Purpose To describe the ophthalmic symptoms and histopathological findings in a case of primary neurolymphomatosis (NL). Observations A man in his 60s with a prior diagnosis of chronic inflammatory demyelinating polyneuropathy developed facial numbness, diplopia, drooling, and difficulty swallowing. Over a 3-month period, he developed total ptosis and ophthalmoplegia of the right eye with a dilated, non-reactive pupil considered secondary to cranial nerve III and VI palsies. His left pupil subsequently became non-reactive to light and accommodation, and extraocular motility of the left eye was partially limited in all directions of gaze without ptosis. Autopsy findings included primary NL, diffuse large B-cell lymphoma of activated B-cell subtype, involving right and left cranial nerves V, VI, IX, and X; spinal nerve roots; both femoral nerves; and extrascleral, intrascleral, and intraocular short and long posterior ciliary nerves with extension into the adjacent choroid of both eyes. No evidence of lymphoma was identified elsewhere in the body. Conclusions and importance Our patient is only the second histological demonstration of ciliary nerve involvement by NL, and the first, to our knowledge, of primary NL spreading secondarily from the ciliary nerves into the choroid. Our patient demonstrates that NL, though rare, should be included in the differential diagnosis of ocular cranial nerve palsies and ophthalmoplegia.
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