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A 47-year-old man had a fever of 39℃, weight loss and night sweat 4 months before admission. Two months later he presented with diplopia, and developed gait disturbance another month later. He was admitted because of disturbed consciousness with a Mini-Mental State Examination score of 24. Brain MRI with fluid-attenuated inversion recovery findings on admission showed bilateral asymmetric high intensity with partial mild swelling on white matter, corpus callosum, cerebellum and brainstem (figure 1A, B). Enhanced MRI detected punctate and curvilinear enhancement suggestive of perivascular infiltration and nodular enhancement (figure 1C, D). A cerebrospinal fluid analysis showed a cell count of 11 cells/µL (mononuclear cells: 94%), an elevated protein level (101 mg/dL). The oligoclonal bands were negative. IgG index was 0.5.
He underwent diagnostic fine needle aspiration brain biopsies. Inflammatory demyelinating lesions with axonal preservation were revealed (figure 1E–G). Diffuse infiltration of CD4+ T cells in the demyelinating lesions and perivascular cuffing with CD20+ B cells were observed. The circumscribed demyelinating lesions were identified in Klüver-Barrera staining (figure 1F) and myelin basic protein (MBP) immunohistochemistry with lipid-laden macrophages containing MBP immunoreactive debris. In the lesion, immunohistochemistry revealed the diminished immunoreactivity with macrophages containing myelin oligodendrocyte glycoprotein (MOG)-immunoreactive debris (figure 1G) and the proliferation of reactive astrocytes without loss of immunoreactivity against anti-glial fibrillary acidic protein antibody.
Established cell-based immunoassays revealed that he was positive for anti-MOG antibodies (1:4096) and negative for anti-AQP4 antibodies. Finally, his diagnosis was made as anti-MOG antibody demyelinating disease of the central nervous system (CNS). He was treated with three courses of intravenous methylprednisolone pulse therapy (1000 mg/day for 3 consecutive days) followed by gradually tapered oral prednisolone. Since mild conscious disturbance of Mini-Mental State Examination score 25 remained, plasma exchange was performed seven times. Then, consciousness disorder gradually improved and he was discharged.
We report a case of anti-MOG antibody demyelinating disease of the CNS with a characteristic perivascular gadolinium enhancement on MRI. To the best of our knowledge, this is the first report of the perivascular enhancement in anti-MOG antibody demyelinating disease. The perivascular enhancement occurs in other diseases, including neurosarcoidosis,1 primary angiitis of the CNS,2 CLIPPERS,3 and lymphoproliferative disorder such as lymphomatoid granulomatosis.4 We consider that the perivascular enhancement on MRI indicated lymphocyte infiltration around blood vessel. Brain biopsy in this case proved demyelinating lesions with lymphocyte infiltration in the tissue and around the blood vessels.
Anti-MOG antibody demyelinating disease has a wide range distribution and variable intracranial lesions. When perivascular enhancement on enhanced MRI is observed, we should be careful in making a differential diagnosis of anti-MOG antibody demyelinating disease.
Contributors TK was the attending doctor of the patient and performed neurological examination, prescribed medicines and made a decision about the patient's management. SM served scientific advices about MRI findings. KK served scientific advices about anti-MOG antibody. TF served scientific advices about neuropathology.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Ethics approval The Regional Ethics and Hospital Management Committee of Jikei University School of Medicine approved the study (approval number 30-089).
Provenance and peer review Not commissioned; externally peer reviewed.
Data sharing statement All authors had access to the data and a role in writing the manuscript.
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