Case Presentation:
A 39‐year‐old man with no medical history presented to the emergency department with a 2‐month history of urinary incontinence, constipation, and erectile dysfunction. He also reported paraesthesias, bilateral lower extremity weakness, and jerking movements of his right foot. Neurological examination revealed sensory abnormalities to pinprick, temperature, and proprioception in both legs up to the perianal region and pathologically brisk reflexes in both lower extremities. Nonsustained right ankle clonus and slight extensor plantar responses were also noted. Motor strength was within normal limits. Extensive serological testing was significant for an elevated ESR of 55 and LDH of 1400. MRI of the cervical and thoracic spine revealed a longitudinal enhancing lesion extending from C7 to T4 with mild expansion of the spinal cord at the epicenter of the lesion, Initially, this lesion was thought to represent a syrinx. CSF analysis was notable for a mildly elevated protein of 62 with normal glucose and cell count. Both CSF cytology and flow‐cytometry were negative for malignancy. Further review of the spinal lesion was suggestive of possible myelitis with syrinx‐like formation. MRI of the lumbar spine further revealed homogeneously enhancing lesions within the L2 and L4 vertebral bodies, suspicious for neoplasm. Bone marrow aspirate and biopsy were negative. Therefore, a CT‐guided biopsy of the L4 lesion was performed, which revealed diffuse large B‐cell lymphoma (DLBCL). The patient was treated with R‐CHOP chemotherapy followed by 4 cycles of intrathecal chemoprophylaxis with cytarabine. He also received oral steroids for 8 weeks. Successful response to this regimen was evidenced by complete eradication of the malignancy without relapse 3 years later, near‐complete neurological recovery, and radiological resolution of the spiral cord abnormalities including the syrinx.
Discussion:
Myelitis‐related transient syringomyelia has been reported in nonparaneoplastic etiologies such as sarcoidosis and postinfections. Myelitis associated with malignancy, however, remains a rare entity, with scattered reports in the world literature between 1903 and the present and the majority of cases reflecting poor outcomes. To the best of our knowledge, no similar cases of paraneoplastic myelitis (PNM) with transient syringomyelia related to DLBCL of the bone have been reported in the literature. We speculate that in our patient, local inflammatory factors provoked by a remote malignancy may have played a role in the pathogenesis of the syringomyelia.
Conclusions:
Early recognition of paraneoplastic myelitis by clinicians and aggressive treatment of the underlying malignancy are critical for improving clinical outcomes and potentially reversing neurological damage.
Author Disclosure:
S. Srour, none; E. Goldberg, none; S. Najjar, none.