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  • Optic Nerve Infiltration in Primary CNS Lymphoma: Presentation and Outcome

    By Lynda Seminara
    Selected By: Deepak P. Edward, MD
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    Journal Highlights

    JAMA Neurology
    Published online October 2, 2017

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    Optic nerve infiltration (ONI) is a rare presentation of primary central ner­vous system lymphoma (PCNSL). To better understand lymphomatous ONI, Ahle et al. retrospectively reviewed data for affected patients. They found that visual and systemic prognosis was poor, even if neuroimaging showed a response to chemotherapy.

    The authors examined databases of 3 French hospitals for a 17-year period and identified 752 cases of PCNSL. Lymphomatous ONI was documented for 7 of them, and data were collected from medical records, including clinical presentation, neuroimaging results, and biological features. Treatment response was assessed clinically and by follow-up magnetic resonance imaging (MRI), utilizing response criteria of the Inter­national PCNSL Collaborative Group.

    The median age at diagnosis was 65 years (range, 49-78 years). Five of the 7 patients were female. Two patients had ONI at initial diagnosis of PCNSL, and 5 experienced ONI during disease relapse after chemotherapy. In all 7 patients, ONI was characterized by subacute severe visual impairment that progressed rapidly. MRI scanning of the optic nerve showed contrast en­hancement in all 7 patients and thick­ening in 3 of them. Additional lesions were observed in 4 patients. Lympho­matous meningitis was detected from cerebrospinal fluid in the 2 patients with ONI at initial presentation.

    At follow-up (median, 13 months), 5 patients had persistent severe low vi­sual acuity or vision loss, and 2 patients exhibited partial recovery. The median progression-free survival time after ONI identification was 11 months; the median overall survival period was 18 months.

    In conclusion, lymphomatous ONI is a rare condition involving rapid severe visual loss and poor optic nerve function, even in patients whose dis­ease responds to chemotherapy. Early diagnosis, which can be difficult in the absence of cerebral lesions or menin­gitis, along with prompt treatment can improve the visual prognosis.

    The original article can be found here.