Optic Nerve Infiltration in Primary Central Nervous System Lymphoma.

Importance: Visual impairment in primary central nervous system lymphoma (PCNSL) is caused mostly by intraocular lymphomatous involvement (vitritis and retinal infiltration), whereas optic nerve infiltration (ONI) is a rare condition. Objective: To describe the clinical presentation of ONI, its imaging characteristics, and outcome. Design, Setting and Participants: A total of 752 patients diagnosed with PCNSL were retrospectively identified from the databases of 3 French hospitals from January 1, 1998, through December 31, 2014. Of these, 7 patients had documented ONI. Exclusion criteria were intraocular involvement, orbital lymphoma, or other systemic lymphoma. Clinical presentation, neuroimaging, biological features, treatment, and outcomes were assessed. Main Outcomes and Measures: Treatment response was evaluated clinically and radiologically on follow-up magnetic resonance imaging (MRI) according to the International PCNSL Collaborative Group response criteria. Results: The 7 patients included 5 women and 2 men. Median age at diagnosis was 65 years (range, 49-78 years). Two patients had initial ONI at diagnosis, and 5 had ONI at relapse. Clinical presentation was marked by rapidly progressive and severe visual impairment for all patients. The MRI findings showed optic nerve enlargement in 3 patients and contrast enhancement of the optic nerve in all patients. Additional CNS lesions were seen in 4 patients. Examination of cerebrospinal fluid samples detected lymphomatous meningitis in 2 patients. Clinical outcome was poor and marked by partial recovery for 2 patients and persistent severe low visual acuity or blindness for 5 patients. Median progression-free survival after optic nerve infiltration was 11 months (95% CI, 9-13 months), and median overall survival was 18 months (95% CI, 9-27 months). Conclusions and Relevance: Optic nerve infiltration is an atypical and challenging presentation of PCNSL. Its visual and systemic prognosis is particularly poor compared with vitreoretinal lymphomas even in response to chemotherapy. Although intraocular involvement is frequent in PCNSL and clinically marked by slowly progressive visual deterioration, lymphomatous ONI is rare and characterized by rapidly progressive severe visual impairment.

Optical coherence tomography in clinically isolated syndrome: no evidence of subclinical retinal axonal loss.

BACKGROUND: Optical coherence tomography has emerged as a new tool for quantifying axonal loss in multiple sclerosis (MS). A reduction in retinal nerve fiber layer (RNFL) thickness is correlated with Expanded Disability Status Scale score and brain atrophy. OBJECTIVE: To investigate RNFL and macular volume measurements using optical coherence tomography in the clinically isolated syndrome population. DESIGN: Prospective case series. Settings Neurologic clinics at the university hospitals of Lille and Strasbourg (France). PARTICIPANTS: Fifty-six consecutive patients with clinically isolated syndrome (18 with optic neuritis and 38 without optic neuritis) and 32 control subjects. MAIN OUTCOME MEASURES: Macular volume and RNFL thickness. RESULTS: Mean (SD) overall RNFL thickness (98.98 [10.26] microm) and macular volume (6.86 [0.32] microm(3)) in the clinically isolated syndrome population were not significantly different compared with the controls (98.71 [9.08] mum and 6.92 [0.38] microm(3), respectively). No link was noted between atrophy of the RNFL or macula and conversion to MS at 6 months. CONCLUSIONS: Optical coherence tomography does not reveal retinal axonal loss at the earliest clinical stage of MS and does not predict conversion to MS at 6 months.

Optical coherence tomography in neuromyelitis optica.

BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease with combined features of optic neuritis and myelitis. This pathologic entity may induce severe disability, including visual loss and paraplegia. Other than clinical follow-up, there is no marker for severity of the disease. OBJECTIVES: To evaluate the use of optical coherence tomography (OCT) in NMO and to determine whether this new technique could be a good marker of axonal loss in NMO. DESIGN: Cross-sectional study. PARTICIPANTS: Thirty-five patients with NMO or at a high risk for NMO (having optic neuritis or myelitis and who are positive for NMO antibody) were prospectively studied. Fifteen healthy individuals served as control subjects. MAIN OUTCOME MEASURE: All patients underwent a complete ophthalmologic evaluation, including OCT, funduscopy, and visual field, visual acuity, and visual evoked potential testing. Expanded Disability Status Scale scores were assessed but without the visual data. Correlations between the visual test results and demographic or clinical characteristics were evaluated. RESULTS: Optical coherence tomography and visual field data were available for only 32 patients because 3 patients were blind. The mean retinal nerve fiber layer thickness was significantly reduced in patients with NMO compared with controls (P < .001). We found good correlation between the OCT results and visual field testing. We also found weak correlation between OCT results and both visual acuity and visual evoked potential latencies. We did not find any correlation between OCT results and age, sex, or disease duration. In contrast, retinal nerve fiber layer thickness was closely correlated with the Expanded Disability Status Scale score (P < .001). CONCLUSIONS: Optical coherence tomography results are significantly altered in patients with NMO. Optical coherence tomography is easy to perform, and the results are well correlated with visual acuity and visual field findings. It could be considered a marker of axonal loss because we found good correlation between OCT and the Expanded Disability Status Scale score. These preliminary results will need to be confirmed in a longitudinal prospective study.