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Case Reports
. 2017 Mar 23;41(3):149-153.
doi: 10.1080/01658107.2017.1292533. eCollection 2017 Jun.

Case of Primary Leptomeningeal Lymphoma Presenting with Papilloedema and Characteristics of Pseudotumor Syndrome

Mai Takagi  1 , Hidehiro Oku  1 , Teruyo Kida  1 , Toshikazu Akioka  2 , Tsunehiko Ikeda  1
Affiliations
Case Reports

Case of Primary Leptomeningeal Lymphoma Presenting with Papilloedema and Characteristics of Pseudotumor Syndrome

Mai Takagi et al. Neuroophthalmology. .

Abstract

The authors describe an immunocompetent, 50-year-old man who complained of a daily transient blurring of his vision with bilateral papilloedema. His visual acuity was 20/20 OU, and the blind spot was enlarged bilaterally. There was intracranial hypertension, but imaging for systemic and brain tumours were negative. These findings suggested a diagnosis of the pseudotumor syndrome. However, MRI showed leptomeningeal enhancement, and acetazolamide successfully resolved his visual symptoms and papilloedema. Cytology and flow cytometry of the CSF led to the final diagnosis of primary leptomeningeal lymphoma (PLML). Clinicians need to be aware that a case of PLML may be misdiagnosed as peudotumor cerebri.

Keywords: Acetazolamide; cerebrospinal fluid cytology; papilloedema; primary leptomeningeallymphoma; pseudotumor cerebri; pseudotumor syndrome; transient visual obscuration.

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Figures

Figure 1.
Figure 1.
Visual fields of a patient with leptomeningeal lymphoma. Blind spots are enlarged bilaterally, but the visual fields are not constricted and a central scotoma is not present.
Figure 2.
Figure 2.
Fundus changes of the patient with leptomeningeal lymphoma. (A) At the initial examination. The optic discs are swollen in both eyes, i.e., papilloedema. (B) After taking acetazolamide for 3 months. Note the decrease in the papilloedema.
Figure 3.
Figure 3.
Axial brain (A, B) and sagittal spine (C) T1-weighted MR images with gadolinium enhancement. (A) There is no parenchymal mass lesion or clear ventriculomegaly in the brain. (B) Mass lesions are not present in the posterior fossa, and the fourth ventricle is not dilated. Leptomeningeal enhancement is seen around the cavernous sinus and roots of the fifth nerve and inner ear on both sides (arrows). (C) Extensive leptomeningeal enhancement of the lumbar and cauda equine can be seen (arrow).
See this image and copyright information in PMC

References

    1. Olson JE, Janney CA, Rao RD, Cerhan JR, Kurtin PJ, Schiff D, Kaplan RS, O’Neill BP.. The continuing increase in the incidence of primary central nervous system non-Hodgkin lymphoma: a surveillance, epidemiology, and end results analysis. Cancer 2002;95:1504–1510. - PubMed
    1. Primary Schlegel U. CNS lymphoma. Ther Adv Neurol Disord 2009;2:93–104. - PMC - PubMed
    1. Haldorsen IS, Espeland A, Larsson EM.. Central nervous system lymphoma: characteristic findings on traditional and advanced imaging. AJNR Am J Neuroradiol 2010;32:984–992. - PMC - PubMed
    1. Sagoo MS, Mehta H, Swampillai AJ, Cohen VM, Amin SZ, Plowman PN, Lightman S.. Primary intraocular lymphoma. Surv Ophthalmol 2014;59:503–516. - PubMed
    1. Kim JL, Mendoza PR, Rashid A, Hayek B, Grossniklaus HE.. Optic nerve lymphoma: report of two cases and review of the literature. Surv Ophthalmol 2015;60:153–165. - PMC - PubMed

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