[A 63-year-old man with progressive cauda equina/conus medullaris syndrome]

A 63-year-old man, whose father died of malignant lymphoma, developed subacutely cauda equina/conus medullaris syndrome progressed over 3 months. Initial radicular pain, ascending motor and sensory paralysis without sacral sparing, vesicorectal dysfunction were similar with signs of spinal dural arteriovenous fistula. However, mild inflammatory signs, raised serum LDH, predominantly of LDH 3, lymphocytic pleocytosis and elevated beta 2 microglobulin in CSF suggested neurolymphomatosis. It was not supported, however, after CSF immunocytochemistry, myelogram, CT, Gd-MRI and Ga scan. Spinal cord/nerve root vascular syndromes of intravascular lymphomatosis (IVL) according to Glass J et al. was suspected because of the unique neurological progression similar to Foix-Alajouanine syndrome, hypoxia without abnormalities in chest X-ray film, response to steroids and raised serum soluble IL-2 receptor. Multiple biopsies were performed with negative results. However, after all muscle biopsy confirmed IVL. The lower spinal irradiation was not effective. But CHOP regimen supplemented by granulocyte colony-stimulating factor (G-CSF) brought about swift neurological improvement and protection from late complications. Self-limiting polyneuropathy emerged during the biweekly CHOP therapy, 6 courses for 12 weeks. Eventually he was neurologically improving 10 months after the chemotherapy and adrenal enlargement, which was possibly of metastasis, was only against complete remission. This case was good outcome by biweekly CHOP using G-CSF when compared with very high mortality in reported IVL cases besides vincristine neurotoxicity under compromised blood-brain/nerve barrier due to IVL might affect the functional recovery. This case with IVL implied raised soluble IL-2 receptor and progressive cauda equina syndrome/ascending myelopathy as diagnostic clues, and efficiency of muscle biopsy to confirm IVL.