Demencia rápidamente progresiva como presentaciÓn de una macroglobulinemia de Waldenström: hallazgos de la resonancia magnética cerebral en el síndrome de Bing-Neel

Case report. A 68-year-old male with no relevant clinical history was admitted to hospital because of symptoms of cognitive impairment (attentional deficit, short-term memory disorders and behavioural disorders), accompanied by apraxia of gait and rectal and urinary incontinence. Results of a general clinical exploration were normal. In the MRI study of the brain numerous areas of hypersignal were observed in different arterial territories, which stood out in the T 2 , FLAIR and, above all, in diffusion-weighted sequences. Analytical studies showed hypergammaglobulinemia with monoclonal IgM-κ and a bone marrow biopsy revealed infiltration by plasmatic cells. Bing-Neel syndrome was diagnosed. Conclusions. We underline this exceptional form of presentation of Waldenstrom's macroglobulinemia and highlight the findings of diffusion-weighted MRI, which suggested multiple infarcts for which a neoplastic vascular obstruction mechanism similar to that involved in malignant angioendotheliomatosis is posited.