We aim to describe a case of Epstein Barr Virus associated extranodal Natural Killer (NK)/T cell lymphoma (ENKL) presenting as primary neurolymphomatosis with rapid loss of nearly all cranial nerve functions.

Neurolymphomatosis (NL) is a rare lymphomatous infiltration of cranial or peripheral nerves.  Diagnosis can be elusive with half of cases diagnosed post mortem.  ENKL, while rare, has a predilection for the central nervous system.

A 52 year-old Asian male traveled to China and suffered a prolonged “flu”.  He developed right vocal cord paralysis followed by presumed left Bell’s palsy.  Less than 10 days after a course of prednisone, he presented with rapid onset of ptosis, ophthalmoplegia, facial diplegia, dysphonia and dysphagia.  He lost bilateral hearing over 2 days, and developed vagus nerve related autonomic instability.

Gadolinium enhanced magnetic resonance imaging (MRI) showed diffuse thickening and enhancement of all cranial nerves with relatively mild leptomeningeal enhancement among the left sacral nerve roots.  Lyme, HIV, quantiferon, ANA, and ENA were negative.  CSF showed 41 white blood cells, 82% lymphocytes, 31 glucose, 211 protein, EBV titer of 36,700 IU/mL.  Body computed tomography (CT), bone marrow biopsy, and CSF cytopathology/ flow cytometry were unrevealing.  Right CN 5 biopsy finally revealed atypical lymphocytes positive for EBV, CD2, CD3, CD8, CD30, CD43, and CD45 while negative for CD20 and GFAP, consistent with ENKL.

He was induced with high-dose methotrexate, procarbazine, vincristine (MPV) and brentuximab, followed by two additional cycles of methotrexate.  He achieved a complete radiographic response.  Ophthalmoplegia, dysphagia, and orthostasis resolved.   He is ambulatory and continuing to regain CN function.  An autologous bone marrow transplant is planned.

Primary NK/T Cell Neurolymphomatosis is a rare and challenging diagnosis.  Early aggressive pursuit of tissue diagnosis and treatment in our case provided meaningful recovery and a chance of remission.