Abstract

A 69 year old retired lady presented with a 3 year history of progressive gait disturbance with unsteadiness and falls. Over the preceding year she had become recluse, failing to attend social gatherings and becoming anxious. She complained of lethargy and poor appetite and had lost weight. A witnessed generalised seizure led to admission. Examination revealed mixed pyramidal and extrapyramidal signs in the limbs with global hyperreflexia and upgoing plantars. There was upper limb ataxia with bilateral intention tremor and myoclonus. She was only obeying one stage commands.

Investigations revealed elevated ESR and monoclonal IgM kappa band with paraprotein concentration 9.4 g/L. MRI whole axis revealed periventricular diffuse T2 and FLAIR signal change and prominence of the leptomeninges along the distal spinal cord, conus medullaris and cauda equina. Bone scan showed prominent uptake within both humeri. Bone marrow trephine biopsy indicated a low-grade B-cell lymphoproliferative disorder; lymphoplasmacytic lymphoma. Serial CSF analysis revealed lymphocytic pleocytosis with elevated protein of 4.47–6.25 g/L. CSF protein electrophoresis demonstrated the presence of an IgM paraprotein with monoclonal IgM kappa band on immunonofixation. CSF immunophenotyping confirmed the presence of clonal B–cells.

We therefore present a rare case of Bing-Neel Syndrome, CNS manifestation of Wäldenstroms Macroglobulinaemia.