Abstract
BackgroundPrimary central nervous system lymphoma (PCNSL) may rarely be preceded by “sentinel demyelination,” a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions—contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively—can be a significant diagnostic challenge.Case presentationWe describe a 57-year-old woman with an unusual clinical course who presented with multi-focal enhancing white matter lesions demonstrated to be inflammatory demyelination by brain biopsy. Despite a good initial response to steroids and rituximab for treatment of presumed tumefactive multiple sclerosis, the patient’s condition rapidly deteriorated, and a repeat brain biopsy six months later was consistent with a diagnosis of diffuse large B-cell lymphoma.ConclusionsEarly clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression. Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed.
Highlights
Primary central nervous system lymphoma (PCNSL) may rarely be preceded by “sentinel demyelination,” a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM)
Early clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression
This case serves as a reminder that PCNSL can be preceded by sentinel lesions indistinguishable from the demyelination of MS or ADEM, and that steroid treatment before biopsy obscures a diagnosis of PCNSL
Summary
This case serves as a reminder that PCNSL can be preceded by sentinel lesions indistinguishable from the demyelination of MS or ADEM, and that steroid treatment before biopsy obscures a diagnosis of PCNSL. Ethics and consent to participate Not applicable for a case report on this patient that was treated without a research intention. This case report does not meet the definition of human research by the United States Department of Health and Human Services or the Food and Drug Administration guidelines. This is in accordance with the ethical manual and guidelines of the World Medical Association (see 59th WMA General Assembly, Seoul, Republic of Korea, October 2008).
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