caa a22 4500 445809892 CHVBK 20180317145208.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s00415-011-5965-5 doi (NATIONALLICENCE)springer-10.1007/s00415-011-5965-5 Lymphomatosis cerebri presenting as a rapidly progressive dementia with a high methylmalonic acid [Elektronische Daten] [G. Leschziner, P. Rudge, S. Lucas, T. Andrews] We report a case of a patient with a rapidly progressive dementing illness and gait disturbance, in whom initial screening demonstrated a high methylmalonic acid level only, suggestive of a functional vitamin B12 deficiency. Despite B12 replacement therapy, he continued to decline. Further investigations demonstrated extensive signal change on magnetic resonance imaging involving grey and white matter within the corpus callosum, deep grey matter, brainstem and cerebellar peduncles, and patchy post-contrast enhancement. Laboratory testing revealed a raised erythrocyte sedimentation rate, raised anti-nuclear, intrinsic factor and lupus anticoagulant antibody titres, and a IgG kappa paraprotein. Cerebrospinal fluid was unremarkable. Bone marrow trephine biopsy showed monoclonal gammopathy of unknown significance. The patient initially responded to steroids, and underwent a brain biopsy, which was uninformative. However, 3weeks following admission, he died due to an aspiration pneumonia. Autopsy findings were consistent with a diffuse primary central nervous system small cell B-cell lymphoma. This has been rarely reported in the medical literature, but our case exhibits typical clinical features, although patchy enhancement on imaging and the high methylmalonic acid have not been previously described. We hypothesise that his functional B12 deficiency may have resulted from rapid cell turnover, perhaps in conjunction with the presence of intrinsic factor antibodies. Springer-Verlag, 2011 Lymphoma nationallicence Lymphomatosis cerebri nationallicence Vitamin B12 nationallicence CNS nationallicence B-cell nationallicence Leschziner G. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut Rudge P. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut Lucas S. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut Andrews T. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut Journal of Neurology Springer-Verlag 258/8(2011-08-01), 1489-1493 0340-5354 258:8<1489 2011 258 415 https://doi.org/10.1007/s00415-011-5965-5 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-5965-5 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Leschziner G. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut NATIONALLICENCE 700 1- Rudge P. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut NATIONALLICENCE 700 1- Lucas S. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut NATIONALLICENCE 700 1- Andrews T. Department of Neurology, Guy's and St Thomas' NHS Foundation Trust, St Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London, UK aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/8(2011-08-01), 1489-1493 0340-5354 258:8<1489 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer