Retrobulbar optic neuropathy and non-Hodgkin lymphoma

Non-Hodgkin lymphomas (NHLs) constitute a group of heterogeneous diseases that can arise in lymphatic nodal or extranodal sites. Ocular lymphomas account for 1% of all NHLs. Tumor of the orbit, which can lead to compression of the optic nerve, is the most frequent presentation of the disease. Primary infiltration of the optic nerve and its sheath remains exceptional. We report the case of a 51-year-old female patient treated for a NHL. While she was considered to be in remission after four courses of chemotherapy, she presented a right visual loss with hand motion acuity. Her examination revealed a right afferent pupillary defect. Brain MRI emphasized an infiltration of her right optic nerve with no other orbit abnormality. Cerebrospinal fluid analysis showed lymphomatous meningitis. She was then considered to have lymphomatous optic neuropathy (LON). Despite initial improvement of the visual acuity with treatment, the patient died of bone marrow aplasia 6 weeks later. LON can be suspected in a painful and sudden visual loss in a context of neoplasia. The diagnosis is confirmed by MRI and cerebrospinal fluid analysis. LON may occur as the sole ocular manifestation of disease recurrence in a patient with systemic NHL, otherwise thought to be in clinical remission.