Researchers Review Optic Nerve Head Plasmacytoma Manfestation of Muliple Myeloma

In patients with a history of multiple myeloma, extramedullary disease spread should be included in the potential differential diagnosis when vision loss is present, a report presented at the North American Neuro-Ophthalmology Society (NANOS) 2021 Annual Meeting shows.

Although ophthalmic manifestations of multiple myeloma have been reported, involvement of the cranial nerves is uncommon, most frequently presenting in advanced disease. Researchers presented the case of a 45-year-old woman with no past ocular history and a significant history for relapse, refractory, immunoglobulin A kappa multiple myeloma. The patient developed progressive vision loss in her left eye, including superior and temporal visual field loss. She was diagnosed with optic neuritis in a clinic setting. 

One month later, the patient returned with concerns from her oncologist that the vision loss was due to central nervous system involvement of the patient’s multiple myeloma. No diplopia or pain with eye movement was noted, but the patient reported bilateral frontal headaches. 

On examination, the researchers found 20/20 visual acuity in the right eye and a left relative afferent pupillary defect. Red desaturation test was 100% in the right eye and 30% in the left eye. In the left eye, disc edema and hyperemia with splinter hemorrhage were noted, with a small area of lobulated elevation on the nasal optic nerve head. Blurring of the disc margin and obscuration of the small vessels were also noted. 

The patient underwent a lumbar puncture, the results of which indicated malignant plasma cells in the cerebrospinal fluid. A T1-weighted postcontrast magnetic resonance image (MRI) of the brain and orbits showed both multifocal areas of nodular leptomeningeal enhancement and abnormal enhancement of the posterior left optic nerve sheath; this abnormality also involved the left orbital apex and anterior clinoid process. An additional MRI of the complete spine demonstrated extensive spinal and paraspinal tumor infiltration throughout the patient’s cervical, thoracic, and lumbar spine. 

The patient then underwent palliative radiotherapy treatment to the orbit, intrathecal methotrexate, and intravenous dexamethasone for 2 days. Visual acuity improved to 20/400 in the left eye, while red saturation remained decreased. Optical coherence tomography of the retinal nerve fiber layer indicated increase thickness in the left eye and normal ganglion cell layer in both eyes. 

Ultimately, the patient entered hospice care and died within 3 months. 

“Our case highlights the importance of keeping extramedullary disease spread in the differential for unilateral vision loss in a patient with a history of multiple myeloma,” the researchers concluded. 

Reference

Micieli JA, Sandor K, Peragallo JH. Optic nerve head plasmacytoma as a manifestation of multiple myeloma. Presented at: North American Neuro-Ophthalmology Society (NANOS) 2021 Annual Meeting; February 20-23, 2021; Poster 1.