Primary CNS vasculitis of the spinal cord

Salvarani C et al. Neurology 2008; 70:2394-2400. Study is retrospective and culls 5 cases from the "Mayo experience" that involve "prominent" spinal symptoms at presentation (another 10 had "slight" spinal symptoms). All patients had weakness, leg paralysis, urinary and in 3, fecal incontinence. It took a mean 29 days to diagnose. 1 patient had constitutional symptoms of fatigue and fever. All had "cerebral" symptoms as well. In one patient, the spinal symptoms preceded the brain symptoms by 10 months, in the others they were concomitant or after cerebral symptoms. CSF findings included mean protein of 280 and WBC in CSF of 350. Sed rates were normal. Gado showed diffuse enhancement of the cauda/nerve roots. 4/5 responded to some form of immune suppression but all had at least one relapse. In lit there are twelve cases associated with malignancy especially Hodgkin's disease. Cases involved the T spine whereas other AI disease often involved the C Spine.