Chronic, progressive myelopathies with longitudinally-extensive T2-hyperintense lesions and specific gadolinium enhancement patterns, such as the axial “trident sign” of enhancement, have shown to be strongly suggestive of spinal cord sarcoidosis. However, the sensitivity and specificity of such enhancement patterns are not known.

Case report with description of history, examination, laboratory values, and imaging findings.

A 50-year-old woman presented with progressive left spastic hemiparesis over three weeks. MRI of the cervical spine revealed a longitudinally-extensive T2-hyperintense lesion with linear dorsal subpial enhancement and an axial trident pattern of enhancement. Cerebrospinal fluid analysis revealed a pleocytosis with a white blood cell count 20 cells/mm³ (89% lymphocytes), protein of 50 mg/dL, and glucose of 59 mg/dL with negative oligoclonal bands, cytology, and flow cytometry. Aquaporin-4-IgG and Myelin Oligodendrocyte Glycoprotein-IgG in serum were negative. Whole-body FDG-PET revealed increased uptake in the cervical cord but no other areas of systemic hypermetabolism. She was treated with three courses of IV methylprednisolone but her weakness continued to progress.

Repeat MRI two months after her initial presentation showed persistent enhancement. Her neurologic examination revealed left spastic hemiplegia and moderate weakness of her right upper extremity. Given the chronic, progressive myelopathy, persistent trident enhancement pattern suggestive of spinal cord sarcoidosis, and the potential risks involved with a cervical cord biopsy, a course of infliximab was trialed empirically. Despite this, she continued to worsen neurologically and developed hypercarbic respiratory failure. Spinal cord biopsy was pursued, ultimately revealing infiltration by diffuse large B-cell lymphoma.

Spinal cord lymphoma should be considered in patients presenting with chronic, progressive myelopathy and can present with similar enhancement patterns as spinal cord sarcoidosis. Atypical clinical features, such as rapid and severe neurologic worsening despite immunosuppression, should raise suspicion for diagnoses other than spinal cord sarcoidosis to allow for earlier detection and expedited disease-directed treatment.