Headaches initially, progressed to develop neurological deficits (slurred speech, ataxia).
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Multiple T1 hypointense, T2/FLAIR hyperintense lesions within the deep white matter, including the anterior aspect of the left cerebral peduncle and right cerebellum, that demonstrate moderate central homogenous enhancement. A number of the lesions have restricted diffusion.
This patient proceeded to have biopsies of the various lesions that all showed similar findings.
MICROSCOPIC: Sections showing cores of grey and white matter tissue with moderate increase in cellularity. Patchy perivascular rarefaction of the white matter with an infiltrate of mononuclear cells including lymphocytes and macrophages that encroach the vessel walls and perivascular space focally. No vessel wall necrosis, endothelial swelling or proliferation and no evidence of thrombosis. No viral cytopathic change or neoplasia identified.
Luxol fast blue stains demonstrate active demyelination in the rarefied areas with myelin debris within mononuclear cells.
Bielschowsky stains highlight reduced axon density within the rarefied areas.
CD3, CD20 and CD68 immunohistochemistry demonstrate predominantly macrophages and a few T-lymphocytes in the parenchymal infiltrate, while the perivascular infiltrate consists mainly of T-lymphocytes and a few B-lymphocytes.
Micro-organism stains and JC virus negative.
Findings are of demyelination of uncertain aetiology with no evidence of vasculitis or neoplasia.
The patient was diagnosed with intravascular lymphoma at autopsy. On discussion with the pathologist, intravascular lymphoma can be difficult to delineate from demyelination.
Thank you to Dr Han Xin Lau for helping to contribute this case.