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We read with interest the report of Ayuso-Peralta et al,1 which describes a 58 year old woman who presented with several neurological symptoms. Brain imaging was consistent with leukoencephalopathy, and analysis of blood and cerebral spinal fluid was unrevealing. A few months later the patient experienced further neurological deterioration and an open brain biopsy showed central nervous system (CNS) lymphoma, together with diffuse demyelination.
The authors observed that the presentation of cerebral lymphoma as a diffuse leukoencephalopathy is not frequent and they discuss possible aetiologies of the predominant demyelination in their case. They do not mention the possibility of a paraneoplastic aetiology.
The authors reference a similar case2 previously reported in the Journal. That report also does not acknowledge a possible paraneoplastic aetiology for prominent diffuse brain demyelination preceding the discovery of CNS lymphoma. Two other recent reports in the Journal3–4 described focal tumour-like lesions of brain demyelination that preceded the discovery of CNS lymphoma. Only one of these reports4 mentioned laboratory data that suggested consideration of a paraneoplastic aetiology, the presence of serum antibodies directed against myelin oligodendrocyte glycoprotein.
One report elsewhere5 has described a patient who had a non-neurological malignancy and seminoma and who subsequently developed a paraneoplastic syndrome simulating encephalitis associated at autopsy with multiple foci of demyelination confined to cerebral white matter. Two other reports elsewhere6–7 have described biopsy confirmation of large focal demyelinating lesions in the brain associated with seminoma. The authors of these three reports all strongly considered the possibility of a paraneoplastic aetiology for the brain demyelination associated with seminoma, probably because the temporal association was close and the spatial association was distant.
The associations between brain demyelination and CNS lymphoma have been close, both temporally and spatially, making considerations of aetiology more complex. Taken together, the seminoma reports and the CNS lymphoma reports have many similarities in their patterns of associated brain demyelination, raising the possibility of similar mechanisms of demyelination. Many questions concerning aetiology remain unanswered. Given the information available, we suspect a paraneoplastic aetiology in all of these cases. We feel that future reports of brain demyelination associated with CNS lymphoma should consider this possibility in their data collection and in their discussion of results.
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