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Optic Nerve Infiltration in Primary Central Nervous System Lymphoma.
Guido Ahle1, Valérie Touitou2, Nathalie Cassoux3
1Service de Neurologie, Hôpitaux Civils de Colmar, Colmar, France.
JAMA Neurology
|October 4, 2017
Summary
Optic nerve infiltration in primary central nervous system lymphoma (PCNSL) is rare and presents with rapid vision loss. This condition has a poor prognosis, even with chemotherapy.
Area of Science:
- Neuro-oncology
- Ophthalmology
- Oncology
Background:
- Primary central nervous system lymphoma (PCNSL) typically causes visual impairment through intraocular involvement.
- Optic nerve infiltration (ONI) is a rare presentation of PCNSL.
Purpose of the Study:
- To detail the clinical presentation, imaging findings, and outcomes of ONI in PCNSL.
- To differentiate ONI from more common intraocular lymphomas in PCNSL.
Main Methods:
- Retrospective analysis of 752 PCNSL patients across 3 French hospitals (1998-2014).
- Inclusion of 7 patients with documented ONI, excluding intraocular, orbital, or systemic lymphoma.
- Assessment of clinical data, neuroimaging (MRI), CSF analysis, treatment, and outcomes.
Main Results:
- Seven patients (5 female, 2 male; median age 65) with ONI were identified.
- All patients experienced rapidly progressive, severe visual impairment.
- MRI revealed optic nerve enlargement (3/7) and contrast enhancement (7/7); 4/7 had additional CNS lesions.
Conclusions:
- Optic nerve infiltration is an uncommon and challenging presentation of PCNSL.
- ONI carries a poor visual and systemic prognosis, worse than vitreoretinal lymphomas.
- Rapidly progressive visual impairment characterizes ONI, contrasting with slower deterioration in intraocular disease.
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