Sentinel inflammatory demyelinating lesions preceding primary CNS lymphoma

A 29-year-old man presented with acute seizures and visual impairment. Brain magnetic resonance imaging (MRI) showed multiple white matter T2-lesions with incomplete peripheral enhancement ([Figures 1A to 1C]). Considering the hypothesis of acute disseminated encephalomyelitis, intravenous methylprednisolone (IVMP) was administrated with full recovery. Two years later, he presented right-sided weakness. MRI disclosed a new T2-lesion, and spectroscopy suggested a tumefactive inflammatory pattern ([Figures 1D to 1H]). New extensive cerebrospinal fluid (CSF) and blood workup, including aquaporin-4-IgG, was unremarkable. Partial improvement was observed following IVMP. Six months later, after new weakness in the left arm along with a new periventricular lesion ([Figures 1I to 1L]), a brain biopsy was performed. Histopathological analysis revealed primary central nervous system (CNS) lymphoma ([Figure 2])[1],[2],[3].

Figure 1 Magnetic resonance imaging exams. (A-C): first magnetic resonance imaging performed on March 2016 indicated diffusion restriction on diffusion-weighted image (A), T2 hypersignal (B), and peripheral enhancement on post-contrast T1 sequences (C). D-H: Neuroimaging performed on July 2018 showed a new lesion with peripheral restricted diffusion on diffusion-weighted image (D), T2 hypersignal (E), thick annular enhancement (F), spectroscopy revealed Cho peak increase (G) and minimal relative cerebral blood volume map (rCBV) increase. (H). (I-L): Brain magnetic resonance imaging performed on January 2019 disclosed a new right periventricular lesion with diffusion restriction on diffusion-weighted image (I), mild T2 hyperintensity sequences (J), and homogeneous contrast enhancement (L).

Figure 2 Histopathological examination showed atypical lymphoid cell proliferation.

Conflict of interest:

There is no conflict of interest to declare.

Authors’ contributions:

DT: literature review, manuscript and imaging preparation, IS: literature review, manuscript preparation; MSP, GJ, KF and FMM: manuscript supervision and revision.

• References

• 1 Mark DK, Deva S, Rudolph J, Castellani RE, Morales SG, Reichs ASK, Robert KS. Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination. BMC Neurol. 2016 May;16-72. https://doi.org/10.1186/s12883-016-0596-1
• 2 Chiavazza A, Pellerino F, Ferrio A, Cistaro R, Soffietti R, Rudà R. Primary CNS lymphomas: challenges in diagnosis and monitoring. Biomed Res Int. 2018 Jun;3606970. https://doi.org/10.1155/2018/3606970
• 3 Jian-Giang Lu, Cian O’Kelly, Safwat G, Derek E, Christopher P, Gregg B. Neuroinflammation preceding and accompanying central nervous system lymphoma: case study and literature review. World Neurosurg. 2016 Apr;88:692.e1-692.e8. https://doi.org/10.1016/j.wneu.2015.11.099

Publication History

Received: 04 September 2020

Accepted: 25 September 2020

Article published online:
04 July 2023