; p-value = 0.051). Taking into consideration that the usual findings and diagnosis of spinal cord ischemia and SDAVF are still challenging for neurologists and in some cases the difficulties are related to technical limitations, we consider these entities to be rare but very important for the life of our patients. Patients were grouped into spinal cord ischemia and SDAVF status and those with acute or chronic pain conditions, measured by the VAS score. Patients with spinal cord ischemia develop acute neurological symptoms. They are much younger than the patients with SDAVF and the recovery rate is higher. Patients with SDAVF develop a progressive myelopathy and they suffer considerable neurological deficits. Imaging the lesions with MR angiography or MRI, we can confirm the diagnosis.