Pain and Syringomyelia: A Review

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Pain and Syringomyelia: A Review
The pathophysiological basis of chronic pain syndromes remains poorly defined. Central and dysesthetic pain are probably the most disabling of sensory disturbances associated with syringomyelia, and, unfortunately, effective treatment remains elusive. In this paper, the authors review their institutional experience with both clinical and laboratory studies of patients with syringomyelia, and they review the relevant literature. To date, there is no consensus as to the best treatment for central cord pain syndromes, although there are many promising areas of current research involving the use of neurochemicals in the spinal cord.

"Illness is the doctor to whom we pay most heed; to kindness, to knowledge, we make promise only; pain we obey." Marcel Proust, Remembrance of Things Past, 1922.

"Evil being the root of mystery, pain is the root of knowledge." Simone Weil, New York Notebook, 1942.

Syringomyelia is a chronic disease characterized by the presence of spinal cord cavitation. It has multiple causes and several classifications but is most commonly seen in association with Chiari I malformation. Pain is a prominent feature in 50 to 90% of adult patients with syringomyelia. Patients typically present with complaints of radicular pain (often in a capelike distribution), interscapular pain, and/or central cord pain. In addition to the more common clinical pain syndromes, approximately 40% of patients with syringomyelia experience significant dysesthetic pain, which is variously described as a burning sensation, pins and needles, or stretching of the skin (Table 1). Other common characteristics include dermatomal patterns of hypersensitivity, as well as trophic changes such as hyperhydrosis, glossy skin, coldness, and paleness. In patients afflicted with this type of pain, it is often an overwhelming and pervasive symptom that over-shadows other complaints. A similar type of pain has also been reported in other spinal cord pathological conditions such as traumatic injury, intramedullary tumors, and multiple sclerosis.

In animal models and clinical studies of spinal cord pain, the investigators have focused on traumatic SCI. There are few papers in which the authors specifically discuss syringomyelia and its relationship to the various pain syndromes. There have been, however, numerous anatomical studies of the normal spinal cord pain pathways in both human and animal models. Yezierski has comprehensively reviewed the ischemic, mechanical, and excitotoxic models of SCI, as well as three mechanisms of post-SCI pain. In patients with syringomyelia, the distribution of symptoms imperfectly correlates with the anatomical location of the spinal cavitation. In our experience syrinx cavities that extend paracentrally have rarely been found to be asymptomatic, and most have been characterized by segmental signs that could be related to the level and side of the paracentral extension.

Current research efforts are now predominantly focused on the role of neurochemicals and their receptor molecules. There is an extensive list of compounds being evaluated for their putative nociceptive function. The most widely investigated pain-related compound is substance P, which has been studied by our group in autopsy specimens. In this paper, we summarize our findings with regard to pain and syringomyelia as well as review the current literature.

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