Clues on Optical Coherence Tomography Leading to Diagnosis of Primary CNS Lymphoma
Negar Moheb1, Timothy W. Olsen2, W. Oliver Tobin3, John J. Chen 1
1Neurology and Ophthalmology, 2Ophthalmology, 3Neurology, Mayo Clinic
Objective:
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Background:
Vitreoretinal lymphoma is a rare variant of primary central nervous system lymphoma (PCNSL). Around 20% of the PCNSL patients develop concurrent ocular involvement in the retina, vitreous, or optic nerve head. However, only 50% of these patients report visual symptoms, thus making a detailed ophthalmic examination a necessity.  
Design/Methods:
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Results:
A 65-year-old man with history of hypertension and hyperlipidemia, presented with progressive hypersomnia, confusion, intermittent horizontal binocular diplopia, tremors, and blood pressure fluctuations over a course of 9 months. A magnetic resonance imaging of the brain with and without gadolinium contrast showed multifocal confluent cerebral and cerebellar enhancing and non-enhancing white matter abnormalities. Lumbar puncture revealed borderline pleocytosis (WBC 5) with negative cytology. The patient was referred to neuro-ophthalmology to be evaluated for ocular signs that may help with the diagnosis.  At that visit, he was noted to have severe vision loss (visual acuity of 7/200) in the left eye that was not noticed by the patient until the time of examination. There was a left relative afferent pupillary defect. Further exam was notable for vitreous cells and yellow retinal placoid deposits. The optic nerve was normal. Optical coherence tomography (OCT) revealed multiple, hyper-reflective foci in the outer layers of the left macula with patchy discontinuity in the ellipsoid and photoreceptor layers. A vitreous biopsy detected CD20+, MYD88+ large B-cell lymphoma, and patient was initiated on systemic chemotherapy.  
Conclusions:
High-resolution OCT has enabled physicians to recognize unique features of different neoplasms such as PCNSL. Our case emphasizes the importance of detailed neuro-ophthalmologic examination and utilization of adjunct tests such as OCT in cases of progressive subacute leukoencephalopathy of unknown etiology. This would also lead to use of less invasive diagnostic methods such as vitreous biopsy instead of brain biopsy, earlier diagnosis, and treatment of patients with PCNSL.  
10.1212/WNL.0000000000203794