Presentation
Paraplegia and bowel and bladder dysfunction over a few days.
Patient Data
Longitudinally extensive cord signal T2 abnormality extends from the conus to the level of T6. The conus is expanded, with high T2 signal and 4mm ovoid faintly rim enhancing intra medullary lesion at the level of T11/12. The lesion demonstrates low T2 signal rim, particularly on the right. Patchy linear enhancement within the cord to the right just above this level is evident. There is enhancement of nerve roots at this level.
Left posterior parafalcine meningeal mass is noted. Optic nerves appear unremarkable. Appearances elsewhere are unremarkable.
Conclusion:
Conus is expanded with an intra medullary rim enhancing lesion centered at the level of T11/12 with further surrounding enhancement over the surface of the cord. Differential remains includes a primary cord mass lesion such as a tumor ( primary or secondary - dural intracranial mass probably represents a meningioma but could also be another secondary deposit) cavernoma, sarcoid or atypical infection. Given the presence of a focal mass lesion, inflammatory entities such as NMO and post infectious myelitis are also possible.
MICROSCOPIC DESCRIPTION:
The section shows a small fragment of loose fibrous tissue which has undergone partial coagulative necrosis. No organisms are identified. There are no granulomas and no evidence of tumor is seen.
The sections of each of the two specimens show fragments of spinal cord white matter and single and small aggregates of neuronal cells, several of which have undergone chromatolysis. There is patchy coagulative necrosis and patchy dense infiltration by lymphoid cells and monocyte-derived macrophages. The lymphoid cells are predominantly CD8+ T cells with lesser numbers of CD4+ T cells and CD20+ B cells (Fig 3).
The monocyte-derived macrophages show strong CD68 immunoreactivity. No organisms are identified. There are no granulomas. No evidence of tumor is seen.
Luxol Fast Blue (Fig 2) staining shows demyelination within and around areas of inflammation and Bodian staining shows extensive axonal destruction.
Immunostaining for HSV-1, HSV-2, CMV, EBV and SV-40/JC virus are negative. In situ hybridization for EBV DNA is negative.
DIAGNOSIS:
Dural, leptomeningeal and spinal cord biopsies: Necrotizing inflammatory process with overall features most consistent with transverse myelitis. Assay for Aquaporin 4 antibodies might be of assistance in determining etiology ; granulomas not identified; no evidence of tumor seen.