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230 A dog with a bone (marrow biopsy)
  1. Vivien Li1,
  2. Mahima Kapoor1,
  3. Michael Lunn1,
  4. Kate Cwynarski2,
  5. Aisling Carr1
  1. 1National Hospital for Neurology and Neurosurgery
  2. 2Department of Haematology, University College London Hospital

Abstract

A 71-year-old woman presented with severe painful meningoradiculoneuritis progressing over 6 weeks. She had localised breast cancer treated 13 years prior. Differentials included a vasculitic or infiltrative process.

Neurophysiology showed a patchy, predominantly proximal axonal process with widespread active denervation. MRI revealed asymmetrical enhancement of the brachial plexus, left S1 and multiple cranial nerves. CSFs were lymphocytic (normal T-cells predominant), with elevated protein and negative cytology. FDG-PET was negative for malignancy. BMAT showed no evidence of a lymphoproliferative condition.

Left sural nerve biopsy was non-diagnostic. Right brachial plexus biopsy showed mixed T-/B-cell endoneurial inflammation not fulfilling criteria for vasculitis. She was stabilised with high-dose steroids and cyclophosphamide, followed by mycophenolate for inflammatory myeloradiculoneuritis. However, symptoms recurred when prednisolone was weaned.

Although T-cell receptor gene analysis from the initial BMAT demonstrated clonal rearrangements, it was only when the same clones were identified on two repeat BMATs and CSF that T-cell neurolymphomatosis was diagnosed. Poor performance status precluded chemotherapy. She continued on prednisolone and cyclosporine for 3.5 years, before dying of sepsis.

T-cell neurolymphomatosis is exceedingly rare. Clonality assessment can increase yield when other investigations including histology are non-diagnostic. This case highlights the value of repeated investigations when clinical suspicion is high.

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