Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging - An illustrative case and review of the literature

We report a patient who developed clinical and radiologic evidence of an inflammatory disease of the central nervous system (CNS), probably systemic lupus erythematosus, in association with a syrinx extending from the fourth ventricle to the upper thoracic spinal cord. A review of the literature concerning previous clinicoradiologic reports of syrinx formation, syringomyelia, or syringobulbia in association with inflammatory (nonvasculitic) CNS disease disclosed 43 other cases, all in patients with a diagnosis of definite or probable multiple sclerosis (n = 36) or neuromyelitis optica (Devic syndrome; n = 7). The available clinical and demographic data (n = 34) showed syrinx formation to be much more common in females; most syringes were cervical and/or thoracic. There was no evident relationship to disease duration or previous episodes of myelopathy. Outcome was generally good, with most patients regaining independent ambulation. Many of the reports emanate from Japan; the association may be more common in Asians. The mechanisms underlying syrinx formation and its relation, if any, to concurrent inflammation are uncertain, and may include both altered cerebrospinal fluid hydrodynamics and cord cavitation due to ischemia and/or necrosis. These reports do suggest that intramedullary spinal inflammation may be causally related to syrinx formation on occasion, rather than an incidental concurrence.