Repeat MRI of the brain with gadolinium demonstrated enhancement of cranial nerves V, VII, IX, X, XI, and the pineal gland cyst. Cervical and thoracic spinal MRI was normal. PET demonstrated hyperplastic marrow but no metastatic disease. He had been treated with a slow oral taper of prednisone, IV glucocorticoids, and IV immunoglobulin (IVIg).
On transfer to a tertiary facility, he endorsed dyspnea, weak cough, difficulty managing secretions, progressive weakness in the legs, diminished sensation in the left arm and leg, and 1 month of urinary incontinence and constipation. His medications on transfer were oral prednisone as well as antibiotics for an aspiration pneumonia, gastric ulcer prophylaxis, and symptom management for nausea and oral secretions. There were no notable medical conditions in his family history. He was a high school graduate with no significant substance use or travel history.
His neurologic examination was remarkable for visual sensation only for light and movement, fixed dilated pupils, downgaze in primary position, upward and lateral eye movement paresis, bilateral ptosis, diminished bilateral facial sensation, bilateral facial weakness, diminished hearing, poor soft palate elevation, weak cough, and absent gag reflex. His left leg was externally rotated with otherwise antigravity strength throughout the bilateral legs. He demonstrated resistance to movement which could be overcome throughout the bilateral arms. His reflexes were brisk throughout with plantar flexor responses bilaterally. He was well coordinated in all extremities except for slow and dysrhythmic left foot tap. Gait could not be assessed.
Repeat brain MRI with gadolinium () and dedicated fast imaging employing steady-state acquisition series () demonstrated multiple areas of cranial nerve enhancement, including the bilateral oculomotor, trigeminal, abducens, facial, and spinal accessory nerves as well as enhancement of the pineal gland and soft tissue masses in the bilateral trigeminal caves. Spine MRI () with gadolinium demonstrated multiple focal lesions within the spinal cord and central canal as well as a heterogeneously enhancing intradural, extramedullary thoracolumbar mass.
Question for consideration:
- What is the differential diagnosis for an intradural, extramedullary spinal cord mass?
Primary tumor considerations include meningioma, schwannoma, neurofibroma, dermoid tumor, and lipoma. The differential also includes drop metastases of medulloblastoma, ependymoma, glioma, and germ cell tumor.